CHiP Network Congenital Heart Journal Watch – July 2019

Pediatric Cardiology Featured Articles

Pediatric Cardiology Featured Manuscripts of May 2019

 Repeatability and Reproducibility of Neonatal Echocardiography: The Copenhagen Baby Heart Study.

Sillesen AS, Pihl C, Raja AA, Davidsen AS, Lind LE, Dannesbo S, Navne J, Raja R, Vejlstrup N, Lange T, Bundgaard H, Iversen K.

J Am Soc Echocardiogr. 2019 May 7. pii: S0894-7317(19)30101-4. doi: 10.1016/j.echo.2019.02.015. [Epub ahead of print]

PMID: 31076139

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Take Home Points: 

  • Reliability of neonatal echocardiographic measurements and acquisition of cardiac measurements are good for most parameters but poor for LV wall thickness measurements and poor for acquisition of TAPSE and mitral valve PW Doppler
  • Intraobserver reproducibility was better than interobserver reproducibility, but both were generally good
  • The lack of assessment of short axis m-mode measurements and function (shortening and ejection fraction) is an important shortcoming, as this is part of the typical study in nearly all echocardiograms

 

Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:The Copenhagen Baby Heart Study (CBHS) was designed to assess the reliability and agreement of neonatal echocardiography in order to better establish reference values in this population. This was a prospective, multicenter, population study of 25,000 neonates from 2016-2018. Standard views and measurements were obtained based on ASE guidelines. The study tested both intraobserver/repeatability (same sonographer) and interobserver/reproducibility (different sonographer) for echocardiographic measurements and acquisition. Figure 1 shows the practical set up:

 

Reliability was expressed via coefficient of variation (CV) and intraclass correlation coefficient (ICC). Table 1 shows the selected standard views.

 

72 infants with a mean birthweight of 3.4 +/- 0.4 kg, gestational age 40.1 weeks (median), and 9 days old at the time of echocardiography, were studied. Multiple results tables show reliability for both measurements and acquisition.  To summarize, for measurements, most parameters showed good reliability except for LV wall thickness (septum and posterior wall). Intraobserver reliability was slightly better than interobserver reliability as seen in Table 2.

 

The authors did note that one sonographer was less experienced, and when excluded from the analyses, overall reliability was improved.

For acquisition reliability, 22 infants were tested on 3 separate days and again, most parameters demonstrated moderate to excellent reliability. Septal and LV posterior wall thickness were poorly reliable for both long and short axis. TAPSE and mitral valve pulse wave Doppler images also showed poor reliability. Aortic annulus measurement was moderately reliable.

The authors spend some time in the discussion section mentioning previous studies of reliability (smaller studies or older children). They also explain why they used 2D views and long axis instead of m-mode/short axis view (flat ventricular septum, difficult to obtain a rounded LV shape). However, given its use in most neonatal echocardiograms worldwide, a significant limitation of the study was the lack of measuring the reliability of these short axis m-mode measurements and even more so, the lack of measurements of shortening and ejection fraction, where small differences may have a more significant clinical effect.

An accompanying well-written editorial makes the clear point that this study was an evaluation of random error, as are most studies of reliability, and the use of standard methodologies should ideally reduce the amount of systematic error. As an example, they discuss how the authors show that LV long axis measurements were better than short axis measurements even though the guidelines recommend short axis measurement to assure a more circular LV shape. Thus, the guidelines prioritize accuracy over reproducibility, even with the knowledge that there may be an increase in random error by doing so. However, since the short axis views have some shortcomings in the neonatal population, there is some justification to the choices of studied measures in this paper, as mentioned above. Overall, major strengths of the paper include the large number of echocardiograms and sonographers included, and that both measurement and acquisition reliability were assessed.

 

 

Exercise stress echocardiography: Impact on clinical decision-making in pediatric patients.

Dasgupta S, Friedman H, Allen N, Stark M, Ferguson E, Sachdeva R, Border WL.

Echocardiography. 2019 May;36(5):938-943. doi: 10.1111/echo.14326. Epub 2019 Apr 1.

PMID: 30934142

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Take Home Points:

 

  • Stress echo may be a helpful adjunctive modality in assessing pediatric patients and aid in the decision making process for exercise permission or restriction
  • Additional studies will be necessary to determine how well and in which patient subgroups stress echo will be helpful beyond the data obtained from the (metabolic) stress test itself

 

Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:Exercise stress echocardiography (ESE) is not a commonly used modality in pediatric patients and there are few published studies reporting the utility of ESE in this population. ESE is commonly used to assess cardiac function in adults with coronary issues, hypertrophic cardiomyopathy (HCM), aortic valve disorders, and EKG abnormalities (e.g. LBBB), but some pediatric labs have started to use ESE in pediatric patients with many of the above issues. The primary aim of this study was to evaluate the utility of ESE in a large single pediatric program and a secondary aim was to see how ESE impacted clinical management.

Test indications were divided into 5 categories: 1) symptoms with exercise, 2) sports/activity clearance, 3) HCM, 4) coronary abnormalities, and 5) abnormal EKG. It was not clearly stated, but patients with pre-existing CHD (besides coronary anomalies) were seemingly most often included in the 1st and 2nd categories. Most patients had ESE with a cycle ergometer at this center due to easier ability to acquire images (a few used a treadmill with a standard Bruce protocol). A James protocol was used, and images were obtained at each stage and immediately after peak. Tests were classified as either normal or abnormal with the latter further being classified as revealing a structural or functional defect or worsening severity of an existing diagnosis. Outcomes of the test were classified as no change in clinical management, change in clinical management, or unknown. If there was a change, the EMR clinical information was reviewed to determine if someone was restricted from activity, released from restrictions, or if there was a surgical/interventional referral.

353 ESE’s met inclusion criteria and the mean age of the patient cohort was 13.9 +/- 3.3 years. Table 1 shows patient demographics and diagnoses.

75% (263) of tests were normal. 23 patients with a normal ESE did not have follow up clinic visit documentation so a change in clinical management could not be determined for them. Clinical management changed as a result of the ESE in 144 (40%) patients (see Figure 1).

90 patients had an abnormal ESE with 2/3 undergoing a change in clinical management (nearly all were restricted). Among those restricted, 57% were for competitive sports, 32% for all activities but recreational, and 11% all activity. Table 3 shows this is more detail and Table 4 shows specific findings on the ESE that apparently warranted the restrictions. 7 patients had surgical or interventional referrals.

This study is mostly a descriptive study of use of ESE at a large pediatric center. While very interesting and novel, the indications for ESE use at this center seem to be non-traditional, and further data would be necessary to determine if and when other centers should perform ESEs. A significant major limitation in this study is that the exercise stress test data was not included in the analysis. Especially with metabolic data (CPET), it is possible that other variables may have been at least as important and perhaps more so in determining clinical management changes. It is also possible that many of the clinical management decisions could have been made without ESE, and the authors do not give insight how ESE exclusively would have been helpful in their patient population except in a handful of patients. In particular, I could imagine that many patients with a normal CPET may have been cleared without the ESE and many with certain diagnoses restricted without ESE (based on current clearance guidelines). I look forward to future analyses from this center, hopefully incorporating more robust CPET data and perhaps looking at ESE use in a larger number of patients within a specific subgroup.

Pediatric cardiology May 2019

 

  1. Apert Syndrome.

Wenger TL, Hing AV, Evans KN.

In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.

2019 May 30.

PMID: 31145570 Free Books & Documents

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  1. Point-of-care hemostasis in children with congenital heart disease, the POCHEMO study: baseline reference values of thromboelastometry and impedance aggregometry.

Longchamp D, Perez MH, Natterer J, Amiet V, Ferry T, Boegli Y, Mauron S, Dolci M, Plaza Wuthrich S, Di Bernardo S.

Blood Coagul Fibrinolysis. 2019 May 30. doi: 10.1097/MBC.0000000000000818. [Epub ahead of print]

PMID: 31157681

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  1. Prevention of delayed diagnosis in congenital heart disease.

Wacker-Gussmann A, Ehringer-Schetitska D, Herceg-Cavrak V, Hidvégi E, Jakab AE, Petropoulos A, Jokinen E, Fritsch P, Oberhoffer R.

Cardiol Young. 2019 May 30:1-2. doi: 10.1017/S1047951119000064. [Epub ahead of print] No abstract available.

PMID: 31145053

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  1. Correlation between pri-miR-124 (rs531564) polymorphism and congenital heart disease susceptibility in Chinese population at two different altitudes: a case-control and in silico study.

Yang W, Yi K, Yu H, Ding Y, Li D, Wei Y, You T, Xie X.

Environ Sci Pollut Res Int. 2019 May 29. doi: 10.1007/s11356-019-05350-4. [Epub ahead of print]

PMID: 31144180

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  1. Small atrial septal defects are associated with psychiatric diagnoses, emotional distress, and lower educational levels.

Udholm S, Nyboe C, Dantoft TM, Jørgensen T, Rask CU, Hjortdal VE.

Congenit Heart Dis. 2019 May 28. doi: 10.1111/chd.12808. [Epub ahead of print]

PMID: 31136098

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  1. Left Atrial Volumes and Phasic Function in Healthy Children: Reference Values Using Real-Time Three-Dimensional Echocardiography.

Linden K, Goldschmidt F, Laser KT, Winkler C, Körperich H, Dalla-Pozza R, Breuer J, Herberg U.

J Am Soc Echocardiogr. 2019 May 27. pii: S0894-7317(19)30159-2. doi: 10.1016/j.echo.2019.03.018. [Epub ahead of print]

PMID: 31147145 Free Article

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  1. Association of maternal disease and medication use with the risk of congenital heart defects in offspring: a case-control study using logistic regression with a random-effects model.

Lai T, Xiang L, Liu Z, Mu Y, Li X, Li N, Li S, Chen X, Yang J, Tao J, Zhu J.

J Perinat Med. 2019 May 27;47(4):455-463. doi: 10.1515/jpm-2018-0281.

PMID: 30794526

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  1. Liver disease secondary to congenital heart disease in children.

Komatsu H, Inui A, Kishiki K, Kawai H, Yoshio S, Osawa Y, Kanto T, Fujisawa T.

Expert Rev Gastroenterol Hepatol. 2019 May 26:1-16. doi: 10.1080/17474124.2019.1621746. [Epub ahead of print]

PMID: 31131680

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  1. Usefulness of Liver Stiffness on Ultrasound Shear-Wave Elastography for the Evaluation of Central Venous Pressure in Children With Heart Diseases.

Terashi E, Kodama Y, Kuraoka A, Ishikawa Y, Nakamura M, Sagawa K, Ishikawa S.

Circ J. 2019 May 24;83(6):1338-1341. doi: 10.1253/circj.CJ-18-1313. Epub 2019 Apr 23.

PMID: 31019158 Free Article

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  1. Assessment of Muscle Oxygenation in Children with Congenital Heart Disease.

Ross FJ, Arakaki LSL, Ciesielski WA, McMullan DM, Richards MJ, Geiduschek J, Latham G, Hsieh V, Schenkman KA.

Paediatr Anaesth. 2019 May 24. doi: 10.1111/pan.13668. [Epub ahead of print]

PMID: 31125476

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  1. [Predictive value of 3 different risk stratification models for patients after congenital heart surgeries].

Gao HW, Chen QM, Zhao W, Li D, Yan J, Wang X, Yang KM, Zhang H, Li SJ.

Zhonghua Xin Xue Guan Bing Za Zhi. 2019 May 24;47(5):388-392. doi: 10.3760/cma.j.issn.0253-3758.2019.05.010. Chinese.

PMID: 31142083

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Select item 20301290

 

  1. Holt-Oram Syndrome.

McDermott DA, Fong JC, Basson CT.

In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.

2004 Jul 20 [updated 2019 May 23].

PMID: 20301290 Free Books & Documents

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  1. Toward a characterization of language development in children with congenital heart disease: A pilot study.

Sommariva G, Zilli T, Crescentini C, Marini A, Pilotto C, Venchiarutti M, Gortan AJ, Fabbro F, Cogo P.

Child Neuropsychol. 2019 May 23:1-14. doi: 10.1080/09297049.2019.1617261. [Epub ahead of print]

PMID: 31120368

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  1. GATA4 screening in Iranian patients of various ethnicities affected with congenital heart disease: Co-occurrence of a novel de novo translocation (5;7) and a likely pathogenic heterozygous GATA4 mutation in a family with autosomal dominant congenital heart disease.

Kalayinia S, Maleki M, Rokni-Zadeh H, Changi-Ashtiani M, Ahangar H, Biglari A, Shahani T, Mahdieh N.

J Clin Lab Anal. 2019 May 22:e22923. doi: 10.1002/jcla.22923. [Epub ahead of print]

PMID: 31115957

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  1. Ancillary referral patterns in infants after initial assessment in a cardiac developmental outcomes clinic.

Monteiro SA, Serrano F, Tsang R, Smith Hollier E, Guffey D, Noll L, Voigt RG, Ghanayem N, Shekerdemian L.

Congenit Heart Dis. 2019 May 21. doi: 10.1111/chd.12789. [Epub ahead of print]

PMID: 31112369

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  1. Association of a Home Monitoring Program With Interstage and Stage 2 Outcomes.

Gardner MM, Mercer-Rosa L, Faerber J, DiLorenzo MP, Bates KE, Stagg A, Natarajan SS, Szwast A, Fuller S, Mascio CE, Fleck D, Torowicz DL, Giglia TM, Rome JJ, Ravishankar C.

J Am Heart Assoc. 2019 May 21;8(10):e010783. doi: 10.1161/JAHA.118.010783.

PMID: 31112448 Free Article

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  1. Congenital Heart Disease and Risk of Cardiovascular Disease: A Meta-Analysis of Cohort Studies.

Wang T, Chen L, Yang T, Huang P, Wang L, Zhao L, Zhang S, Ye Z, Chen L, Zheng Z, Qin J.

J Am Heart Assoc. 2019 May 21;8(10):e012030. doi: 10.1161/JAHA.119.012030.

PMID: 31070503 Free Article

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  1. Pulmonary hypertension during respiratory syncytial virus bronchiolitis: a risk factor for severity of illness.

Kimura D, McNamara IF, Wang J, Fowke JH, West AN, Philip R.

Cardiol Young. 2019 May 20:1-5. doi: 10.1017/S1047951119000313. [Epub ahead of print]

PMID: 31104634

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  1. Searching for modifiable risk factors for neurodevelopment in congenital heart disease: Lessons from the Giessen/Zurich hypoplastic left heart syndrome experience.

Seed M.

Int J Cardiol. 2019 May 20. pii: S0167-5273(19)31605-5. doi: 10.1016/j.ijcard.2019.05.038. [Epub ahead of print] No abstract available.

PMID: 31151765

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  1. A new vessel segmentation algorithm for robust blood flow quantification from two-dimensional phase-contrast magnetic resonance images.

Bidhult S, Hedström E, Carlsson M, Töger J, Steding-Ehrenborg K, Arheden H, Aletras AH, Heiberg E.

Clin Physiol Funct Imaging. 2019 May 18. doi: 10.1111/cpf.12582. [Epub ahead of print]

PMID: 31102479

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  1. Improving neurodevelopment in infants with complex congenital heart disease.

Harrison TM.

Birth Defects Res. 2019 May 17. doi: 10.1002/bdr2.1517. [Epub ahead of print] Review.

PMID: 31099484

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  1. First steps to a clinical research unit for developmental research in paediatric cardiology: conception and progress of the LEADER project (Long Term Early Development Research) in CHD.

Pfitzer C, Ferentzi H, Rosenthal LM, Kramer P, Berger F, Schmitt KRL.

Cardiol Young. 2019 May 17:1-7. doi: 10.1017/S1047951119000787. [Epub ahead of print]

PMID: 31097048

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  1. Comprehensive left ventricular myocardial deformation assessment in children with Kawasaki disease.

Sanchez AA, Sexson Tejtel SK, Almeida-Jones ME, Feagin DK Jr, Altman CA, Pignatelli RH.

Congenit Heart Dis. 2019 May 17. doi: 10.1111/chd.12787. [Epub ahead of print]

PMID: 31099489

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  1. Value of Rotational Thromboelastometry and Impedance Aggregometry for Evaluating Coagulation Disorders in Patients With Cyanotic and Nongenetic Congenital Heart Disease.

Pujol C, Stöckl A, Mebus S, Röschenthaler F, Holdenrieder S, Ewert P, Nagdyman N, Neidenbach RC, Kaemmerer H.

Am J Cardiol. 2019 May 15;123(10):1696-1702. doi: 10.1016/j.amjcard.2019.02.031. Epub 2019 Feb 23.

PMID: 30885417

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  1. MCP-1 Promotes Detrimental Cardiac Physiology, Pulmonary Edema and Death in the cpk model of Polycystic Kidney Disease.

Salah SM, Meisenheimer JD, Rao R, Peda JD, Wallace DP, Foster D, Li X, Li X, Zhou X, Vallejo JA, Wacker MJ, Fields TA, Swenson Fields KI.

Am J Physiol Renal Physiol. 2019 May 15. doi: 10.1152/ajprenal.00240.2018. [Epub ahead of print]

PMID: 31091126

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  1. Newborn Screening Pulse Oximetry to Detect Critical Congenital Heart Disease: A Follow-Up Survey of Current Practice at Army, Navy and Air Force Hospitals.

Robinson DL, Craig MS, Wells RS, Liesemer KN, Studer MA.

Mil Med. 2019 May 15. pii: usz116. doi: 10.1093/milmed/usz116. [Epub ahead of print]

PMID: 31090912

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  1. Risk factors of mortality and recoarctation after coarctation repair in infancy.

Lehnert A, Villemain O, Gaudin R, Méot M, Raisky O, Bonnet D.

Interact Cardiovasc Thorac Surg. 2019 May 14. pii: ivz117. doi: 10.1093/icvts/ivz117. [Epub ahead of print]

PMID: 31089681

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  1. Congenital Aortic Valve Stenosis.

Singh GK.

Children (Basel). 2019 May 13;6(5). pii: E69. doi: 10.3390/children6050069. Review.

PMID: 31086112 Free Article

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  1. EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Three-dimensional echocardiography in congenital heart disease.

Simpson JM, van den Bosch A.

Echo Res Pract. 2019 May 13;6(2):R75-R86. doi: 10.1530/ERP-18-0074. Review.

PMID: 31026813 Free PMC Article

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  1. Quantification of pulmonary/systemic shunt ratio by single-acquisition phase-contrast cardiovascular magnetic resonance.

Dunn TS 2nd, Patel P, Abazid B, Nagaraj HM, Desai RV, Gupta H, Lloyd SG.

Echocardiography. 2019 May 13. doi: 10.1111/echo.14358. [Epub ahead of print]

PMID: 31087463

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  1. Right ventricular-vascular coupling ratio in pediatric pulmonary arterial hypertension: A comparison between cardiac magnetic resonance and right heart catheterization measurements.

Breeman KTN, Dufva M, Ploegstra MJ, Kheyfets V, Willems TP, Wigger J, Hunter KS, Ivy DD, Berger RMF, Truong U.

Int J Cardiol. 2019 May 10. pii: S0167-5273(19)31199-4. doi: 10.1016/j.ijcard.2019.05.021. [Epub ahead of print]

PMID: 31109778

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  1. Comparative proteomic analysis of plasma of children with congenital heart disease.

Huang Q, Geng Z, Chen T, Cheng X, Gu H, Li Q, Li D, Liu R.

Electrophoresis. 2019 May 9. doi: 10.1002/elps.201900098. [Epub ahead of print]

PMID: 31070245

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  1. Cognitive Function in Pediatric Hypoplastic Left Heart Syndrome: Systematic Review and Meta-Analysis.

Siciliano RE, Prussien KV, Lee CA, Patel NJ, Murphy LK, Compas BE, Jordan LC.

J Pediatr Psychol. 2019 May 9. pii: jsz021. doi: 10.1093/jpepsy/jsz021. [Epub ahead of print]

PMID: 31069392

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  1. Glucose metabolism in children and adolescents: Population-based reference values and comparisons to children and adolescents enrolled in obesity treatment.

Frithioff-Bøjsøe C, Lund MAV, Kloppenborg JT, Nielsen TTH, Fonvig CE, Lausten-Thomsen U, Hedley PL, Hansen T, Pedersen OB, Christiansen M, Baker JL, Hansen T, Holm JC.

Pediatr Diabetes. 2019 May 9. doi: 10.1111/pedi.12859. [Epub ahead of print]

PMID: 31074070

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Select item 31067002

 

  1. Repeatability and Reproducibility of Neonatal Echocardiography: The Copenhagen Baby Heart Study.

Sillesen AS, Pihl C, Raja AA, Davidsen AS, Lind LE, Dannesbo S, Navne J, Raja R, Vejlstrup N, Lange T, Bundgaard H, Iversen K.

J Am Soc Echocardiogr. 2019 May 7. pii: S0894-7317(19)30101-4. doi: 10.1016/j.echo.2019.02.015. [Epub ahead of print]

PMID: 31076139

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Select item 31165593

 

  1. Tetralogy of Fallot.

Diaz-Frias J, Guillaume M.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.

2019 May 6.

PMID: 30020660 Free Books & Documents

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  1. Clinical and molecular spectrum of CHOPS syndrome.

Raible SE, Mehta D, Bettale C, Fiordaliso S, Kaur M, Medne L, Rio M, Haan E, White SM, Cusmano-Ozog K, Nishi E, Guo Y, Wu H, Shi X, Zhao Q, Zhang X, Lei Q, Lu A, He X, Okamoto N, Miyake N, Piccione J, Allen J, Matsumoto N, Pipan M, Krantz ID, Izumi K.

Am J Med Genet A. 2019 May 6. doi: 10.1002/ajmg.a.61174. [Epub ahead of print]

PMID: 31058441

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  1. Neonatal Lupus Erythematosus.

Diaz-Frias J, Badri T.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.

2019 May 5.

PMID: 30252317 Free Books & Documents

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  1. Familial GATA6 mutation causing variably expressed diabetes mellitus and cardiac and renal abnormalities.

Du YT, Moore L, Poplawski NK, De Sousa SMC.

Endocrinol Diabetes Metab Case Rep. 2019 May 3;2019. pii: EDM190022. doi: 10.1530/EDM-19-0022. [Epub ahead of print]

PMID: 31051468 Free PMC Article

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  1. Rare cause of cardiac failure in an infant.

Henriques C, Andrade H, Pires A.

Cardiol Young. 2019 May 2:1-3. doi: 10.1017/S1047951119000428. [Epub ahead of print]

PMID: 31044690

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Select item 31044297

 

  1. A child with a stroke, drug-refractory epilepsy and congenital heart disease: can a hemispherectomy be safely performed between staged cardiac procedures?

Jain P, Haller C, Pulcine E, Ochi A, Dipchand A, Weiss SK, Chin V, Ibrahim GM.

Childs Nerv Syst. 2019 May 2. doi: 10.1007/s00381-019-04171-7. [Epub ahead of print]

PMID: 31044297

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  1. What are the non-cardiac prognostic factors affecting mortality in neonates with aortopulmonary shunt.

Özlü F, Erdem S, Göçen U, Demir F, Atalay A, Akçalı M, Özbarlas N, Satar M.

J Matern Fetal Neonatal Med. 2019 May 2:1-6. doi: 10.1080/14767058.2019.1609928. [Epub ahead of print]

PMID: 30999804

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Select item 30923087

 

  1. Clinical spectrum of previously undiagnosed pediatric cardiac disease.

Fisher JD, Bechtel RJ, Siddiqui KN, Nelson DG, Nezam A.

Am J Emerg Med. 2019 May;37(5):933-936. doi: 10.1016/j.ajem.2019.02.029. Epub 2019 Feb 22.

PMID: 30833044

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  1. A De novo HDAC2 variant in a patient with features consistent with Cornelia de Lange syndrome phenotype.

Wagner VF, Hillman PR, Britt AD, Ray JW, Farach LS.

Am J Med Genet A. 2019 May;179(5):852-856. doi: 10.1002/ajmg.a.61101. Epub 2019 Feb 25.

PMID: 30806031

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  1. Ovine Models of Congenital Heart Disease and the Consequences of Hemodynamic Alterations for Pulmonary Artery Remodeling.

Johnson Kameny R, Datar SA, Boehme JB, Morris C, Zhu T, Goudy BD, Johnson EG, Galambos C, Raff GW, Sun X, Wang T, Chiacchia SR, Lu Q, Black SM, Maltepe E, Fineman JR.

Am J Respir Cell Mol Biol. 2019 May;60(5):503-514. doi: 10.1165/rcmb.2018-0305MA.

PMID: 30620615

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Select item 30506840

 

  1. High prevalence of truncus arteriosus in pediatric congenital heart disease in Uganda.

Namuyonga J, Lubega S, Aliku T, Omagino J, Sable C, Lwabi P.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):186-188. doi: 10.4103/apc.APC_161_18. No abstract available.

PMID: 31143055 Free PMC Article

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  1. A new low-cost method of virtual cardiac dissection of computed tomographic datasets.

Gupta SK, Spicer DE, Anderson RH.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):110-116. doi: 10.4103/apc.APC_167_18. Review.

PMID: 31143035 Free PMC Article

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  1. Perfusion index cannot be currently recommended as an additional newborn screen for critical congenital heart disease: more data needed.

Ewer AK.

Arch Dis Child. 2019 May;104(5):411-412. doi: 10.1136/archdischild-2018-316515. Epub 2019 Apr 10. No abstract available.

PMID: 30971399 Free Article

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Select item 30554150

 

  1. Health-related quality of life in surgical children and adolescents with congenital heart disease compared with their age-matched healthy sibling: a cross-sectional study from a lower middle-income country, Pakistan.

Ladak LA, Hasan BS, Gullick J, Awais K, Abdullah A, Gallagher R.

Arch Dis Child. 2019 May;104(5):419-425. doi: 10.1136/archdischild-2018-315594. Epub 2018 Dec 15.

PMID: 30554150

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Select item 30413491

 

  1. Does pulsatility index add value to newborn pulse oximetry screening for critical congenital heart disease?

Searle J, Thakkar DD, Banerjee J.

Arch Dis Child. 2019 May;104(5):504-506. doi: 10.1136/archdischild-2018-315891. Epub 2018 Nov 9. No abstract available.

PMID: 30413491

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Select item 29674517

 

  1. Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral pulses.

Fabi M, Brighenti M, Donti A, Lanari M.

Arch Dis Child. 2019 May;104(5):507. doi: 10.1136/archdischild-2017-314663. Epub 2018 Apr 19. No abstract available.

PMID: 29674517

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Select item 30791694

 

  1. Study on the correlation of modified Blalock Taussig duct occlusion and platelet parameters in congenital heart disease.

Zhu Y, Zhang Y, Liu Y, Fan X, Ding N, Li Z.

Asian J Surg. 2019 May;42(5):599-603. doi: 10.1016/j.asjsur.2018.08.012. Epub 2018 Oct 16.

PMID: 30340821 Free Article

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Select item 30819513

 

  1. Neonatal Skin-to-Skin Contact: Implications for Learning and Autonomic Nervous System Function in Infants With Congenital Heart Disease.

Harrison TM, Chen CY, Stein P, Brown R, Heathcock JC.

Biol Res Nurs. 2019 May;21(3):296-306. doi: 10.1177/1099800419827599. Epub 2019 Feb 5.

PMID: 30722675

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Select item 30920148

 

  1. Left temporal plane growth predicts language development in newborns with congenital heart disease.

Jakab A, Meuwly E, Feldmann M, Rhein MV, Kottke R, O’Gorman Tuura R, Latal B, Knirsch W; Research Group Heart and Brain.

Brain. 2019 May 1;142(5):1270-1281. doi: 10.1093/brain/awz067.

PMID: 30957841

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Select item 30747481

 

  1. Tetralogy of Fallot with absent pulmonary valve-When the ductus is present: A case of isolated branch pulmonary artery and review of literature.

Rao S, Najm HK, Stewart RD, Ahmad M, Erenberg F, Yaman M.

Echocardiography. 2019 May;36(5):996-1000. doi: 10.1111/echo.14334. Epub 2019 Apr 22.

PMID: 31006901

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Select item 30683928

 

  1. High prevalence of congenital heart disease at high altitudes in Tibet.

Chun H, Yue Y, Wang Y, Dawa Z, Zhen P, La Q, Zong Y, Qu Y, Mu D.

Eur J Prev Cardiol. 2019 May;26(7):756-759. doi: 10.1177/2047487318812502. Epub 2018 Nov 12.

PMID: 30419180

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Select item 30293987

 

  1. Image quality and radiation dose of two prospective ECG-triggered protocols using 128-slice dual-source CT angiography in infants with congenital heart disease.

Chen B, Zhao S, Gao Y, Cheng Z, Duan Y, Das P, Wang X.

Int J Cardiovasc Imaging. 2019 May;35(5):937-945. doi: 10.1007/s10554-018-01526-0. Epub 2019 Jan 17.

PMID: 30656460

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Select item 30568155

 

  1. Left Atrial Muscular Band Assessed by Three-dimensional Echocardiography.

Saito Y, Nagashima K, Watanabe R, Okumura Y.

Intern Med. 2019 May 1;58(9):1369-1370. doi: 10.2169/internalmedicine.2096-18. Epub 2018 Dec 18. No abstract available.

PMID: 30568155 Free PMC Article

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Select item 30826226

 

  1. Dynamic Three-Dimensional Geometry of the Tricuspid Valve Annulus in Hypoplastic Left Heart Syndrome with a Fontan Circulation.

Nguyen AV, Lasso A, Nam HH, Faerber J, Aly AH, Pouch AM, Scanlan AB, McGowan FX, Mercer-Rosa L, Cohen MS, Simpson J, Fichtinger G, Jolley MA.

J Am Soc Echocardiogr. 2019 May;32(5):655-666.e13. doi: 10.1016/j.echo.2019.01.002. Epub 2019 Feb 28.

PMID: 30826226

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Select item 30942505

 

  1. Altered plasma proteins released from platelets and endothelial cells are associated with human patent ductus arteriosus.

Hou HT, Xi-Zhang, Wang J, Liu LX, Zhang JF, Yang Q, He GW.

J Cell Physiol. 2019 May;234(5):6842-6853. doi: 10.1002/jcp.27433. Epub 2018 Nov 27.

PMID: 30480800

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Select item 30478832

 

  1. Coronary Artery Thrombosis in a Neonate With Critical Aortic Stenosis.

Tzifa A, Avramidis D, Patris K.

J Invasive Cardiol. 2019 May;31(5):E91-E92.

PMID: 31034441 Free Article

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Select item 30614102

 

  1. Nomograms for Cardiovascular Magnetic Resonance Measurements in the Pediatric Age Group: To Define the Normal and the Expected Abnormal Values in Corrected/Palliated Congenital Heart Disease: A Systematic Review.

Voges I, Giordano R, Koestenberg M, Marchese P, Scalese M, Ait-Ali L, Santoro G, Iervasi G, Valverde I, Kutty S, Cantinotti M.

J Magn Reson Imaging. 2019 May;49(5):1222-1235. doi: 10.1002/jmri.26614. Epub 2019 Jan 7. Review.

PMID: 30614102

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Select item 30954228

 

  1. Congenital heart disease associated with congenital diaphragmatic hernia: A systematic review on incidence, prenatal diagnosis, management, and outcome.

Montalva L, Lauriti G, Zani A.

J Pediatr Surg. 2019 May;54(5):909-919. doi: 10.1016/j.jpedsurg.2019.01.018. Epub 2019 Jan 31.

PMID: 30826117

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Select item 30911081

 

  1. Cardiac mechanics in infants with Down syndrome in the early neonatal period.

Breatnach CR, Bussmann N, Smith A, Levy P, McCallion N, Franklin O, El-Khuffash A.

J Perinatol. 2019 May;39(5):626-633. doi: 10.1038/s41372-019-0354-5. Epub 2019 Mar 25.

PMID: 30911081

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Select item 30867544

 

  1. Pediatric Cardiac MR Imaging:: Practical Preoperative Assessment.

Muscogiuri G, Suranyi P, Eid M, Varga-Szemes A, Griffith L, Pontone G, Schoepf UJ, De Cecco CN.

Magn Reson Imaging Clin N Am. 2019 May;27(2):243-262. doi: 10.1016/j.mric.2019.01.004. Epub 2019 Feb 14. Review.

PMID: 30910096

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Select item 30834692

 

  1. Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.

Berteloot L, Proisy M, Jais JP, Lévy M, Boddaert N, Bonnet D, Raimondi F.

Pediatr Radiol. 2019 May;49(5):575-585. doi: 10.1007/s00247-018-04331-y. Epub 2019 Jan 16.

PMID: 30652195

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Select item 30613845

 

  1. Imaging of central lymphatic abnormalities in Noonan syndrome.

Biko DM, Reisen B, Otero HJ, Ravishankar C, Victoria T, Glatz AC, Rome JJ, Dori Y.

Pediatr Radiol. 2019 May;49(5):586-592. doi: 10.1007/s00247-018-04337-6. Epub 2019 Jan 6.

PMID: 30613845

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Select item 30948683

 

  1. Infant With Trisomy 18 and Hypoplastic Left Heart Syndrome.

Kukora S, Firn J, Laventhal N, Vercler C, Moore B, Lantos JD.

Pediatrics. 2019 May;143(5). pii: e20183779. doi: 10.1542/peds.2018-3779. Epub 2019 Apr 4.

PMID: 30948683

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Select item 31109767

 

  1. Workforce Cost Model for Expanding Congenital and Rheumatic Heart Disease Services in Kenya.

Kontchou NT, McCrary AW, Schulman KA.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):321-327. doi: 10.1177/2150135119837201.

PMID: 31084310

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Select item 31084309

 

  1. Challenges of Caring for Functionally Single Ventricle Patients in Africa.

Edwin F, Elgamal MA, Dorra A, Reddy D, Entsua-Mensah K, Adzamli I, Yao NA, Tettey M, Tamatey M, Vosloo S, Kinsley R.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):338-342. doi: 10.1177/2150135118817769.

PMID: 31084309

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Select item 31084306

 

  1. [Clinical and genetic features of Mowat-Wilson syndrome: an analysis of 3 cases].

Wang H, Yan YC, Li Q, Zhang Z, Xiao P, Yuan XY, Li L, Jiang Q.

Zhongguo Dang Dai Er Ke Za Zhi. 2019 May;21(5):468-473. Chinese.

PMID: 31104665

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  1. Phenotype characterisation of TBX4 mutation and deletion carriers with neonatal and pediatric pulmonary hypertension.

Galambos C, Mullen MP, Shieh JT, Schwerk N, Kielt MJ, Ullmann N, Boldrini R, Stucin-Gantar I, Haass C, Bansal M, Agrawal PB, Johnson J, Peca D, Surace C, Cutrera R, Pauciulo MW, Nichols WC, Griese M, Ivy D, Abman SH, Austin ED, Danhaive O.

Eur Respir J. 2019 May 31. pii: 1801965. doi: 10.1183/13993003.01965-2018. [Epub ahead of print]

PMID: 31151956

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Select item 31148382

 

  1. Regional practice norms for the care of childhood cancer survivors at risk for cardiomyopathy: A Delphi study.

Kenney LB, Ames B, Margossian R, Moss K, Michaud AL, Williams DN, Nohria A.

Pediatr Blood Cancer. 2019 May 31:e27868. doi: 10.1002/pbc.27868. [Epub ahead of print]

PMID: 31148382

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Select item 31152184

 

  1. Prevention of delayed diagnosis in congenital heart disease.

Wacker-Gussmann A, Ehringer-Schetitska D, Herceg-Cavrak V, Hidvégi E, Jakab AE, Petropoulos A, Jokinen E, Fritsch P, Oberhoffer R.

Cardiol Young. 2019 May 30:1-2. doi: 10.1017/S1047951119000064. [Epub ahead of print] No abstract available.

PMID: 31145053

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Select item 31145542

 

  1. The association of elevated maternal genetic risk scores for hypertension, type 2 diabetes and obesity and having a child with a congenital heart defect.

Kaplinski M, Taylor D, Mitchell LE, Hammond DA, Goldmuntz E, Agopian AJ; Pediatric Cardiac Genomics Consortium.

PLoS One. 2019 May 29;14(5):e0216477. doi: 10.1371/journal.pone.0216477. eCollection 2019.

PMID: 31141530 Free PMC Article

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Select item 31152976

 

  1. An Infant With Hemodynamic Collapse.

Prejean SP, Raulston JEB, Kay GN.

Circulation. 2019 May 28;139(22):2588-2590. doi: 10.1161/CIRCULATIONAHA.119.040906. Epub 2019 May 28. No abstract available.

PMID: 31136225

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Select item 31132865

 

  1. Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association.

Lipshultz SE, Law YM, Asante-Korang A, Austin ED, Dipchand AI, Everitt MD, Hsu DT, Lin KY, Price JF, Wilkinson JD, Colan SD; American Heart Association Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; and Council on Genomic and Precision Medicine.

Circulation. 2019 May 28:CIR0000000000000682. doi: 10.1161/CIR.0000000000000682. [Epub ahead of print]

PMID: 31132865

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Select item 30362323

 

  1. Diazoxide Causality Assessment of a Pericardial Effusion in a Child with Kabuki Syndrome

Maffre I, Vincenti M, Dalla Vale F, Amouroux C, Werner O, Meilhac A, de Barry G, Amedro P.

J Clin Res Pediatr Endocrinol. 2019 May 28;11(2):218-219. doi: 10.4274/jcrpe.galenos.2018.2018.0193. Epub 2018 Oct 23. No abstract available.

PMID: 30362323 Free Article

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Select item 31138304

 

  1. A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review.

Liu X, Wang L, Zhou K, Hua Y, Shi X, Wang C.

Pediatr Rheumatol Online J. 2019 May 28;17(1):26. doi: 10.1186/s12969-019-0331-8.

PMID: 31138304 Free PMC Article

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Select item 31136621

 

  1. The factors affecting the disease course in Kawasaki disease.

Arslanoglu Aydin E, Ertugrul I, Bilginer Y, Batu ED, Sonmez HE, Demir S, Arici ZS, Sag E, Alehan D, Ozen S.

Rheumatol Int. 2019 May 28. doi: 10.1007/s00296-019-04336-2. [Epub ahead of print]

PMID: 31139951

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Select item 31150828

 

  1. Predictors and Consequences of Pediatric Hypertension: Have Advanced Echocardiography and Vascular Testing Arrived?

Hope KD, Zachariah JP.

Curr Hypertens Rep. 2019 May 27;21(7):54. doi: 10.1007/s11906-019-0958-3. Review.

PMID: 31134437

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Select item 31132082

 

  1. Left Atrial Volumes and Phasic Function in Healthy Children: Reference Values Using Real-Time Three-Dimensional Echocardiography.

Linden K, Goldschmidt F, Laser KT, Winkler C, Körperich H, Dalla-Pozza R, Breuer J, Herberg U.

J Am Soc Echocardiogr. 2019 May 27. pii: S0894-7317(19)30159-2. doi: 10.1016/j.echo.2019.03.018. [Epub ahead of print]

PMID: 31147145 Free Article

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Select item 31026225

 

  1. Liver disease secondary to congenital heart disease in children.

Komatsu H, Inui A, Kishiki K, Kawai H, Yoshio S, Osawa Y, Kanto T, Fujisawa T.

Expert Rev Gastroenterol Hepatol. 2019 May 26:1-16. doi: 10.1080/17474124.2019.1621746. [Epub ahead of print]

PMID: 31131680

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Select item 31129800

 

  1. Pediatric Cardio-Oncology: Development of Cancer Treatment-Related Cardiotoxicity and the Therapeutic Approach to Affected Patients.

Ryan TD, Nagarajan R, Godown J.

Curr Treat Options Oncol. 2019 May 25;20(7):56. doi: 10.1007/s11864-019-0658-x. Review.

PMID: 31129800

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Select item 31127942

 

  1. Very Large Patent Ductus Arteriosus With Severe Pulmonary Arterial Hypertension.

Konagai N, Fukui S, Kitano M, Fujimoto K, Nishii T, Ogo T, Yasuda S.

Circ J. 2019 May 24. doi: 10.1253/circj.CJ-19-0144. [Epub ahead of print] No abstract available.

PMID: 31130586 Free Article

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Select item 31019158

 

  1. Decompression of suprasystemic right ventricular pressure as palliative treatment for a child with tetralogy of Fallot.

Kuźma J, Skorek P, Pitak M, Konarska K, Weryński P.

Kardiol Pol. 2019 May 24;77(5):578-579. doi: 10.33963/KP.14849. Epub 2019 May 24. No abstract available.

PMID: 31125030 Free Article

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Select item 31123765

 

  1. New Strategies for the Conduct of Clinical Trials in Pediatric Pulmonary Arterial Hypertension: Outcome of a Multistakeholder Meeting With Patients, Academia, Industry, and Regulators, Held at the European Medicines Agency on Monday, June 12, 2017.

Ollivier C, Sun H, Amchin W, Beghetti M, Berger RMF, Breitenstein S, Garnett C, Gullberg N, Hassel P, Ivy D, Kawut SM, Klein A, Lesage C, Migdal M, Nije B, Odermarsky M, Strait J, de Graeff PA, Stockbridge N.

J Am Heart Assoc. 2019 May 21;8(10):e011306. doi: 10.1161/JAHA.118.011306. No abstract available.

PMID: 31088189 Free Article

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Select item 31152993

 

  1. Palliative care and paediatric cardiology: current evidence and future directions.

Morell E, Moynihan K, Wolfe J, Blume ED.

Lancet Child Adolesc Health. 2019 May 21. pii: S2352-4642(19)30121-X. doi: 10.1016/S2352-4642(19)30121-X. [Epub ahead of print] Erratum in: Lancet Child Adolesc Health. 2019 May 24;:.

PMID: 31126897

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Select item 31130326

 

  1. Familial aggregation of “apple peel” intestinal atresia and cardiac left-sided obstructive lesions: A possible causal relationship with NOTCH1 gene mutations.

Digilio MC, Magliozzi M, Di Pede A, Valfrè L, Dentici ML, Auriti C, Marino B, Novelli A, Dallapiccola B.

Am J Med Genet A. 2019 May 20. doi: 10.1002/ajmg.a.61195. [Epub ahead of print]

PMID: 31111652

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Select item 31111245

 

  1. HLA-C variants associated with amino acid substitutions in the peptide binding groove influence susceptibility to Kawasaki disease.

Shimizu C, Kim J, Eleftherohorinou H, Wright VJ, Hoang LT, Tremoulet AH, Franco A, Hibberd ML, Takahashi A, Kubo M, Ito K, Tanaka T, Onouchi Y, Coin LJM, Levin M, Burns JC, Shike H; International Kawasaki Disease Genetic Consortium.

Hum Immunol. 2019 May 20. pii: S0198-8859(18)31016-4. doi: 10.1016/j.humimm.2019.04.020. [Epub ahead of print]

PMID: 31122742

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Select item 31111603

 

  1. Differences in cerebral and muscle oxygenation patterns during exercise in children with univentricular heart after Fontan operation compared to healthy peers.

Vandekerckhove K, Coomans I, Moerman A, Panzer J, De Groote K, De Wilde H, Bove T, François K, De Wolf D, Boone J.

Int J Cardiol. 2019 May 18. pii: S0167-5273(18)35901-1. doi: 10.1016/j.ijcard.2019.05.040. [Epub ahead of print]

PMID: 31133431

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Select item 31113702

 

  1. Real-World Outcomes with Lomitapide Use in Paediatric Patients with Homozygous Familial Hypercholesterolaemia.

Ben-Omran T, Masana L, Kolovou G, Ariceta G, Nóvoa FJ, Lund AM, Bogsrud MP, Araujo M, Hussein O, Ibarretxe D, Sanchez-Hernández RM, Santos RD.

Adv Ther. 2019 May 17. doi: 10.1007/s12325-019-00985-8. [Epub ahead of print]

PMID: 31102204

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Select item 31043452

 

  1. The miRNA-608 rs4919510 G>C polymorphism confers reduce coronary injury of Kawasaki disease in a Southern Chinese population.

Wang Y, Lu Z, Fu L, Tan Y, Che D, Huang P, Pi L, Xu Y, Liang Q, Zhang L, Qiu X, Gu X.

Biosci Rep. 2019 May 17;39(5). pii: BSR20181660. doi: 10.1042/BSR20181660. Print 2019 May 31.

PMID: 31043452 Free PMC Article

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Select item 31097048

 

  1. Incomplete isolation of the left innominate artery from the right aortic arch: a rare case of double-outlet right ventricle with pulmonary stenosis.

Sato J, Kato A, Ohashi N.

Cardiol Young. 2019 May 16:1-3. doi: 10.1017/S1047951119000702. [Epub ahead of print]

PMID: 31094309

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Select item 31095787

 

  1. Lung Ultrasound to Assess the Etiology of Persistent Pulmonary Hypertension of the Newborn (LUPPHYN Study): A Pilot Study.

Del Rey Hurtado de Mendoza B, Sánchez-de-Toledo J, Bobillo Perez S, Girona M, Balaguer Gargallo M, Rodríguez-Fanjul J.

Neonatology. 2019 May 16:1-7. doi: 10.1159/000499047. [Epub ahead of print]

PMID: 31096216

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Select item 31098675

 

  1. Thenar Muscle Oxygen Saturation Using Vascular Occlusion Test: A Novel Technique to Study Microcirculatory Abnormalities in Pediatric Heart Failure Patients.

Samraj RS, Lopez-Colon D, Kerrigan M, Fricker FJ, Pietra BA, Bleiweis M, Gupta D.

Pediatr Cardiol. 2019 May 16. doi: 10.1007/s00246-019-02118-y. [Epub ahead of print]

PMID: 31098675

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Select item 31098798

 

  1. Posttraumatic stress and health-related quality of life in parents of children with cardiac rhythm devices.

Werner H, Balmer C, Lehmann P.

Qual Life Res. 2019 May 16. doi: 10.1007/s11136-019-02202-z. [Epub ahead of print]

PMID: 31098798

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Select item 30885417

 

  1. Useful Platelet Indices for the Diagnosis and Follow-Up of Patent Ductus Arteriosus.

Guler Kazanci E, Buyuktiryaki M, Unsal H, Tayman C.

Am J Perinatol. 2019 May 14. doi: 10.1055/s-0039-1688821. [Epub ahead of print]

PMID: 31087312

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Select item 31088126

 

  1. A Severe Form of Arterial Tortuosity Syndrome Presenting With Significant Airway Obstruction in an Infant.

Dodge-Khatami J, Simpson SA, Dodge-Khatami A.

World J Pediatr Congenit Heart Surg. 2019 May 14:2150135119829009. doi: 10.1177/2150135119829009. [Epub ahead of print]

PMID: 31088211

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Select item 31087377

 

  1. Right ventricular-vascular coupling ratio in pediatric pulmonary arterial hypertension: A comparison between cardiac magnetic resonance and right heart catheterization measurements.

Breeman KTN, Dufva M, Ploegstra MJ, Kheyfets V, Willems TP, Wigger J, Hunter KS, Ivy DD, Berger RMF, Truong U.

Int J Cardiol. 2019 May 10. pii: S0167-5273(19)31199-4. doi: 10.1016/j.ijcard.2019.05.021. [Epub ahead of print]

PMID: 31109778

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Select item 31077250

 

  1. Rapid Involution of Large Cardiac Rhabdomyomas With Everolimus Therapy.

Garg A, Gorla SR, Kardon RE, Swaminathan S.

World J Pediatr Congenit Heart Surg. 2019 May 9:2150135118822711. doi: 10.1177/2150135118822711. [Epub ahead of print]

PMID: 31072227

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Select item 31067002

 

  1. Green tea extract catechin improves cardiac function in pediatric cardiomyopathy patients with diastolic dysfunction.

Quan J, Jia Z, Lv T, Zhang L, Liu L, Pan B, Zhu J, Gelb IJ, Huang X, Tian J.

J Biomed Sci. 2019 May 8;26(1):32. doi: 10.1186/s12929-019-0528-7.

PMID: 31064352 Free PMC Article

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Select item 31057083

 

  1. Combination therapy for severe portopulmonary hypertension in a child allows for liver transplantation.

Serrano RM, Subbarao GC, Mangus RS, Montgomery G, Johansen M.

Pediatr Transplant. 2019 May 7:e13461. doi: 10.1111/petr.13461. [Epub ahead of print]

PMID: 31062925

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Select item 31065052

 

  1. Computed Tomography Angiography and Magnetic Resonance Angiography of Congenital Anomalies of Pulmonary Veins.

Abdel Razek AAK, Al-Marsafawy H, Elmansy M, El-Latif MA, Sobh D.

J Comput Assist Tomogr. 2019 May 6. doi: 10.1097/RCT.0000000000000857. [Epub ahead of print]

PMID: 31082974

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Select item 31058565

 

  1. Safety of enteral sildenafil in hemodynamically unstable children.

Bednarz AM, Israel EN, Beckman EJ, Johansen M, Thomas CA.

Cardiol Young. 2019 May 3:1-5. doi: 10.1017/S1047951119000209. [Epub ahead of print]

PMID: 31046848

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Select item 31058878

 

  1. Normal Values for Left Ventricular Strain and Synchrony in Children Based on Speckle Tracking Echocardiography.

Adar A, Ghelani SJ, Sleeper LA, Lu M, Marcus E, Ferraro AM, Colan SD, Banka P, Powell AJ, Harrild DM.

Am J Cardiol. 2019 May 1;123(9):1546-1554. doi: 10.1016/j.amjcard.2019.01.044. Epub 2019 Feb 8.

PMID: 30857642

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Select item 30797558

 

  1. Unguarded left atrioventricular orifice: An unusual cause of hypoplastic left ventricle and double-outlet right ventricle with intact ventricular septum.

Subramanian A, Bharath AP, Jayaranganath M.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):153-155. doi: 10.4103/apc.APC_124_18.

PMID: 31143045 Free PMC Article

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Select item 31143044

 

  1. Persistent great artery dilatation in Beals syndrome: A novel finding.

Siddiqui S, Panesar L.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):150-152. doi: 10.4103/apc.APC_8_19.

PMID: 31143044 Free PMC Article

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Select item 31143038

 

  1. The matter of “unbalance” in right dominant atrioventricular septal defect.

Lugones I, Biancolini MF, Lugones G, Biancolini JC, de Dios AM.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):132-134. doi: 10.4103/apc.APC_107_18.

PMID: 31143038 Free PMC Article

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Select item 31143037

 

  1. Isolation of the left brachiocephalic artery revisited: A 52-year literature review and introduction of a novel anatomic-clinical-prognostic classification.

Malakan Rad E, Pouraliakbar HR.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):117-129. doi: 10.4103/apc.APC_74_18. Review.

PMID: 31143036 Free PMC Article

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Select item 31143035

 

 

  1. Acetaminophen in low doses for closure of the ductus arteriosus of the premature.

Guimarães AFM, Araújo FDR, Meira ZMA, Tonelli HAF, Duarte GG, Ribeiro LC, Rezende GQM, Castilho SRT.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):97-102. doi: 10.4103/apc.APC_42_18.

PMID: 31143033 Free PMC Article

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Select item 31143030

 

  1. Giant Left Atrium in a 14-Month-Old Child With Compression of Airways.

Kar S, Joshi R, Joshi RK, Aggarwal N, Agarwal M.

Ann Thorac Surg. 2019 May;107(5):e311-e312. doi: 10.1016/j.athoracsur.2018.08.081. Epub 2018 Oct 22.

PMID: 30359594

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Select item 30359593

 

  1. Coronary artery aneurysms are more severe in infants than in older children with Kawasaki disease.

Cameron SA, Carr M, Pahl E, DeMarais N, Shulman ST, Rowley AH.

Arch Dis Child. 2019 May;104(5):451-455. doi: 10.1136/archdischild-2018-314967. Epub 2018 Nov 9.

PMID: 30413485

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Select item 29674517

 

  1. Tricky case of Takayasu arteritis in a young child presenting with heart failure and femoral pulses.

Fabi M, Brighenti M, Donti A, Lanari M.

Arch Dis Child. 2019 May;104(5):507. doi: 10.1136/archdischild-2017-314663. Epub 2018 Apr 19. No abstract available.

PMID: 29674517

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Select item 30798610

 

  1. Why do we break one of the first rules of anatomy when describing the components of the heart?

De Almeida MC, Spicer DE, Anderson RH.

Clin Anat. 2019 May;32(4):585-596. doi: 10.1002/ca.23356. Epub 2019 Mar 9. Review.

PMID: 30786071

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Select item 30868567

 

  1. Exercise stress echocardiography: Impact on clinical decision-making in pediatric patients.

Dasgupta S, Friedman H, Allen N, Stark M, Ferguson E, Sachdeva R, Border WL.

Echocardiography. 2019 May;36(5):938-943. doi: 10.1111/echo.14326. Epub 2019 Apr 1.

PMID: 30934142

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Select item 30801777

 

  1. Correction: Genetic evaluation of cardiomyopathy: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG).

Hershberger RE, Givertz MM, Ho CY, Judge DP, Kantor PF, McBride KL, Morales A, Taylor MRG, Vatta M, Ware SM; ACMG Professional Practice and Guidelines Committee.

Genet Med. 2019 May 1. doi: 10.1038/s41436-019-0521-2. [Epub ahead of print]

PMID: 31040388

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Select item 30287924

 

  1. Dynamic Three-Dimensional Geometry of the Tricuspid Valve Annulus in Hypoplastic Left Heart Syndrome with a Fontan Circulation.

Nguyen AV, Lasso A, Nam HH, Faerber J, Aly AH, Pouch AM, Scanlan AB, McGowan FX, Mercer-Rosa L, Cohen MS, Simpson J, Fichtinger G, Jolley MA.

J Am Soc Echocardiogr. 2019 May;32(5):655-666.e13. doi: 10.1016/j.echo.2019.01.002. Epub 2019 Feb 28.

PMID: 30826226

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Select item 30763159

 

  1. Pulmonary Hypertension Secondary to Scurvy in a Developmentally Typical Child.

Frank BS, Runciman M, Manning WA, Ivy DD, Abman SH, Howley L.

J Pediatr. 2019 May;208:291-291.e2. doi: 10.1016/j.jpeds.2018.12.068. Epub 2019 Feb 6. No abstract available.

PMID: 30738657

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Select item 30679055

 

  1. Congenital Portosystemic Shunts: Current Diagnosis and Management.

McLin VA, Franchi Abella S, Debray D, Guérin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E; Members of the International Registry of Congenital Porto-Systemic Shunts.

J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.

PMID: 30628988

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Select item 30608489

 

  1. Congenital Portosystemic Shunts: Current Diagnosis and Management.

McLin VA, Franchi Abella S, Debray D, Guérin F, Beghetti M, Savale L, Wildhaber BE, Gonzales E; Members of the International Registry of Congenital Porto-Systemic Shunts.

J Pediatr Gastroenterol Nutr. 2019 May;68(5):615-622. doi: 10.1097/MPG.0000000000002263.

PMID: 30628988

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Select item 30608489

 

  1. Role of echocardiography in the assessment of right ventricular function in the pediatric population.

Kamra K, Punn R.

Paediatr Anaesth. 2019 May;29(5):530-538. doi: 10.1111/pan.13641. Epub 2019 May 2.

PMID: 30934152

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Select item 30758099

 

  1. Current understanding and perioperative management of pediatric pulmonary hypertension.

Latham GJ, Yung D.

Paediatr Anaesth. 2019 May;29(5):441-456. doi: 10.1111/pan.13542. Epub 2018 Dec 19.

PMID: 30414333

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Select item 31058786

 

  1. Cardiac Manifestations of Noonan Syndrome.

Karnik R, Geiger M.

Pediatr Endocrinol Rev. 2019 May;16(Suppl 2):471-476. doi: 10.17458/per.vol16.2019.kpg.manifestationsnoonan.

PMID: 31115199

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Select item 30346369

 

  1. Clinical Characteristics of Infective Endocarditis in Children.

Kelchtermans J, Grossar L, Eyskens B, Cools B, Roggen M, Boshoff D, Louw J, Frerich S, Veloso TR, Claes J, Ditkowski B, Rega F, Meyns B, Gewillig M, Heying R.

Pediatr Infect Dis J. 2019 May;38(5):453-458. doi: 10.1097/INF.0000000000002212.

PMID: 30346369

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Select item 30281546

 

  1. PHACE syndrome with unnoticeable skin lesion and rare anomaly of coronary artery.

Sekioka A, Fukumoto K, Horikoshi Y, Nii M, Urushihara N.

Pediatr Int. 2019 May;61(5):524-526. doi: 10.1111/ped.13841. Epub 2019 May 15. No abstract available.

PMID: 31090165

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Select item 31087585

 

  1. Idiopathic, heritable and veno-occlusive pulmonary arterial hypertension in childhood: computed tomography angiography features in the initial assessment of the disease.

Berteloot L, Proisy M, Jais JP, Lévy M, Boddaert N, Bonnet D, Raimondi F.

Pediatr Radiol. 2019 May;49(5):575-585. doi: 10.1007/s00247-018-04331-y. Epub 2019 Jan 16.

PMID: 30652195

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Select item 30795005

 

  1. Baroreceptor activity and sensitivity: normal values in children and young adults using the head up tilt test.

Alnoor MS, Varner HK, Butler IJ, Zhu L, Numan MT.

Pediatr Res. 2019 May;85(6):841-847. doi: 10.1038/s41390-019-0327-6. Epub 2019 Feb 6.

PMID: 30728445

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Select item 30723312

 

  1. Research Gaps in Primary Pediatric Hypertension.

Taylor-Zapata P, Baker-Smith CM, Burckart G, Daniels SR, Flynn JT, Giacoia G, Green D, Kelly AS, Khurana M, Li JS, Pratt C, Urbina EM, Zajicek A.

Pediatrics. 2019 May;143(5). pii: e20183517. doi: 10.1542/peds.2018-3517.

PMID: 31023830

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  1. Oxytocin antagonism prevents pregnancy-associated aortic dissection in a mouse model of Marfan syndrome.

Habashi JP, MacFarlane EG, Bagirzadeh R, Bowen C, Huso N, Chen Y, Bedja D, Creamer TJ, Rykiel G, Manning M, Huso D, Dietz HC.

Sci Transl Med. 2019 May 1;11(490). pii: eaat4822. doi: 10.1126/scitranslmed.aat4822.

PMID: 31043570

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Select item 30861550

 

  1. Late presentation of iatrogenic diversion of inferior vena cava to the left atrium.

Kumar R, Munirathinam GK, Raja J, Srivastava A, Mishra AK, Singh RS, Thingnam SKS.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):188-189. doi: 10.4103/apc.APC_98_18. No abstract available.

PMID: 31143056 Free PMC Article

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  1. Truncus arteriosus associated with double aortic arch in a patient with DiGeorge syndrome: A rare case report.

Moghadam EA, Mirzaaghayan MR, Zeinaloo A, Mohebbi A, Ghamari A.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):185-186. doi: 10.4103/apc.APC_80_18. No abstract available.

PMID: 31143054 Free PMC Article

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  1. Pulmonary artery from the left main coronary artery.

Bansal R, Ramakrishnan S, Verma SK.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):176-177. doi: 10.4103/apc.APC_163_18.

PMID: 31143051 Free PMC Article

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  1. Isolated left common carotid artery in an infant with pulmonary atresia and intact ventricular septum.

Cohen JL, Stanford N, Torres A.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):156-158. doi: 10.4103/apc.APC_75_18.

PMID: 31143046 Free PMC Article

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  1. Systemic arterial aneurysm complicated by thrombosis in an infant with resistant Kawasaki disease.

Ozdemir E, Peterson RE.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):147-149. doi: 10.4103/apc.APC_73_18.

PMID: 31143043 Free PMC Article

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  1. “Double-lumen” aortic arch with “double-lumen” brachiocephalic artery.

Rajagopal R, Garg PK, Khera PS, Sharma S.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):141-143. doi: 10.4103/apc.APC_106_18.

PMID: 31143041 Free PMC Article

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  1. Isolated agenesis of the right pulmonary veins with pulmonary sequestration.

Patil S, Sachdeva S, Bakhru S, Dalvi B, Koneti NR.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):135-137. doi: 10.4103/apc.APC_55_18.

PMID: 31143039 Free PMC Article

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  1. The matter of “unbalance” in right dominant atrioventricular septal defect.

Lugones I, Biancolini MF, Lugones G, Biancolini JC, de Dios AM.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):132-134. doi: 10.4103/apc.APC_107_18.

PMID: 31143038 Free PMC Article

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  1. Prevalence of hypertension and prehypertension in schoolchildren from Central India.

Patel A, Bharani A, Sharma M, Bhagwat A, Ganguli N, Chouhan DS.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):90-96. doi: 10.4103/apc.APC_13_18.

PMID: 31143032 Free PMC Article

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Select item 31143031

 

  1. Modulation of plasma triglycerides concentration by sterol-based treatment in children carrying different genes.

San Mauro Martín I, Sanz Rojo S, Garicano Vilar E, Collado Yurrita L, Blumenfeld Olivares JA.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):83-89. doi: 10.4103/apc.APC_86_18.

PMID: 31143031 Free PMC Article

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  1. High blood pressure in children: The invisible dragon.

Sivasankaran S.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):73-76. doi: 10.4103/apc.APC_46_19. No abstract available.

PMID: 31143029 Free PMC Article

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ACHD Featured Articles in association with ISACHD

ACHD Featured Manuscripts of May 2019 (sponsored by ISACHD)

Elevated ventricular filling pressures and long-term survival in adults post-Fontan.

Miranda WR, Hagler DJ, Taggart NW, Borlaug BA, Connolly HM, Egbe AC.

Catheter Cardiovasc Interv. 2019 May 30. doi: 10.1002/ccd.28340. [Epub ahead of print]

PMID:  31145542

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Take Home Points: 

  • Though elevated VEDP portends a poor long-term prognosis, PAWP was better in predicting mortality amongst the many other factors we assess in Fontan patients. However, if it is normal, we cannot necessarily be reassured.

Commentary by Dr. Helen Parry (Leeds, United Kingdom), section editor of ACHD Journal Watch:  Diastolic dysfunction is part of the natural history of patients with Fontan palliations – ventricular end diastolic pressures (VEDP) and pulmonary capillary wedge pressure (PCW) serve as surrogates for left atrial pressures (LAP). Data pertaining to the haemodynamic predictors of survival in this cohort are limited.

This study from the Mayo clinic, enrolled 148 adult patients with Fontan circulation who underwent cardiac catheterisation within an 18-year period (December 1999 to November 2017). Patients with atrioventricular prostheses were excluded. Findings with respect to ventricular end diastolic pressure and pulmonary artery wedge pressure were correlated with mortality.

Mean age at the time of Fontan was 10.1±8.1 years. The mean ventricular ejection fraction was 51.1% and the atrioventricular regurgitation was more than moderate in 24 patients (16.2%). Mean VEDP was 11.5 ±4.7mmHg and PAWP 10.6 ± 4.5 mmHg.

PAWP correlated better with Fontan pressures compared to VEDP (correlation coefficient 0.82 vs 0.63, p<0.0001).

 

Patients were followed up for a mean of 6.0±4.8 years. A total of 45 deaths occurred during this period (30.4%). Survival was greater in those with a VEDP <12 compared to a VEDP >12 mmHg (p=0.02). Similarly this held true for patients with a PCWP using a cut-off of 12mmHg. There was no survival difference in those patients with a VEDP > 12 mmHg vs VEDP <12mmHg if the PCWP was <12mmHg.

 

 

Pulmonary artery wedge pressure was a better predictor of mortality than ventricular end diastolic pressure.

Positive aspects of the study:

  • Large sample size for a Fontan study
  • Reproducible methodology
  • Offers a tool for helping to predict outcome in Fontan patients

Negative aspects of the study:

  • The team point out that many patients who died after the catheter study did not have raised PAWP. This was far in excess of what would be expected for background, non-Fontan related deaths.
  • No potential measures are suggested to prevent deterioration.

 

 

Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome.

Cauldwell M, Steer PJ, Curtis SL, Mohan A, Dockree S, Mackillop L, Parry HM, Oliver J, Sterrenberg M, Wallace S, Malin G, Partridge G, Freeman LJ, Bolger AP, Siddiqui F, Wilson D, Simpson M, Walker N, Hodson K, Thomas K, Bredaki F, Mercaldi R, Walker F, Johnson MR.

Heart. 2019 May 25. pii: heartjnl-2019-314817. doi: 10.1136/heartjnl-2019-314817. [Epub ahead of print]

PMID: 31129614

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Select item 31126344

 

Take Home Points: 

  • No deaths occurred in this cohort of 258 pregnancies in 151 Marfan patients
  • The aortic dissection rate was 1.9% (mainly type B)
  • No specific clinical or imaging predictors of dissection were identified
  • A significant but small increase in aortic root diameters was noted following pregnancy
  • Neonates of those on betablocker therapy had lower birth weights
  • The elective Caesarian section rate was higher in patients with dilated aortic roots
  • Post-partum haemorrhage complicated 40% of deliveries (double that of the general population).

Commentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal Watch:

Marfan syndrome is associated with an increase in cardiovascular mortality, especially relating to aortic dissection. Pregnancy is thought to increase the risk of aortic dissection but previous studies were small and variable in its descriptions of specific complications. There is no good data to show that betablocker use reduces the risk of aortic dissection in pregnancy.

This retrospective observational study was conducted at 12 UK centers with the aim of gaining information to help direct counselling and management of Marfan Syndrome in pregnancy. Patients delivering between January 1998 and March 2018 were enrolled and data pertaining to maternal and neonatal outcomes recorded.

The primary outcome was the occurrence of aortic dissection during pregnancy or up to 6 months post-partum. The secondary cardiac outcomes were cardiac surgery or stroke. Obstetric outcomes (gestational hypertension, pre-eclampsia, preterm delivery, postpartum haemorrhage and venous thromboembolism) and neonatal outcomes (small for gestational age, stillbirth and neonatal unit admission) were recorded.

Two hundred and fifty-eight pregnancies occurred in 151 patients. These included 226 pregnancies >24 weeks gestation, 20 miscarriages and 12 terminations.  There were 213 live births. Thirty-five pregnancies occurred in 21 women with previous ascending aorta replacements. Only 47.8% of women had preconception counseling in their first pregnancy. The median age at diagnosis was 14.5 years and the median age at pregnancy 29 years. Most patients (80%) had an aortic root diameter of <40mm on echo pre-conception.

Five patients had an aortic dissection during pregnancy (1 type A, 4 type B). No specific predictors for aortic dissection were identified, but the sample size was small and moderate associations could therefore not be excluded. 6 Cardiac surgeries and 2 strokes occurred during the pregnancies and post-partum period.

After excluding women with prior aortic root replacements, echo data pre- and post-pregnancy was available in 58 women. The median aortic root size pre-pregnancy was 36mm and 37mm post pregnancy – a small but significant increase (p=0.01).  This change was not significantly modified by the use of betablockers.

 

There was no correlation between aortic root size and the time of delivery. However, in patients with a maximum aortic root diameter of <40mm, the caesarian section rate was 26% compared to 70.4% in those with a root diameter > 40mm (p<0.001).

Betablocker use was documented on 64.2% of pregnancies and was more likely to be prescribed in women with larger aortic roots. The median birth weight was significantly reduced in women on betablockers compared to those who were not taking it (3024 g vs 3310 g, p=0.002).

 

40% of women experienced post-partum haemmorhage – this is double the rate of the general population.

 

The Prognostic Value of Myocardial Deformation in Adult Patients With Corrected Tetralogy of Fallot.

van Grootel RWJ, van den Bosch AE, Baggen VJM, Menting ME, Baart SJ, Cuypers JAAE, Witsenburg M, Roos-Hesselink JW.

J Am Soc Echocardiogr. 2019 Jul;32(7):866-875.e2. doi: 10.1016/j.echo.2019.03.014. Epub 2019 May 4.

PMID:  31064677

Similar articles

 

Take Home Points:

  • There is a clear role for strain analysis in adult patients with repaired ToF.
  • Reduced myocardial deformation parameters, including RV longitudinal strain and LV apical rotation, are associated with worse outcomes in clinically stable patients with repaired ToF, independent of currently known markers.
  • To enhance risk stratification, myocardial deformation imaging should be considered as an additional tool in routine clinical follow-up, but not to replace currently used strategies.

Commentary by Dr. Soha Romeih (Aswan, Egypt), section editor of ACHD Journal Watch:

van Grootel et al studied the myocardial deformation by echocardiography speckle-tracking analysis of both ventricles in asymptomatic adults with repaired ToF and its relationship with adverse outcome. This is a prospective observational study that included all clinically stable patients with repaired ToF, visiting the outpatient clinic between 2011 and 2013. Exclusion criteria were age < 18 years, pregnancy, and inability to understand and sign informed consent forms; patients were also excluded in case of poor echocardiographic image quality.

  • Myocardial strain analysis included:

1- The left ventricle was assessed according to the 17-segment model as stated in the guidelines for echocardiographic chamber quantification. The rotation at the basal and apical levels of the left ventricle was measured by applying speckle-tracking after identifying the endo- and epicardial borders in the basal and apical short-axis views.

2- RV systolic function was assessed by measuring RV longitudinal strain (LS) of the free wall. The software describes clockwise rotation as a positive value and counterclockwise rotation as a negative value. The peak apical rotation and basal rotation during systole was assessed. Twist was calculated and defined as the maximum value of the simultaneous difference between apical rotation and basal rotation (apical minus basal rotation) during systole.

Strain measurements were blinded.

  • Definition and assessment of events: The primary endpoint was a composite of death or heart failure. Heart failure was defined as requiring changes in HF medication or hospital admission. The secondary endpoint was a supraventricular or ventricular arrhythmia (symptomatic and recorded or treated), hospitalization for cardiac reasons, or reintervention (both surgical and percutaneous). End points were manually tracked on a yearly basis. 

Results:

  • In total, 179 patients were eligible in the original cohort. Of these, 28 (16%) were excluded because of poor echocardiographic image quality. Of the 151 patients included, the median age was 34.6 years [IQR, 27.9–44.9 years] and  1% were men. The control group of 88 subjects had a median age of 31.2 years (IQR, 25.3–41.9 years), and 48 (54.5%) were men.
  • All patients had significantly lower myocardial deformation compared to controls (Figure 1)

  • The patients with decreased LV GLS had larger LV end-systolic dimensions and volumes and lower LVEFs. RV free wall strain was significantly lower in patients with reduced LV GLS. (ventricular-ventricular interaction) There were no differences in functional valvular status.

 

  • The primary and secondary endpoints occurred in 14 (9.3%) and 62 (41.1%) patients, respectively. Baseline RV free wall LS and LV GLS were significantly associated with the primary endpoint and also with the secondary endpoint.
  • Survival was significantly better in patients with higher strain values with regard to both the primary and secondary endpoints. (Figure 2)

 

Conclusion:

This study shows that myocardial deformation is important in determining prognosis in patients with repaired ToF.  Baseline longitudinal strain of either the left or right ventricle was significantly lower in patients who died or developed heart failure.

Strikingly, apical rotation and NYHA functional class II were the only variables that were independently associated with the secondary endpoint in the multivariate model after correction for several clinical and echocardiographic variables known to be associated with outcome.

Limitations:

As mentioned in the article being as single center study, may have led to inclusion bias and limited numbers.

Moreover, comparing echocardiography myocardial deformation mentioned in the current study would be better compared with well-studied parameters from MRI as ventricular volumes and functions particularly for RV.

Also, though patients claim to be “asymptomatic” , many may lead a sedentary lifestyle – exercise capacity measured by exercise test parameters such  as METS and VO2 max would therefore be of greater help to correlate with myocardial deformation.

 

Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital Heart Disease.

Lluri G, Lin J, Reardon L, Miner P, Whalen K, Aboulhosn J.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):292-295. doi: 10.1177/2150135119825599.

PMID:  31084317

Similar articles 

Take Home Points:

  1. Congestive heart failure due to systolic heart dysfunction in ACHD patients is a common cause of early death and disability. Many medications in the general population have copious evidence of improving outcomes in the general CHF population; however ACHD has very little evidence of any drugs working due to patient heterogeneity, small sample sizes, limited follow-up, and other issues that make a well-designed clinical trial very hard to execute.
  2. Sacubitril/valsartan is a newer FDA-approved drug in a new class known as an ARNI. It combines an angiotensin II receptor blocker (ARB) with a neprilysin inhibitor (NI). This combination affects both the renin-angiotensin-aldosterone system (RAAS) and the natriuretic peptide (NP) systems.  This drug showed ~20% reduction in mortality compared to standard therapy in the PARADIGM-HF trial, with over 8000 patients randomized.
  3. There is limited evidence that drugs affecting the NP system may improve symptoms in patients with pulmonary hypertension and/or systemic right ventricles, which are both commonly encountered in ACHD.
  4. To date, there has been very little study of ARNI in ACHD. This paper is the 2nd of 3 published to evaluate this topic.  This paper had 15 patients; the first paper had 4 and the most recent 23 patients under study.
  5. The study was underpowered to show statistically significant results but did demonstrate anecdotal improvement in 4/5 patients with NYHA class III symptoms and also only 1/15 (6.6%) had to stop the medication (the rate of stopping the medication was 10.7% in PARADIGM-HF)

Commentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch: 

Although most congenital defects can be repaired with excellent survival to adulthood, congestive heart failure due to significant systolic dysfunction is often a common final pathway leading to early disability and death in ACHD patients.  Sacubitril/valsartan, a unique drug in a novel class known as ARNIs (angiotensin receptor inhibitor combined with a neprilysin inhibitor) has recently entered the armamentarium of heart failure drugs, and although there hasn’t been a lot of long-term experience with it, it has been studied in the largest heart failure trial ever, in terms of sheer volume of patients, in the PARADIGM-HF trial, which randomized over 8000 patients to ARNI versus enalapril.  That trial, in the general non-ACHD population, showed significant superiority of ARNI over enalapril; however to date there has been very little formal study of ARNI usage and outcomes in the ACHD population.

This small retrospective review coming from University of California Los Angeles (UCLA) shows the entirety of their first year experience using sacubitril/valsartan. Patients included a variety of anatomic congenital heart diagnoses (2 Fontans, the rest with 2-ventricle repairs); all patients had either NYHA II or NYHA III symptoms at baseline.  Of the 15 total patients on the medication, 14 were able to tolerate it (one stopped due to renal failure).  In the PARADIGM-HF trial, ~10.7% of patients had to stop ARNI due to side effects, most commonly hypotension and hyperkalemia.

The number of patients was very low, in one of the largest ACHD centers in the United States, which likely reflects the ACHD community’s general reluctance to try new drugs until they have been proven safe in the larger non-CHD community, as well as issues of expense – most ACE inhibitors are available even without insurance for $4 a month, whereas sacubitril/valsartan costs more than $1000 a month without insurance.

The study presented here was retrospective, and too small to have adequate power to draw meaningful statistical conclusions.  However, it is notable that that 4/5 patients with NYHA class III CHF symptoms had improvement in their degree of heart failure symptoms.  Mechanistically, it stands to reason that patients with 2-ventricle repairs and a subaortic left ventricle would be similar to the general heart failure population studied in PARADIGM-HF; however ACHD has been plagued by lack of large enough trials to prove or disprove that ACE inhibitors, ARBs, and other heart failure drugs make a significant difference in those with systemic right ventricles or single ventricles.

This study is quite interesting, and perhaps when the price of sacubitril/valsartan comes down more it will be adopted more commonly in ACHD versus cheaper alternatives.  Given the lack of evidence of heart failure drugs in general in ACHD, as well as the robustness that ARNI appears to be superior to ACE in PARADIGM-HF, the authors appropriately call for a large multicenter trial by the US/Canada -based Alliance for Adult Research in Congenital Cardiology (AARCC) to be performed on a prospective basis, as we have a multitude of ACHD patients suffering premature death due to systolic ventricular dysfunction despite good repairs as children, thus more evidence that ARNI can alleviate that burden will be very helpful.

 

 

 

ACHD May 2019

  1. Incidence and mortality of brain abscess in Denmark: A nationwide population-based study.

Bodilsen J, Dalager-Pedersen M, van de Beek D, Brouwer MC, Nielsen H.

Clin Microbiol Infect. 2019 May 31. pii: S1198-743X(19)30279-4. doi: 10.1016/j.cmi.2019.05.016. [Epub ahead of print]

PMID: 31158518

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Select item 31160230

 

  1. Development of a System to Measure and Improve Outcomes in Congenital Heart Disease: Heart Institute Safety, Quality, and Value Program.

Clarke-Myers K, Cooper DS, Hanke SP, Storey J, McDonald M, Kimball TR, Anderson JB.

Jt Comm J Qual Patient Saf. 2019 May 31. pii: S1553-7250(18)30536-1. doi: 10.1016/j.jcjq.2019.04.003. [Epub ahead of print]

PMID: 31160230

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Select item 31162372

 

  1. Age- and Sex-Related Aortic Valve Dysfunction and Aortopathy Difference in Patients with Bicuspid Aortic Valve.

Ren X, Li F, Wang C, Hou Z, Gao Y, Yin W, Lu B.

Int Heart J. 2019 May 30;60(3):637-642. doi: 10.1536/ihj.18-363. Epub 2019 May 17.

PMID: 31105146 Free Article

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Select item 31152730

 

  1. Pathophysiology and management of combined aortic and mitral regurgitation.

Unger P, Lancellotti P, Amzulescu M, David-Cojocariu A, de Cannière D.

Arch Cardiovasc Dis. 2019 May 29. pii: S1875-2136(19)30096-8. doi: 10.1016/j.acvd.2019.04.003. [Epub ahead of print] Review.

PMID: 31153874

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Select item 31142541

 

  1. Cardiovascular risk factors and arterial thrombotic events in congenital heart disease patients.

Martínez-Quintana E, Rodríguez-Hernández JL, Rodríguez-González F, Riaño-Ruiz M, Fraguela-Medina C, Girolimetti A, Jiménez-Rodríguez S.

Int J Clin Pract. 2019 May 29:e13378. doi: 10.1111/ijcp.13378. [Epub ahead of print]

PMID: 31141298

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Select item 31141592

 

  1. Open-Source Routines for Building Personalized Left Ventricular Models from Cardiac MRI Data.

Phung TN, Waters C, Holmes JW.

J Biomech Eng. 2019 May 29. doi: 10.1115/1.4043876. [Epub ahead of print]

PMID: 31141592

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Select item 31141490

 

  1. Quality of life research in adult patients with congenital heart disease: current status and the way forward.

Moons P, Luyckx K.

Acta Paediatr. 2019 May 28. doi: 10.1111/apa.14876. [Epub ahead of print] Review.

PMID: 31136004

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Select item 31139902

 

  1. Brain vascular changes in adults with congenital heart disease: A systematic review.

Melazzini L, Codari M, Vitali P, Sardanelli F.

Neuroimage Clin. 2019 May 27;23:101873. doi: 10.1016/j.nicl.2019.101873. [Epub ahead of print] Review.

PMID: 31158693 Free PMC Article

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Select item 31131680

 

  1. Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome.

Cauldwell M, Steer PJ, Curtis SL, Mohan A, Dockree S, Mackillop L, Parry HM, Oliver J, Sterrenberg M, Wallace S, Malin G, Partridge G, Freeman LJ, Bolger AP, Siddiqui F, Wilson D, Simpson M, Walker N, Hodson K, Thomas K, Bredaki F, Mercaldi R, Walker F, Johnson MR.

Heart. 2019 May 25. pii: heartjnl-2019-314817. doi: 10.1136/heartjnl-2019-314817. [Epub ahead of print]

PMID: 31129614

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Select item 31126344

 

  1. Long-term health-related burden of adult congenital heart diseases in Hong Kong.

Lee VWY, Yan BP, Fong TMC, Fung AKP, Cheng FWT.

J Med Econ. 2019 May 23:1-4. doi: 10.1080/13696998.2019.1613239. [Epub ahead of print]

PMID: 31038380

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Select item 31021079

 

  1. Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.

El Dick J, El-Rassi I, Tayeh C, Bitar F, Arabi M.

Echocardiography. 2019 May 22. doi: 10.1111/echo.14368. [Epub ahead of print] Review.

PMID: 31116466

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Select item 31119575

 

  1. Persistent left superior vena cava draining into the left superior pulmonary vein in a scuba diver: a case report and literature study.

Blokland D, Lentjes GW, Velthuis BK, Chamuleau SAJ, Rienks R.

Scand J Med Sci Sports. 2019 May 20. doi: 10.1111/sms.13471. [Epub ahead of print]

PMID: 31111567

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Select item 31106773

 

  1. Cardiovascular risk factors in adults with coarctation of the aorta.

Fedchenko M, Mandalenakis Z, Dellborg H, Hultsberg-Olsson G, Björk A, Eriksson P, Dellborg M.

Congenit Heart Dis. 2019 May 17. doi: 10.1111/chd.12785. [Epub ahead of print]

PMID: 31099471

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Select item 31157192

 

  1. An overview of the molecular mechanisms underlying development and progression of bicuspid aortic valve disease.

Balistreri CR, Forte M, Greco E, Paneni F, Cavarretta E, Frati G, Sciarretta S.

J Mol Cell Cardiol. 2019 May 17;132:146-153. doi: 10.1016/j.yjmcc.2019.05.013. [Epub ahead of print] Review.

PMID: 31103478

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Select item 31094706

 

  1. Lifelong burden of small unrepaired atrial septal defect: Results from the Danish National Patient Registry.

Udholm S, Nyboe C, Karunanithi Z, Christensen AI, Redington A, Nielsen-Kudsk JE, Hjortdal VE.

Int J Cardiol. 2019 May 15;283:101-106. doi: 10.1016/j.ijcard.2019.02.024. Epub 2019 Feb 16.

PMID: 30826194

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Select item 30616811

 

  1. Impact of a centre and home-based cardiac rehabilitation program on the quality of life of teenagers and young adults with congenital heart disease: The QUALI-REHAB study rationale, design and methods.

Amedro P, Gavotto A, Legendre A, Lavastre K, Bredy C, De La Villeon G, Matecki S, Vandenberghe D, Ladeveze M, Bajolle F, Bosser G, Bouvaist H, Brosset P, Cohen L, Cohen S, Corone S, Dauphin C, Dulac Y, Hascoet S, Iriart X, Ladouceur M, Mace L, Neagu OA, Ovaert C, Picot MC, Poirette L, Sidney F, Soullier C, Thambo JB, Combes N, Bonnet D, Guillaumont S.

Int J Cardiol. 2019 May 15;283:112-118. doi: 10.1016/j.ijcard.2018.12.050. Epub 2018 Dec 20.

PMID: 30616811

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Select item 31090489

 

  1. Challenges of the Transition: A Case of Noncompliance in an Adult with Congenital Heart Disease.

Jersak T, Gustin J, Humphrey L.

J Palliat Med. 2019 May 15. doi: 10.1089/jpm.2018.0467. [Epub ahead of print]

PMID: 31090489

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Select item 31090912

 

  1. Coronary artery disease in adults with tetralogy of Fallot.

Egbe AC, Ananthaneni S, Jadav R, Kothapalli S, Rihal CS, Masood M, Angirekula M, Najam M, Bajwa N, Tarek K, Matthew J, Connolly HM.

Congenit Heart Dis. 2019 May 13. doi: 10.1111/chd.12782. [Epub ahead of print]

PMID: 31083772

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Select item 31026813

 

  1. Impact of Pregnancy on Aortic Root in Women with Repaired Conotruncal Anomalies.

Horiuchi C, Kamiya CA, Ohuchi H, Nakanishi A, Tsuritani M, Iwanaga N, Kurosaki K, Niwa K, Ikeda T, Yoshimatsu J.

Pediatr Cardiol. 2019 May 13. doi: 10.1007/s00246-019-02112-4. [Epub ahead of print]

PMID: 31087143

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Select item 31087396

 

  1. Large unrepaired aortopulmonary window presenting in adulthood.

Dev M, Sharma M, Rana N.

Curr Cardiol Rev. 2019 May 12. doi: 10.2174/1573403X15666190513105231. [Epub ahead of print]

PMID: 31092183

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Select item 31081250

 

  1. Pregnancy outcomes in mixed connective tissue disease: a multicentre study.

Radin M, Schreiber K, Cuadrado MJ, Cecchi I, Andreoli L, Franceschini F, Caleiro T, Andrade D, Gibbone E, Khamashta M, Buyon J, Izmirly P, Aguirre MA, Benedetto C, Roccatello D, Marozio L, Sciascia S.

Rheumatology (Oxford). 2019 May 11. pii: kez141. doi: 10.1093/rheumatology/kez141. [Epub ahead of print]

PMID: 31079145

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Select item 31015364

 

  1. Effect and safety of treatment with ACE-inhibitor Enalapril and β-blocker metoprolol on the onset of left ventricular dysfunction in Duchenne muscular dystrophy – a randomized, double-blind, placebo-controlled trial.

Dittrich S, Graf E, Trollmann R, Neudorf U, Schara U, Heilmann A, von der Hagen M, Stiller B, Kirschner J, Pozza RD, Müller-Felber W, Weiss K, von Au K, Khalil M, Motz R, Korenke C, Lange M, Wilichowski E, Pattathu J, Ebinger F, Wiechmann N, Schröder R; German Competence Network for Congenital Heart Defects and the Treat-NMD Neuromuscular Network Investigators list of additional local Investigators and co-workers of the German Competence Network for Congenital Heart Defects and the Treat-NMD Neuromuscular Network.

Orphanet J Rare Dis. 2019 May 10;14(1):105. doi: 10.1186/s13023-019-1066-9.

PMID: 31077250 Free PMC Article

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Select item 31030447

 

  1. The role of echocardiography for quantitative assessment of right ventricular size and function in adults with repaired tetralogy of Fallot.

Egbe AC, Pislaru SV, Kothapalli S, Jadav R, Masood M, Angirekula M, Pellikka PA.

Congenit Heart Dis. 2019 May 9. doi: 10.1111/chd.12783. [Epub ahead of print]

PMID: 31070871

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Select item 31070866

 

  1. Clinic nonattendance is associated with increased emergency department visits in adults with congenital heart disease.

Awh K, Venuti MA, Gleason LP, Rogers R, Denduluri S, Kim YY.

Congenit Heart Dis. 2019 May 9. doi: 10.1111/chd.12784. [Epub ahead of print]

PMID: 31070866

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Select item 31070245

 

  1. Hippocampal alterations and functional correlates in adolescents and young adults with congenital heart disease.

Fontes K, Rohlicek CV, Saint-Martin C, Gilbert G, Easson K, Majnemer A, Marelli A, Chakravarty MM, Brossard-Racine M.

Hum Brain Mapp. 2019 May 9. doi: 10.1002/hbm.24615. [Epub ahead of print]

PMID: 31070841

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Select item 31069392

 

  1. Lost in the system? Transfer to adult congenital heart disease care-Challenges and solutions.

Gerardin J, Raskind-Hood C, Rodriguez FH 3rd, Hoffman T, Kalogeropoulos A, Hogue C, Book W.

Congenit Heart Dis. 2019 May 8. doi: 10.1111/chd.12780. [Epub ahead of print]

PMID: 31066199

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Select item 31066183

 

  1. Elevated plasma Pim-1 and its clinical significance in patients with pulmonary arterial hypertension.

Zhu T, Luo J, Wang Y, Xiong X, Sheng B, Yang X, Ndongala NAMT, Li J.

Clin Exp Pharmacol Physiol. 2019 May 7. doi: 10.1111/1440-1681.13102. [Epub ahead of print]

PMID: 31066078

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Select item 31134172

 

  1. Adverse Delivery Events in Pregnant Women With Congenital Heart Defects: Wish You an Easy Delivery.

Bhatt AB.

J Am Coll Cardiol. 2019 May 7;73(17):2192-2194. doi: 10.1016/j.jacc.2019.02.038. No abstract available.

PMID: 31047007

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Select item 31047006

 

  1. Maternal Comorbidities and Complications of Delivery in Pregnant Women With Congenital Heart Disease.

Schlichting LE, Insaf TZ, Zaidi AN, Lui GK, Van Zutphen AR.

J Am Coll Cardiol. 2019 May 7;73(17):2181-2191. doi: 10.1016/j.jacc.2019.01.069.

PMID: 31047006 Free Article

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Select item 31039662

 

  1. External Validation of 3 Risk Scores in Adults with Congenital Heart Disease.

Ramchandani BK, Polo L, Sánchez R, Rey J, González Á, Díez J, Aroca Á.

Korean Circ J. 2019 May 7. doi: 10.4070/kcj.2019.0015. [Epub ahead of print]

PMID: 31165593 Free Article

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Select item 30020660

 

  1. Eisenmenger Syndrome.

Basit H, Wallen TJ, Sergent BN.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.

2019 May 6.

PMID: 29939577 Free Books & Documents

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Select item 31058441

 

  1. Use of Continuous Dexmedetomidine at Home for Nearly Three Years in a Young Woman with End-Stage Cyanotic Congenital Heart Disease and Cyclic Vomiting.

Thienprayoon R, Meyer M, Flint H, Weidner N, Hirsch R.

J Palliat Med. 2019 May 6. doi: 10.1089/jpm.2018.0516. [Epub ahead of print]

PMID: 31058565

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Select item 30252317

 

  1. Too little too late? Communication with patients with congenital heart disease about challenges of adult life.

Deng LX, Gleason LP, Awh K, Khan AM, Drajpuch D, Fuller S, Goldberg LA, Mascio CE, Partington SL, Tobin L, Kovacs AH, Kim YY.

Congenit Heart Dis. 2019 May 4. doi: 10.1111/chd.12778. [Epub ahead of print]

PMID: 31054179

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Select item 31085083

 

  1. Does gender affect the prognosis and risk of complications in patients with congenital heart disease in the modern era?

D’Alto M, Budts W, Diller GP, Mulder B, Egidy Assenza G, Oreto L, Ciliberti P, Bassareo PP, Gatzoulis MA, Dimopoulos K.

Int J Cardiol. 2019 May 4. pii: S0167-5273(19)30763-6. doi: 10.1016/j.ijcard.2019.05.010. [Epub ahead of print]

PMID: 31085083

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Select item 31060207

 

  1. The Prognostic Value of Myocardial Deformation in Adult Patients With Corrected Tetralogy of Fallot.

van Grootel RWJ, van den Bosch AE, Baggen VJM, Menting ME, Baart SJ, Cuypers JAAE, Witsenburg M, Roos-Hesselink JW.

J Am Soc Echocardiogr. 2019 May 4. pii: S0894-7317(19)30155-5. doi: 10.1016/j.echo.2019.03.014. [Epub ahead of print]

PMID: 31064677

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Select item 31046858

 

  1. Real-world healthcare utilization in adult congenital heart disease: a systematic review of trends and ratios.

Willems R, Werbrouck A, De Backer J, Annemans L.

Cardiol Young. 2019 May 3:1-11. doi: 10.1017/S1047951119000441. [Epub ahead of print]

PMID: 31046858

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Select item 31051468

 

  1. Association of Adult Congenital Heart Disease With Pregnancy, Maternal, and Neonatal Outcomes.

Ramage K, Grabowska K, Silversides C, Quan H, Metcalfe A.

JAMA Netw Open. 2019 May 3;2(5):e193667. doi: 10.1001/jamanetworkopen.2019.3667.

PMID: 31074818 Free PMC Article

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Select item 31053163

 

  1. Tissue oxygen saturation assessment of microvascular perfusion in adults with fontan palliation and comparator groups using vascular optical spectrophotometry – a pilot study.

Agarwal R, Chaudhry B, Jansen K, O’Sullivan J, Hudson M, Allen J, Coats L.

Physiol Meas. 2019 May 3. doi: 10.1088/1361-6579/ab1f1c. [Epub ahead of print]

PMID: 31051474

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Select item 31044690

 

  1. Use of gestational surrogates for women with Eisenmenger syndrome: a cost-effectiveness analysis.

Clennon EK, Pare E, Amato P, Caughey AB.

J Matern Fetal Neonatal Med. 2019 May 2:1-6. doi: 10.1080/14767058.2019.1610734. [Epub ahead of print]

PMID: 31006283

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Select item 30999804

 

  1. The Liver in Circulatory Disturbances.

Hilscher MB, Kamath PS.

Clin Liver Dis. 2019 May;23(2):209-220. doi: 10.1016/j.cld.2018.12.004. Epub 2019 Mar 8. Review.

PMID: 30947872

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Select item 30585527

 

  1. Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease.

Cassidy AR, Bernstein JH, Bellinger DC, Newburger JW, DeMaso DR.

Clin Neuropsychol. 2019 May;33(4):760-778. doi: 10.1080/13854046.2018.1503333. Epub 2018 Dec 26.

PMID: 30585527

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Select item 30784161

 

  1. A 26-year-old man with severe right ventricular hypertrophy.

Awerbach JD, Ward C, Campbell MJ.

Heart. 2019 May;105(10):774-789. doi: 10.1136/heartjnl-2018-314123. Epub 2019 Jan 19. No abstract available.

PMID: 30661039

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Select item 30415204

 

  1. Aortic dilatation and outcome in women with Turner syndrome.

Duijnhouwer AL, Bons LR, Timmers HJLM, van Kimmenade RRL, Snoeren M, Timmermans J, van den Hoven AT, Kempers M, van Dijk APJ, Fleischer K, Roos-Hesselink JW.

Heart. 2019 May;105(9):693-700. doi: 10.1136/heartjnl-2018-313716. Epub 2018 Oct 27.

PMID: 30368486

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Select item 30368485

 

  1. Coronary anatomy in Turner syndrome versus patients with isolated bicuspid aortic valves.

Koenraadt WMC, Siebelink HJ, Bartelings MM, Schalij MJ, van der Vlugt MJ, van den Bosch AE, Budde RPJ, Roos-Hesselink JW, Duijnhouwer AL, van den Hoven AT, DeRuiter MC, Jongbloed MRM.

Heart. 2019 May;105(9):701-707. doi: 10.1136/heartjnl-2018-313724. Epub 2018 Oct 27.

PMID: 30368485

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Select item 31147189

 

  1. Relationship between non-osmotic arginine vasopressin secretion and hemoglobin A1c levels in adult patients with congenital heart disease.

Murakami T, Horibata Y, Tateno S, Kawasoe Y, Niwa K.

Heart Vessels. 2019 May;34(5):809-814. doi: 10.1007/s00380-018-1309-z. Epub 2018 Nov 20.

PMID: 30460574

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Select item 30704770

 

  1. Subclinical atherosclerosis in patients with cyanotic congenital heart disease.

Mohammad Nijres B, Samuel BP, Vettukattil JJ.

Int J Cardiol. 2019 May 1;282:44. doi: 10.1016/j.ijcard.2018.10.044. Epub 2019 Jan 28. No abstract available.

PMID: 30704770

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Select item 30583924

 

  1. Serial pulmonary vascular resistance assessment in patients late after ventricular septal defect repair.

Gabriels C, Buys R, Van de Bruaene A, De Meester P, Goetschalckx K, Helsen F, Moons P, Goossens E, Rega F, Voigt JU, Delcroix M, Budts W.

Int J Cardiol. 2019 May 1;282:38-43. doi: 10.1016/j.ijcard.2018.12.044. Epub 2018 Dec 16.

PMID: 30583924

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Select item 30656460

 

  1. Computed Tomography Appearance of Uncorrected Congenital Heart Disease in Adults.

Maldjian PD, Yen LH.

J Thorac Imaging. 2019 May;34(3):W13-W22. doi: 10.1097/RTI.0000000000000397.

PMID: 30741756

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Select item 30553676

 

  1. Atrial Fibrillation Therapy and Heart Failure Hospitalization in Adults With Tetralogy of Fallot.

Egbe AC, Miranda WR, Ammash NM, Ananthaneni S, Sandhyavenu H, Farouk Abdelsamid M, Yogeswaran V, Kapa S, Fatola A, Kothapalli S, Connolly HM.

JACC Clin Electrophysiol. 2019 May;5(5):618-625. doi: 10.1016/j.jacep.2019.01.005. Epub 2019 Feb 27.

PMID: 31122385

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Select item 30910096

 

  1. Four-dimensional Flow MRI: Principles and Cardiovascular Applications.

Azarine A, Garçon P, Stansal A, Canepa N, Angelopoulos G, Silvera S, Sidi D, Marteau V, Zins M.

Radiographics. 2019 May-Jun;39(3):632-648. doi: 10.1148/rg.2019180091. Epub 2019 Mar 22.

PMID: 30901284

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  1. Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital Heart Disease.

Lluri G, Lin J, Reardon L, Miner P, Whalen K, Aboulhosn J.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):292-295. doi: 10.1177/2150135119825599.

PMID: 31084317

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Select item 31084314

 

  1. Cardiopulmonary Exercise Testing-A Valuable Tool, Not Gatekeeper When Referring Patients With Adult Congenital Heart Disease for Transplant Evaluation.

Menachem JN, Reza N, Mazurek JA, Burstein D, Birati EY, Fox A, Kim YY, Molina M, Partington SL, Tanna M, Tobin L, Wald J, Goldberg LR.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):286-291. doi: 10.1177/2150135118825263. Epub 2019 Mar 4.

PMID: 30832541

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Select item 30354929

 

  1. Hemodynamic Effects of Paracentesis in a Patient With a Fontan Circulation.

Rajpal S, Opotowsky AR, Rutherford A, Wu F.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):357-359. doi: 10.1177/2150135118779351. Epub 2018 Oct 24.

PMID: 30354929

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Select item 29121836

 

  1. Elevated ventricular filling pressures and long-term survival in adults post-Fontan.

Miranda WR, Hagler DJ, Taggart NW, Borlaug BA, Connolly HM, Egbe AC.

Catheter Cardiovasc Interv. 2019 May 30. doi: 10.1002/ccd.28340. [Epub ahead of print]

PMID: 31145542

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Select item 31145795

 

  1. Altered ascending aortic wall shear stress in patients with corrected atrioventricular septal defect: a comprehensive cardiovascular magnetic resonance and 4D flow MRI evaluation.

Elders B, Westenberg J, van den Boogaard P, Calkoen E, Blom N, Kroft L, de Roos A, Roest A.

Cardiol Young. 2019 May 29:1-6. doi: 10.1017/S1047951119000374. [Epub ahead of print]

PMID: 31138335

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Select item 31144250

 

  1. Anomalous origin of a pulmonary artery in a teenager with tetralogy of Fallot with associated double aortic arch and coronary arteriovenous fistula.

Agrawal V, Solanki P, Shah R, Parmar D, Mishra A.

Cardiol Young. 2019 May 28:1-3. doi: 10.1017/S1047951119000933. [Epub ahead of print]

PMID: 31133077

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Select item 31136225

 

  1. MID TERM OUTCOME OF RIGHT VENTRICLE TO PULMONARY ARTERY SHUNT FOR OLDER CHILDREN AND YOUNG ADULTS WITH VENTRICULAR SEPTAL DEFECT, PULMONARY ATRESIA AND HYPOPLASTIC PULMONARY ARTERIES.

Patel K, Rajan SK, Garg P, Gajjar T, Mishra A, Kumar R, Rana Y, Ananthnarayan C, Sharma P, Patel S.

Semin Thorac Cardiovasc Surg. 2019 May 25. pii: S1043-0679(19)30152-2. doi: 10.1053/j.semtcvs.2019.05.014. [Epub ahead of print]

PMID: 31136797

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  1. Mechanics of the Tricuspid Valve-From Clinical Diagnosis/Treatment, In-Vivo and In-Vitro Investigations, to Patient-Specific Biomechanical Modeling.

Lee CH, Laurence DW, Ross CJ, Kramer KE, Babu AR, Johnson EL, Hsu MC, Aggarwal A, Mir A, Burkhart HM, Towner RA, Baumwart R, Wu Y.

Bioengineering (Basel). 2019 May 22;6(2). pii: E47. doi: 10.3390/bioengineering6020047. Review.

PMID: 31121881 Free Article

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Select item 31119861

 

  1. 18F-FDG-PET/CT in diagnosis of Q fever endocarditis.

Domingues CM, Ferreira MJ, Silva R, Gonçalves V, Silva AM, Gonçalves L.

J Nucl Cardiol. 2019 May 22. doi: 10.1007/s12350-019-01750-8. [Epub ahead of print] No abstract available.

PMID: 31119713

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Select item 31116053

 

  1. The Golden Proportion in the scallop geometry of normal mitral valves. When nature plays with jigsaw puzzles.

Deorsola L, Bellone A.

Echocardiography. 2019 May 16. doi: 10.1111/echo.14366. [Epub ahead of print]

PMID: 31095787

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Select item 31130490

 

  1. Differences in pulmonary arterial flow hemodynamics between children and adults with pulmonary arterial hypertension as assessed by 4D-flow CMR studies.

Schäfer M, Ivy DD, Abman SH, Stenmark K, Browne LP, Barker AJ, Mitchell MB, Morgan GJ, Wilson N, Shah A, Kollengode M, Naresh N, Fonseca B, DiMaria M, Buckner JK, Hunter KS, Kheyfets V, Fenster BE, Truong U.

Am J Physiol Heart Circ Physiol. 2019 May 1;316(5):H1091-H1104. doi: 10.1152/ajpheart.00802.2018. Epub 2019 Mar 1.

PMID: 30822118

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Select item 30614192

 

  1. Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease.

Cassidy AR, Bernstein JH, Bellinger DC, Newburger JW, DeMaso DR.

Clin Neuropsychol. 2019 May;33(4):760-778. doi: 10.1080/13854046.2018.1503333. Epub 2018 Dec 26.

PMID: 30585527

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Select item 30739311

 

ACHD Electrophysiology Featured Articles

Congenital Heart and Pediatric EP Featured Manuscripts of May 2019

 

Establishment of Specialized Clinical Cardiovascular Genetics Programs: Recognizing the Need and Meeting Standards: A Scientific Statement From the American Heart Association.

Ahmad F, McNally EM, Ackerman MJ, Baty LC, Day SM, Kullo IJ, Madueme PC, Maron MS, Martinez MW, Salberg L, Taylor MR, Wilcox JE; American Heart Association Council on Genomic and Precision Medicine; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Basic Cardiovascular Sciences; Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; and Stroke Council.

Circ Genom Precis Med. 2019 May 23:HCG0000000000000054. doi: 10.1161/HCG.0000000000000054. [Epub ahead of print]

PMID: 31117808

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Select item 31136304

Take Home Points:

  1. Cardiovascular genetics is evolving as a new frontier with advances in genome sequencing, genetic testing with application to a wide variety of cardiac conditions.
  2. The next big leap is to integrate clinical cardiovascular findings with genetic information to allow for improved diagnosis, prognostication of genetically vulnerable families with identification of proband.
  3. This scientific statement outlines current best practices for establishing clinical cardiovascular genetics programs (CVGPs) and on delivering cardiovascular genetic evaluation and care in both the pediatric and the adult settings, with a focus on a heart team approach along with a medical geneticist.
  4. It would be interesting to see the evolution of the medicolegal aspect of CV genetics given the vast information from genetic testing. Genetic Information Nondiscrimination Act (GINA) provides protection against denial of employment or health insurance based on genetic test results.
  5. Prediagnosis with genetic studies, antemortem imaging or invasive studies and postmortem information when needed constitutes a full spectrum analysis of a heritable cardiac condition.
  6. Depending on the heritable CVD under consideration, genetic testing can impact diagnostic, prognostic, and therapeutic decision making. In some disorders, such as LQTS, FH, and aortopathies, the impact is robust in all three respects, whereas in other disorders such as familial atrial fibrillation, the impact is limited perhaps to identification of potential at-risk family members by cascade testing.
  7. Despite the effort by the American College of Medical Genetics and Genomics to standardize guidelines for interpretation, genetic testing laboratories may not agree on variant interpretation. Misclassification of variants has serious clinical implications.
  1. The American College of Medical Genetics and Genomics identified 59 genes as medically actionable, indicating that variants in these genes should prompt follow-up medical care. Thirty-one of the 59 genes are linked to CVDs, including cardiomyopathies, heritable arrhythmias, aortopathies, and FH.
  2. The use of multigene panels is standard for most clinical cardiovascular genetic testing because of the extent of locus and allelic heterogeneity in cardiac conditions.
  3. The development of formal CVGP clinics is a relatively new phenomenon. Detailed studies of their impact, resource needs, funding models, patient outcomes, and overall roles and value within the healthcare system generally have not been conducted.
  4. Core personnel include cardiologists, medical geneticists, genetic counselors, nurse managers, and clinical coordinators.

Comment from Dr. Khyati Pandya  (Mississippi), section editor of Congenital Electrophysiology Journal Watch:  Major advances in the field of molecular genetics have expanded our ability to identify genetic substrates underlying the pathogenesis of various disorders that follow Mendelian inheritance patterns. Included among these disorders are the potentially lethal and heritable channelopathies and cardiomyopathies for which the underlying genetic basis has been identified and is now better understood (Ackerman et al).

Inherited cardiac conditions (ICCs) comprise a broad range of syndromes that affect the heart and major blood vessels, including cardiomyopathies, arrhythmias, aortopathies, familial hypercholesterolemia (FH), and congenital structural heart defects. Collectively, these disorders are relatively common and are associated with adverse clinical outcomes such as sudden cardiac death (SCD), heart failure, and premature coronary heart disease (CHD).

Advances in sequencing technology have expanded testing panels for inherited cardiac conditions and driven down costs, further improving the cost-effectiveness of genetic testing. However, this expanded testing requires great rigor in the identification of pathogenic variants, with domain-specific knowledge required for variant interpretation (Walsh et al).

The table below is a comprehensive list of the major genes associated with various inheritable cardiac disorders. (Walsh et al; Clinical Chemistry 63:1 116–128 (2017) )

There are significant challenges to the establishment of clinical cardiovascular genetics programs (CVGPs) in smaller programs, as these require presence of on-site clinical geneticist, genetic counselors and a significant time commitment from multiple specialties for effective coordination. The presence of remote support from genetic counselors via telemedicine may serve to mitigate this problem. A lot more remains to be desired regarding insurance coverage for genetic testing, although, it has considerably improved compared to previous.

 

 

Illustrative workflow for evaluation and management in a cardiovascular genetics program clinic of new patients who do not have a known pathogenic variant.

Illustrative workflow for evaluation and management in a cardiovascular genetics program clinic of members of families with a known pathogenic variant.

The principal benefit of clinical genetic testing in the current era, is the cascade screening of family members of patients with a pathogenic variant, enabling targeted follow up of presymptomatic genotype-positive individuals and discharge of genotype-negative individuals to health. For the proband, diagnostic sequencing can also be useful in discriminating inherited disease from alternative diagnoses, directing treatment, and for molecular autopsy in cases of sudden unexplained death. Advances in sequencing technology have expanded testing panels for inherited cardiac conditions and driven down costs, further improving the cost-effectiveness of genetic testing. However, this expanded testing requires great rigor in the identification of pathogenic variants, with domain-specific knowledge required for variant interpretation. Diagnostic sequencing can potentially become an integral part of the clinical management of patients with inherited cardiac conditions. However, to move beyond just confirmatory and predictive testing, a much greater understanding is needed of the genetic basis of these conditions, the role of the environment, and the underlying disease mechanisms. With this additional information it is likely that genetic testing will increasingly be used for stratified and preventative strategies in the era of genomic medicine. The establishment of CVGPs is probably the most effective and comprehensive method for diagnosis and treatment of a wide array of heritable cardiac conditions, that, heretofore, remained somewhat elusive. These programs are up and running in some major/ tertiary institutions. However, making this a routine practice for diagnosis of heritable cardiac conditions, would be the standard of care. Till such time, that the creation of CVGPs is made uniform practice, a primary need, is establishing screening programs. These maybe in the form of community screening which in turn maybe achieved by distributing screening questionnaires to identify individuals at risk for sudden cardiac death, premature atherosclerosis or heart failure. Once identified, these patients can then be referred to CVGPs for further investigations. Genetic counselors form the cornerstone of CVGPs and serve as a liaison between the clinician and the patient. As of today, cardiogenetics largely remains underutilized due to various factors. A fully established CVGP is an exciting frontier for identification and management of heritable cardiac conditions. Collaborating with the coroner for cascade screening of families of young individuals who have suffered sudden cardiac death cannot be overemphasized.

 

 

CHD EP May 2019

 

  1. Trend and risk factors of recurrence and complications after arrhythmias radiofrequency catheter ablation: a nation-wide observational study in Taiwan.

Lin Y, Wu HK, Wang TH, Chen TH, Lin YS.

BMJ Open. 2019 May 30;9(5):e023487. doi: 10.1136/bmjopen-2018-023487.

PMID: 31152025 Free Article

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Select item 31145053

 

  1. Implantable cardioverter-defibrillators in previously undiagnosed patients with catecholaminergic polymorphic ventricular tachycardia resuscitated from sudden cardiac arrest.

van der Werf C, Lieve KV, Bos JM, Lane CM, Denjoy I, Roses-Noguer F, Aiba T, Wada Y, Ingles J, Leren IS, Rudic B, Schwartz PJ, Maltret A, Sacher F, Skinner JR, Krahn AD, Roston TM, Tfelt-Hansen J, Swan H, Robyns T, Ohno S, Roberts JD, van den Berg MP, Kammeraad JA, Probst V, Kannankeril PJ, Blom NA, Behr ER, Borggrefe M, Haugaa KH, Semsarian C, Horie M, Shimizu W, Till JA, Leenhardt A, Ackerman MJ, Wilde AA.

Eur Heart J. 2019 May 30. pii: ehz309. doi: 10.1093/eurheartj/ehz309. [Epub ahead of print]

PMID: 31145795

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Select item 31105146

 

  1. Feasibility and safety of cryoballoon ablation for atrial fibrillation in patients with congenital heart disease.

Abadir S, Waldmann V, Dyrda K, Laredo M, Mondésert B, Dubuc M, Khairy P.

World J Cardiol. 2019 May 26;11(5):149-158. doi: 10.4330/wjc.v11.i5.149.

PMID: 31171960

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Select item 31131388

 

  1. How to deliver personalized Cardiac Resynchronization Therapy through the precise measurement of the acute hemodynamic response: insights from the iSpot trial.

Shun-Shin MJ, Miyazawa AA, Keene D, Sterliński M, Sokal A, Van Heuverswyn F, Rinaldi CA, Cornelussen R, Stegemann B, Francis DP, Whinnett Z.

J Cardiovasc Electrophysiol. 2019 May 22. doi: 10.1111/jce.14001. [Epub ahead of print]

PMID: 31115945

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Select item 31115957

 

  1. Intra-atrial re-entrant tachycardia around atretic tricuspid annulus.

Waldmann V, Amet D, Ladouceur M, Soulat G, Marijon E, Iserin L.

Europace. 2019 May 21. pii: euz134. doi: 10.1093/europace/euz134. [Epub ahead of print] No abstract available.

PMID: 31114849

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Select item 31112448

 

  1. Long Term Follow up after VT Ablation in Patients with Congenital Heart Disease.

Yang J, Brunnquell M, Liang JJ, Callans DJ, Garcia FC, Lin D, Frankel DS, Kay J, Marchlinski FE, Tzou W, Sauer WH, Mbbs BL, Ruckdeschel ES, Collins K, Santangeli P, Nguyen DT.

J Cardiovasc Electrophysiol. 2019 May 20. doi: 10.1111/jce.13996. [Epub ahead of print]

PMID: 31111602

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Select item 31106637

 

  1. Stenting the vertical neonatal ductus arteriosus via the percutaneous axillary approach.

Lee J, Ratnayaka K, Moore J, El-Said H.

Congenit Heart Dis. 2019 May 13. doi: 10.1111/chd.12786. [Epub ahead of print]

PMID: 31083775

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Select item 31083772

 

  1. Long-term proarrhythmic pharmacotherapy among patients with congenital long QT syndrome and risk of arrhythmia and mortality.

Weeke PE, Kellemann JS, Jespersen CB, Theilade J, Kanters JK, Hansen MS, Christiansen M, Marstrand P, Gislason GH, Torp-Pedersen C, Bundgaard H, Jensen HK, Tfelt-Hansen J.

Eur Heart J. 2019 May 11. pii: ehz228. doi: 10.1093/eurheartj/ehz228. [Epub ahead of print]

PMID: 31079148

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Select item 31079145

 

  1. Initial Experience with Transcatheter Pacemaker Implantation for Adults with Congenital Heart Disease.

Russell MR, Galloti R, Moore JP.

J Cardiovasc Electrophysiol. 2019 May 2. doi: 10.1111/jce.13961. [Epub ahead of print]

PMID: 31045294

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Select item 31006283

 

  1. Electro-vectorcardiographic and electrophysiological aspects of Ebstein’s anomaly.

Pérez-Riera AR, Barbosa-Barros R, Daminello-Raimundo R, de Abreu LC, Nikus K.

Ann Noninvasive Electrocardiol. 2019 May;24(3):e12590. doi: 10.1111/anec.12590. Epub 2018 Aug 14. Review.

PMID: 30106198

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Select item 31143055

 

  1. Use of the Abbott EnSite Precision three-dimensional mapping system for the placement of an atrial pacemaker lead in a patient with congenital heart disease.

Paech C, Schnappauf D, Gehre T, Wagner F, Gebauer RA.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):130-131. doi: 10.4103/apc.APC_138_18.

PMID: 31143037 Free PMC Article

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Select item 31143035

 

  1. Cardiac Resynchronization Therapy for Treatment of Chronic Subpulmonary Right Ventricular Dysfunction in Congenital Heart Disease.

Janoušek J, Kovanda J, Ložek M, Tomek V, Gebauer R, Kubuš P, Delhaas T.

Circ Arrhythm Electrophysiol. 2019 May;12(5):e007157. doi: 10.1161/CIRCEP.119.007157. No abstract available.

PMID: 30991822

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Select item 30868567

 

  1. Incidence and effect of early postoperative ventricular arrhythmias after congenital heart surgery.

Fuchs SR, Smith AH, Van Driest SL, Crum KF, Edwards TL, Kannankeril PJ.

Heart Rhythm. 2019 May;16(5):710-716. doi: 10.1016/j.hrthm.2018.11.032. Epub 2018 Dec 5.

PMID: 30528449

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Select item 30460574

 

  1. Successful biventricular cardiac resynchronization therapy in a failing Fontan patient: Implications of ventriculo-ventricular interdependency in single ventricle physiology.

Udink Ten Cate FEA, Germund I, Goossens SS, Bennink G, Sreeram N.

Echocardiography. 2019 May 31. doi: 10.1111/echo.14376. [Epub ahead of print]

PMID: 31148229

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Select item 31151956

 

  1. Right Atrial Appendage Aneurysm Resection to Cure Aneurysm-Related Atrial Tachyarrhythmia.

Zhang Y, Li XM, Jiang H, Jin YQ, Zhang FQ, Liu HJ, Ge HY.

Pediatr Cardiol. 2019 May 31. doi: 10.1007/s00246-019-02117-z. [Epub ahead of print]

PMID: 31152184

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Select item 31162368

 

  1. Evaluation of Atrial Electromechanical Delay in Children with Obesity.

Temiz F, Güneş H, Güneş H.

Medicina (Kaunas). 2019 May 30;55(6). pii: E228. doi: 10.3390/medicina55060228.

PMID: 31151287 Free Article

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Select item 31145818

 

  1. Establishment of Specialized Clinical Cardiovascular Genetics Programs: Recognizing the Need and Meeting Standards: A Scientific Statement From the American Heart Association.

Ahmad F, McNally EM, Ackerman MJ, Baty LC, Day SM, Kullo IJ, Madueme PC, Maron MS, Martinez MW, Salberg L, Taylor MR, Wilcox JE; American Heart Association Council on Genomic and Precision Medicine; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Basic Cardiovascular Sciences; Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; and Stroke Council.

Circ Genom Precis Med. 2019 May 23:HCG0000000000000054. doi: 10.1161/HCG.0000000000000054. [Epub ahead of print]

PMID: 31117808

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Select item 31136304

 

  1. Ventricular repolarization changes in children with breath holding spells.

Akpinar M, Ocal M, Irdem A.

J Electrocardiol. 2019 May 21;55:116-119. doi: 10.1016/j.jelectrocard.2019.05.013. [Epub ahead of print]

PMID: 31152993

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Select item 31112258

 

  1. Double cryoenergy application (freeze-thaw-freeze) at growing myocardium: Lesion volume and effects on coronary arteries late after energy application. Implications for efficacy and safety in pediatric patients.

Schneider HE, Stahl M, Schillinger W, Müller M, Backhoff D, Schill M, Groene N, Krause U, Sigler M, Paul T.

J Cardiovasc Electrophysiol. 2019 May 20. doi: 10.1111/jce.13993. [Epub ahead of print]

PMID: 31111603

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Select item 31111600

 

  1. “A Song of Ice and Fire” – Another Verse from the World of Ablation.

Saul JP, LaPage MJ.

J Cardiovasc Electrophysiol. 2019 May 20. doi: 10.1111/jce.13987. [Epub ahead of print]

PMID: 31111600

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Select item 31116157

 

  1. Elimination of arrhythmogenesis after subtotal resection of congenital cardiac fibroma: a case report.

Nabeshima T, Sato S, Nakayashiro M.

Cardiol Young. 2019 May 17:1-3. doi: 10.1017/S1047951119000180. [Epub ahead of print]

PMID: 31097046

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Select item 31099489

 

  1. Long-Term Follow-Up of Idiopathic Ventricular Fibrillation in a Pediatric Population: Clinical Characteristics, Management, and Complications.

Frontera A, Vlachos K, Kitamura T, Mahida S, Pillois X, Fahy G, Marquie C, Cappato R, Stuart G, Defaye P, Kaski JP, Ector J, Maltret A, Scanu P, Pasquie JL, Deisenhofer I, Blankoff I, Scherr D, Manninger M, Aizawa Y, Koutbi L, Denis A, Pambrun T, Ritter P, Sacher F, Hocini M, Maury P, Jaïs P, Bordachar P, Haïssaguerre M, Derval N.

J Am Heart Assoc. 2019 May 7;8(9):e011172. doi: 10.1161/JAHA.118.011172.

PMID: 31057083 Free PMC Article

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Select item 31023121

 

  1. Prenatal diagnosis and management of congenital complete heart block.

Pruetz JD, Miller JC, Loeb GE, Silka MJ, Bar-Cohen Y, Chmait RH.

Birth Defects Res. 2019 May 1;111(8):380-388. doi: 10.1002/bdr2.1459. Epub 2019 Mar 1. Review.

PMID: 30821931

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Select item 30957841

 

  1. Mexiletine Shortens the QT Interval in Patients With Potassium Channel-Mediated Type 2 Long QT Syndrome.

Bos JM, Crotti L, Rohatgi RK, Castelletti S, Dagradi F, Schwartz PJ, Ackerman MJ.

Circ Arrhythm Electrophysiol. 2019 May;12(5):e007280. doi: 10.1161/CIRCEP.118.007280.

PMID: 31006312

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Select item 31112425

 

  1. Response by Kass et al to Letter Regarding Article, “Chronic Atrial and Ventricular Pacing in the Mouse: Application to Model Cardiac Dyssynchrony and Resynchronization in Heart Failure”.

Kass DA, Nakagawa R, Ståhlberg M.

Circ Heart Fail. 2019 May;12(5):e006094. doi: 10.1161/CIRCHEARTFAILURE.119.006094. No abstract available.

PMID: 31079489

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Select item 30786071

 

  1. Interdigitating Myocardial Tongues in Pediatric Cardiac Fibromas: Plausible Substrate for Ventricular Tachycardia and Cardiac Arrest.

Carreon CK, Sanders SP, Perez-Atayde AR, Del Nido PJ, Walsh EP, Geva T, Alexander ME.

JACC Clin Electrophysiol. 2019 May;5(5):563-575. doi: 10.1016/j.jacep.2019.01.022. Epub 2019 Mar 27.

PMID: 31122378

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  1. A Novel Truncating Variant in FLNC-Encoded Filamin C May Serve as a Proarrhythmic Genetic Substrate for Arrhythmogenic Bileaflet Mitral Valve Prolapse Syndrome.

Bains S, Tester DJ, Asirvatham SJ, Noseworthy PA, Ackerman MJ, Giudicessi JR.

Mayo Clin Proc. 2019 May;94(5):906-913. doi: 10.1016/j.mayocp.2018.11.028. Epub 2019 Mar 29.

PMID: 30935706

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Select item 30629048

 

  1. A systematic review and meta-analysis of the effect of dispatcher-assisted CPR on outcomes from sudden cardiac arrest in adults and children.

Nikolaou N, Dainty KN, Couper K, Morley P, Tijssen J, Vaillancourt C; International Liaison Committee on Resuscitation’s (ILCOR) Basic Life Support and Pediatric Task Forces.

Resuscitation. 2019 May;138:82-105. doi: 10.1016/j.resuscitation.2019.02.035. Epub 2019 Mar 8. Review.

PMID: 30853623

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Select item 30844415

ACHD Interventional Cardiology Featured Articles

Interventional Cardiology Featured Manuscripts of May 2019

 

Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory.

O’Byrne ML, Millenson ME, Steven JM, Gillespie MJ, Dori Y, Glatz AC, Rome JJ.

JACC Cardiovasc Interv. 2019 May 13;12(9):835-843. doi: 10.1016/j.jcin.2019.01.224. Epub 2019 Apr 10.

PMID: 30981573

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Select item 30981569

 

Comment from Dr. Wendy Whiteside (Michigan), section editor of Congenital Heart Disease Interventions Journal Watch:  Procedural sedation, without the use of an anesthesiologist, has historically been used commonly within the pediatric cardiac catheterization lab.  More recently, however, there has been a shift towards the use of anesthesiologists in these cases, with the intention of providing improved patient safety with a dedicated provider in charge of patient sedation/anesthetic.  With this transition has come an increasing use of general anesthesia (GA) in the pediatric catheterization lab.  The safety of procedural sedation versus general anesthesia has not, however, been critically evaluated in this setting.

O’Byrne et al therefore present a single-center retrospective cohort study comparing the relative safety, cost, and case times of catheterization procedures performed using operator-directed sedation (ODS) compared with general anesthesia administered by a cardiac anesthesiologist.  ODS consisted of either IV sedating agents or local analgesia administered by a registered nurse under the supervision of the interventional cardiologist.  Over a 6.5 year period from 2011-2017, they studied 4,424 cases in 2,547 patients.  Of these, 27% of cases were performed with ODS (93% performed with IV sedation and 7% with local anesthetic alone).  Major adverse events (MAE) were higher in GA cases compared with ODS cases (6.6% vs 3.4%; p<0.001), a difference which remained in propensity score-adjusted models, with odds of a MAE lower in cases performed using ODS compared with GA (0.66, 95% CI 0.4-0.9, p=0.03).  Applying the CRISP score retrospectively to their cases, they determined that only a minority (10.2%) of procedures using ODS would have aligned with the 2016 expert consensus recommendations and would have been appropriate to be performed without an anesthesiologist.  Despite this, when the examined observed-to-expected MAE ratios for cases stratified by CRISP score and mode of sedation, they found that this deviation from recommendations was not associated with a change in the observed-to-expected ratio for MAE.  Total room time, total sheath time, and room exit time (time from hemostasis to room exit) were also significantly shorter in the ODS group.  Again using propensity score adjusted models, ODS was associated with decreased hospital and professional charges compared with GA with an estimated savings in hospital costs of 2017 US dollars of $2,238 per procedure.

Clearly, as the authors state, these results are certainly not meant to imply that involvement of an anesthesiologist increases the risk of a cath procedure, however they do suggest that a careful review of each individual case may be able to identify cases in whom ODS may be safe and effective and can thereby reduce resource use (both cost and case times).  The safety and comfort of either of these techniques are certainly very center- and provider-specific and available resources in a particular center may very well dictate their distribution of sedation vs GA. There can be an argument made to utilizing sedation over GA in the cath lab, particularly in diagnostic cases, where free breathing with procedural sedation will provide a more accurate assessment of baseline hemodynamics.  GA, however, may provide a better steady-state, with less patient movement, that may be advantageous for particular procedures, especially in light of the more complex procedures being performed in more hemodynamically vulnerable patients in current cardiac catheterization labs. While involvement of anesthesiologists has become more common in catheterization labs through the country, this should not automatically imply the use of GA.  There should be careful consideration of the most appropriate sedation/anesthesia for a particular case, particularly in light of increased safety and efficiency of sedation over GA.

 

Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension.

Frank BS, Schäfer M, Grenolds A, Ivy DD, Abman SH, Darst JR.

J Pediatr. 2019 May;208:127-133. doi: 10.1016/j.jpeds.2018.12.004. Epub 2019 Mar 11.

PMID: 30871795

 

Take Home Points: 

  • Positive response to AVT but not baseline hemodynamics is associated with better long-term clinical outcomes in premature infants with BPD-associated PH.
  • Cardiac catheterization plays an important diagnostic and therapeutic role in the management of this patient population.

Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch:  Bronchopulmonary dysplasia (BPD) associated pulmonary hypertension (PH) contributes to significant long-term mortality and morbidity in premature infants. The optimal strategies to diagnose and treat this patient population are poorly defined. The authors sought to evaluate the role of acute vasoreactivity testing (AVT) in the assessment of premature infants with BPD-associated PH.

From 2009-2017 twenty-six premature infants (mean birth weight 580g, gestational age 26 weeks) with PH underwent cardiac catheterization and made up the study cohort; those with complex congenital heart disease were excluded. At the time of catheterization the mean age was 156 days and weight 4.1kg. Patients first underwent a baseline assessment on room air (or as close as possible) and then on 100% FiO2 and 20-40ppm iNO. Baseline hemodynamics reflected the presence of PH; 7 patients had pulmonary vein stenosis identified (not suspected pre-cath imaging in 4); and 6 patients had interventions performed (pulmonary vein dilation [4]; ASD closure [1]; PDA closure [1]). There was no relationship between baseline pulmonary hemodynamics and clinical outcomes.

Nine patients had a positive response to AVT (based on the Barst criteria) with responders having a higher birth weight (690 v 540 g), gestational age (28 vs. 26 weeks) and baseline PVRi (6.7 vs. 4 iWu). Only one of the responders (11%) had the combined outcome of death or tracheostomy vs. 6 of non-responders (35%). Kaplan-Meier event-free survival comparison is shown below. There were 4 procedural complications: 3 minor (vascular thrombosis [2]; atrial flutter requiring cardioversion [1]) and 1 major (ventricular tachycardia requiring cardioversion).

The authors conclude that while baseline hemodynamics did not predict long-term clinical outcomes, positive response to AVT was associated with a lower long-term risk of tracheostomy or death. While the risk of cardiac catheterization in patients PH is known to be significant the authors report only 1 major adverse event with no mortality and report that many patients had a change in the clinical management based on the catheterization. The findings presented in this manuscript raise important questions about the diagnosis and management of patients with BPD-associated PH. Should more patients with BPD-associated PH undergo invasive evaluation with AVT? Should non-responders be treated more aggressively given their higher long-term risk? As the survival of premature infants continues to improve it will be increasingly important to answer these (and other) questions.

 

 

CHD Interventions May 2019

 

  1. Biodegradable atrial septal defect occluder: A current review.

Shi D, Kang Y, Zhang G, Gao C, Lu W, Zou H, Jiang H.

Acta Biomater. 2019 May 31. pii: S1742-7061(19)30401-5. doi: 10.1016/j.actbio.2019.05.073. [Epub ahead of print] Review.

PMID: 31158496

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  1. The clinical characteristics of coronary artery fistula anomalies in children and adults: A 24-year experience.

Huynh KT, Truong VT, Ngo TNM, Dang TB, Mazur W, Chung ES, Tretter JT, Kereiakes DJ, Le TK, Pham VN.

Congenit Heart Dis. 2019 May 28. doi: 10.1111/chd.12781. [Epub ahead of print]

PMID: 31136097

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  1. Pol-CDRIE registry – 1-year observational data on patients hospitalized due to cardiac device-related infective endocarditis in Polish referential cardiology centres.

Płońska-Gościniak E, Olędzki S, Kukulski T, Kosmala W, Młynarski R, Oko-Sarnowska Z, Wojtkowska A, Gościniak P, Gąsior Z, Hryniewiecki T, Boczar K, Daniłowicz-Szymanowicz L, Kempa M, Jaroch J, Nowalany-Kozielska E, Lisowska A, Mizia-Stec K, Sinkiewicz W, Świątkiewicz I, Szyszka A, Kosior D, Kasprzak JD.

Kardiol Pol. 2019 May 24;77(5):561-567. doi: 10.33963/KP.14811. Epub 2019 Apr 26.

PMID: 31066721 Free Article

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  1. Determinants of long-term outcome of repaired pulmonary valve stenosis.

Galian-Gay L, Gordon B, Marsal JR, Rafecas A, Pijuan Domènech A, Castro MA, Subirana Domènech MT, Sureda C, Miranda B, Martí G, García-Dorado D, Dos Subirà L.

Rev Esp Cardiol (Engl Ed). 2019 May 24. pii: S1885-5857(19)30075-1. doi: 10.1016/j.rec.2019.02.014. [Epub ahead of print] English, Spanish.

PMID: 31130344

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Select item 31142083

 

  1. Electrospun Nanofiber-Based Patches for the Delivery of Cardiac Progenitor Cells.

Streeter BW, Xue J, Xia Y, Davis ME.

ACS Appl Mater Interfaces. 2019 May 22;11(20):18242-18253. doi: 10.1021/acsami.9b04473. Epub 2019 May 7.

PMID: 31021079

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  1. Flexible Modeling of the Association Between Cumulative Exposure to Low-Dose Ionizing Radiation from Cardiac Procedures and the Malignancy Risk in Adults with Congenital Heart Disease.

Danieli C, Cohen S, Liu A, Pilote L, Guo L, Beauchamp ME, Marelli AJ, Abrahamowicz M.

Am J Epidemiol. 2019 May 20. pii: kwz114. doi: 10.1093/aje/kwz114. [Epub ahead of print]

PMID: 31107497

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Select item 31104634

 

  1. Three-dimensional printing in congenital heart disease: Considerations on training and clinical implementation from a teaching session.

Biglino G, Milano EG, Capelli C, Wray J, Shearn AI, Caputo M, Bucciarelli-Ducci C, Taylor AM, Schievano S.

Int J Artif Organs. 2019 May 20:391398819849074. doi: 10.1177/0391398819849074. [Epub ahead of print]

PMID: 31104546

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Select item 31151765

 

  1. Sedation in the Pediatric and Congenital Catheterization Laboratory: Is it Time We Wake Them Up?

Jayaram N, Kaine S, Divekar AA.

JACC Cardiovasc Interv. 2019 May 13;12(9):844-846. doi: 10.1016/j.jcin.2019.02.001. Epub 2019 Apr 10. No abstract available.

PMID: 30981569

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Select item 31087143

 

  1. Comparison of drug eluting versus bare metal stents for pulmonary vein stenosis in childhood.

Khan A, Qureshi AM, Justino H.

Catheter Cardiovasc Interv. 2019 May 8. doi: 10.1002/ccd.28328. [Epub ahead of print]

PMID: 31067002

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Select item 31066199

 

  1. Parental anxiety before invasive cardiac procedure in children with congenital heart disease: Contributing factors and consequences.

Werner O, El Louali F, Fouilloux V, Amedro P, Ovaert C.

Congenit Heart Dis. 2019 May 8. doi: 10.1111/chd.12777. [Epub ahead of print]

PMID: 31066183

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Select item 31102825

 

  1. Interhospital Variation in the Costs of Pediatric/Congenital Cardiac Catheterization Laboratory Procedures: Analysis of Data From the Pediatric Health Information Systems Database.

O’Byrne ML, Glatz AC, Faerber JA, Seshadri R, Millenson ME, Mi L, Shinohara RT, Dori Y, Gillespie MJ, Rome JJ, Kawut SM, Groeneveld PW.

J Am Heart Assoc. 2019 May 7;8(9):e011543. doi: 10.1161/JAHA.118.011543.

PMID: 31023121 Free PMC Article

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  1. X-ray fused with MRI guidance of pre-selected transcatheter congenital heart disease interventions.

Grant EK, Kanter JP, Olivieri LJ, Cross RR, Campbell-Washburn A, Faranesh AZ, Cronin I, Hamann KS, O’Byrne ML, Slack MC, Lederman RJ, Ratnayaka K.

Catheter Cardiovasc Interv. 2019 May 6. doi: 10.1002/ccd.28324. [Epub ahead of print]

PMID: 31062506

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Select item 31059210

 

  1. A Model for Assessment of Catheterization Risk in Adults With Congenital Heart Disease.

Taggart NW, Du W, Forbes TJ, Nykanen DG, Wax DF, Cabalka AK, Reeves JH, Du Y, Kobayashi D.

Am J Cardiol. 2019 May 1;123(9):1527-1531. doi: 10.1016/j.amjcard.2019.01.042. Epub 2019 Feb 8.

PMID: 30797558

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Select item 30833044

 

  1. Fetal cardiac intervention for right sided heart disease: Pulmonary atresia with intact ventricular septum.

Strainic J.

Birth Defects Res. 2019 May 1;111(8):395-399. doi: 10.1002/bdr2.1499. Epub 2019 Mar 28. Review.

PMID: 30920148

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Select item 30868768

 

  1. Fetal aortic valvuloplasty to prevent progression to hypoplastic left heart syndrome in utero.

Crystal MA, Freud LR.

Birth Defects Res. 2019 May 1;111(8):389-394. doi: 10.1002/bdr2.1478. Epub 2019 Mar 14. Review.

PMID: 30868768

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Select item 30430770

 

  1. Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

Miranda WR, Hagler DJ, Reeder GS, Warnes CA, Connolly HM, Egbe AC, Taggart NW.

Catheter Cardiovasc Interv. 2019 May 1;93(6):1069-1075. doi: 10.1002/ccd.28150. Epub 2019 Feb 12.

PMID: 30747481

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Select item 30723998

 

  1. Working backward: Retrograde balloon angioplasty of atretic arteries in chronic thromboembolic pulmonary hypertension.

Allen Ligon R, Petit CJ.

Catheter Cardiovasc Interv. 2019 May 1;93(6):1076-1079. doi: 10.1002/ccd.28115. Epub 2019 Feb 5.

PMID: 30723998

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  1. Trans-apical systemic tricuspid valve-in-ring replacement.

Ghobrial J, Reemtsen B, Levi DS, Aboulhosn J.

Catheter Cardiovasc Interv. 2019 May 1;93(6):1165-1169. doi: 10.1002/ccd.28049. Epub 2018 Dec 18.

PMID: 30565408

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Select item 30038385

 

  1. Sinus Venosus Defects: Anatomic Variants and Transcatheter Closure Feasibility Using Virtual Reality Planning.

Tandon A, Burkhardt BEU, Batsis M, Zellers TM, Velasco Forte MN, Valverde I, McMahan RP, Guleserian KJ, Greil GF, Hussain T.

JACC Cardiovasc Imaging. 2019 May;12(5):921-924. doi: 10.1016/j.jcmg.2018.10.013. Epub 2018 Dec 12. No abstract available.

PMID: 30553676

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  1. Anesthesia for high-risk procedures in the catheterization laboratory.

Daaboul DG, DiNardo JA, Nasr VG.

Paediatr Anaesth. 2019 May;29(5):491-498. doi: 10.1111/pan.13571. Epub 2019 Jan 24.

PMID: 30592354

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Select item 30592107

 

  1. Surgical Versus Percutaneous Closure of PDA in Preterm Infants: Procedural Charges and Outcomes.

Kim HS, Schechter MA, Manning PB, Eghtesady P, Balzer DT, Shahanavaz S, Rockefeller TA, Abarbanell AM.

J Surg Res. 2019 May 30;243:41-46. doi: 10.1016/j.jss.2019.04.069. [Epub ahead of print]

PMID: 31154131

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  1. Interventional atrial septal defect closure: histology of long-term performance.

Sigler M, Hofbeck M.

Eur Heart J. 2019 May 27. pii: ehz338. doi: 10.1093/eurheartj/ehz338. [Epub ahead of print] No abstract available.

PMID: 31132082

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Select item 31134320

 

  1. Transcatheter closure of residual patent ductus arteriosus.

El-Saiedi SA, Elshedoudy SA, El-Sisi AM, Hanna BM, Fattouh AM, Hijazi Z.

Catheter Cardiovasc Interv. 2019 May 23. doi: 10.1002/ccd.28338. [Epub ahead of print]

PMID: 31120630

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Select item 31117808

 

  1. The risk of infective endocarditis following interventional pulmonary valve implantation: A meta-analysis.

Lehner A, Haas NA, Dietl M, Jakob A, Schulze-Neick I, Dalla Pozza R, Rodriguez SF, Fischer M.

J Cardiol. 2019 May 18. pii: S0914-5087(19)30114-5. doi: 10.1016/j.jjcc.2019.04.007. [Epub ahead of print] Review.

PMID: 31113702

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Select item 31102204

 

  1. Balloon Atrial Septostomy in Neonates: Moving from Catheterization Laboratory to Bedside.

Arya A, Azad S.

Indian Pediatr. 2019 May 15;56(5):421. No abstract available.

PMID: 31102384 Free Article

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Select item 30826194

 

  1. Intravascular Papillary Endothelial Hyperplasia in the Coronary Artery: An Unusual Cause of Massive Myocardial Infarction in Hypoplastic Left Heart Syndrome.

Safa R, Garcia R, Delius R, Kaur G, Youssef L, Poulik J, Shehata BM.

Fetal Pediatr Pathol. 2019 May 14:1-7. doi: 10.1080/15513815.2019.1613704. [Epub ahead of print]

PMID: 31084387

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Select item 31094887

 

  1. Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory.

O’Byrne ML, Millenson ME, Steven JM, Gillespie MJ, Dori Y, Glatz AC, Rome JJ.

JACC Cardiovasc Interv. 2019 May 13;12(9):835-843. doi: 10.1016/j.jcin.2019.01.224. Epub 2019 Apr 10.

PMID: 30981573

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Select item 30981569

 

  1. Holographic Augmented Reality and 3D Printing for Advanced Planning of Sinus Venosus ASD/Partial Anomalous Pulmonary Venous Return Percutaneous Management.

Butera G, Sturla F, Pluchinotta FR, Caimi A, Carminati M.

JACC Cardiovasc Interv. 2019 May 11. pii: S1936-8798(19)30751-4. doi: 10.1016/j.jcin.2019.03.020. [Epub ahead of print] No abstract available.

PMID: 31103542

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Select item 31073996

 

  1. Interhospital Variation in the Costs of Pediatric/Congenital Cardiac Catheterization Laboratory Procedures: Analysis of Data From the Pediatric Health Information Systems Database.

O’Byrne ML, Glatz AC, Faerber JA, Seshadri R, Millenson ME, Mi L, Shinohara RT, Dori Y, Gillespie MJ, Rome JJ, Kawut SM, Groeneveld PW.

J Am Heart Assoc. 2019 May 7;8(9):e011543. doi: 10.1161/JAHA.118.011543.

PMID: 31023121 Free PMC Article

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Select item 31062925

 

  1. Percutaneous Closure of a Complex Intradevice Residual Patent Foramen Ovale Under Transesophageal Echocardiographic Guidance.

VanLoozen D, Amin Z, Javaheri S, Agarwal S.

A A Pract. 2019 May 1;12(9):340-343. doi: 10.1213/XAA.0000000000000971. No abstract available.

PMID: 31048601

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Select item 30923087

 

  1. A Model for Assessment of Catheterization Risk in Adults With Congenital Heart Disease.

Taggart NW, Du W, Forbes TJ, Nykanen DG, Wax DF, Cabalka AK, Reeves JH, Du Y, Kobayashi D.

Am J Cardiol. 2019 May 1;123(9):1527-1531. doi: 10.1016/j.amjcard.2019.01.042. Epub 2019 Feb 8.

PMID: 30797558

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Select item 30833044

 

  1. Left subclavian artery stenosis treated with transcatheter stent placement in pediatric patients.

Roy M, Chattopadhyay A, Bandhyopadhyay B, Gangopadhyay D.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):163-168. doi: 10.4103/apc.APC_128_18.

PMID: 31143048 Free PMC Article

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  1. Introduction to “fetal interventions to alleviate heart defects”.

Watanabe M, Strainic JP.

Birth Defects Res. 2019 May 1;111(8):367-369. doi: 10.1002/bdr2.1483. Epub 2019 Mar 12. No abstract available.

PMID: 30860313

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Select item 30821931

 

  1. Stent-retriever thrombectomy in the treatment of infants with acute thrombosis of the superior vena cava and innominate vein.

Hanser A, Sieverding L, Hauser TK, Wiegand G, Hofbeck M.

Catheter Cardiovasc Interv. 2019 May 1;93(6):E357-E361. doi: 10.1002/ccd.28142. Epub 2019 Feb 9.

PMID: 30737974

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  1. Long-term outcomes of melody valve-in-valve implantation for bioprosthetic mitral valve dysfunction.

Joseph TA, Eleid MF, Cabalka AK, Maalouf JF, Rihal CS.

Catheter Cardiovasc Interv. 2019 May 1;93(6):1087-1094. doi: 10.1002/ccd.27988. Epub 2018 Nov 20.

PMID: 30461167

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Select item 31006312

 

  1. Upper-Extremity Venous Access for Children and Adults in Pediatric Cardiac Catheterization Laboratory.

Randall JT, Aldoss O, Khan A, Challman M, Hiremath G, Qureshi AM, Bansal M.

J Invasive Cardiol. 2019 May;31(5):141-145. Epub 2019 Feb 15.

PMID: 30765619 Free Article

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Select item 30614102

 

  1. Acute Vasoreactivity Testing during Cardiac Catheterization of Neonates with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension.

Frank BS, Schäfer M, Grenolds A, Ivy DD, Abman SH, Darst JR.

J Pediatr. 2019 May;208:127-133. doi: 10.1016/j.jpeds.2018.12.004. Epub 2019 Mar 11.

PMID: 30871795

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Select item 30738657

 

  1. Sinus Venosus Defects: Anatomic Variants and Transcatheter Closure Feasibility Using Virtual Reality Planning.

Tandon A, Burkhardt BEU, Batsis M, Zellers TM, Velasco Forte MN, Valverde I, McMahan RP, Guleserian KJ, Greil GF, Hussain T.

JACC Cardiovasc Imaging. 2019 May;12(5):921-924. doi: 10.1016/j.jcmg.2018.10.013. Epub 2018 Dec 12. No abstract available.

PMID: 30553676

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Select item 31122378

 

  1. Multisite measurement of regional oxygen saturation in Fontan patients with and without protein-losing enteropathy at rest and during exercise.

Schröer S, Fahlbusch FB, Münch F, Alkassar M, Toka HR, Rauh M, Rüffer A, Cesnjevar R, Dittrich S, Toka O, Moosmann J.

Pediatr Res. 2019 May;85(6):777-785. doi: 10.1038/s41390-019-0346-3. Epub 2019 Feb 22.

PMID: 30795005

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Select item 30770863

 

  1. The definition of a hemodynamically significant ductus arteriosus.

El-Khuffash A, Levy PT, Gorenflo M, Frantz ID 3rd.

Pediatr Res. 2019 May;85(6):740-741. doi: 10.1038/s41390-019-0342-7. Epub 2019 Feb 15. No abstract available.

PMID: 30770863

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Select item 30728445

 

  1. Left pulmonary artery stenting for relief of left pulmonary artery stenosis following ductal closure using Amplatzer Duct Occluder II.

Sheth K, Dalvi B.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):172-175. doi: 10.4103/apc.APC_101_18.

PMID: 31143050 Free PMC Article

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Select item 31143049

 

  1. Closure of insufficient, native right ventricular outflow tract with AMPLATZER™ muscular ventricular septal defect occluder in a patient with tetralogy of Fallot post-Melody® valve.

Bansal N, Aggarwal S, Turner DR.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):159-162. doi: 10.4103/apc.APC_76_18.

PMID: 31143047 Free PMC Article

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  1. Transcatheter occlusion of partial anomalous pulmonary venous connection with dual drainage to left atrium.

Gangadhara MB, Magee AG.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):144-146. doi: 10.4103/apc.APC_72_18.

PMID: 31143042 Free PMC Article

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Select item 31143041

 

  1. Inadvertent ascending aortic perforation after transseptal puncture: Successful treatment with an Amplatzer Duct Occluder II device.

Cao Y, Rosenthal E, Qureshi SA.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):138-140. doi: 10.4103/apc.APC_153_18.

PMID: 31143040 Free PMC Article

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ACHD Surgery

Congenital Heart Surgery Featured Manuscripts of May 2019

 

Impact of Regional Cerebral Perfusion on Outcomes Among Neonates Undergoing Norwood Operation.

Migally K, Rettiganti M, Gossett JM, Reemtsen B, Gupta P.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):261-267. doi: 10.1177/2150135118825274.

PMID: 31084315

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Take Home Points: 

  • The use of regional cerebral perfusion (RCP) during the Norwood surgery is a technique utilized by many (but not all) surgeons to improve cerebral protection to the neonatal brain during aortic arch reconstruction.
  • This study utilized the public use data from the Pediatric Heart Network Single Ventricle Reconstruction trial to evaluate outcomes whether RCP affected outcomes such as mortality, need for heart transplant, prolonged mechanical ventilation and prolonged hospital stay … but not neurologic outcomes. And the dataset used to come to these conclusions was not designed to evaluate RCP vs. DHCA.

 

Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch:  The traditional method of protecting the brain during aortic arch surgery has been deep hypothermic circulatory arrest (DHCA). However, in recent years, an increasing number of surgeons have been using regional cerebral perfusion (or selective antegrade cerebral perfusion) during arch surgeries to maintain some perfusion to the brain for a hypothetical improvement in outcomes, especially neurologic outcomes. The Single Ventricle Trial was conducted between 2005 and 2009 at 15 North American centers and was designed as a randomized controlled trial to evaluate modified BT shunts and Sano RV-PA conduits during the Norwood procedure for hypoplastic left heart syndrome.  The dataset from this study was made available for public use in 2013.

This present study was designed by researchers at the University of Arkansas to use this public dataset to evaluate whether patients who received RCP (as opposed to DHCA) at the time of their Norwood had different outcomes with respect to mortality, need for heart transplant, prolonged mechanical ventilation and prolonged hospital stay. These endpoints were evaluated between the two study groups (RCP vs. DHCA) from the initial study (Sano vs. BT shunt). However, the study was not designed to evaluate the difference between DHCA and RCP. This is clear because the evaluation of neurologic outcomes is not one of the primary endpoints of this present study (even though it is the main reason why surgeons may employ RCP vs. DHCA). The researchers also examined a host of other factors to determine the need for heart transplant or mortality, but only a few were statistically significant and those that were significant were not surprising. (Table 1).

Of the 549 patients qualified for inclusion in this study, 252 (45.9%) received RCP during their Norwood procedure. The information in Table 2 shows the variables that were related to the use of RCP that were statistically significant. Essentially, RCP was associated with longer CPB times, increased use of ultrafiltration and open chest after Norwood. It also showed that surgeons performing ≤ 10 Norwoods/year were more likely to use RCP.  Table 3 shows that these longer surgeries and the use of RCP had no effect on the study outcomes, which is the key point of this study. Figure 2 depicts the increasing effect no mortality of increasing RCP and DHCA times.

What do we learn from this study?  Well, when it comes to evaluating the principal difference between why a surgeon uses RCP and DHCA, which is neurologic outcomes, we learn nothing.  The “secondary” outcomes that are evaluated here show there are no differences between the use of RCP and DHCA from this dataset. The authors also emphasized the finding that surgeons who do fewer Norwoods are more likely to perform RCP during their surgeries. As a surgeon, this makes perfect sense since the cases (should) take you longer if you do fewer of them. Also surgeons that are performing the bulk of the Norwood procedure at any center in this study are likely to be at a more senior level in their career, faster, and (in general) used to performing the surgery under DHCA.  So this finding is little more than an unsurprising footnote. The inherent bias of some (especially experienced) surgeons to use DHCA vs. RCP really undermines the entire point of this study. There is no good way to parse out this factor from this dataset.

Is there really a difference in the selected outcomes in this study?  No, not from what this dataset says. But the study was not randomized (or even powered) to look at RCP vs. DHCA, so it is unclear how valid the researchers’ findings are.  And, again, there is zero mention of neurologic outcomes (not even stroke) which also is a major flaw. To break the results down to their core, we learn that during that 4 year period of the SVR trial there was no major difference between the chosen outcomes between Norwoods with RCP vs DHCA at these 15 institutions. And this fact may, or may not, be applicable to present-day practices. As usual, a better trial is needed to answer this question.

 

 

CHD Surgery May 2019

 

  1. Spontaneous Breathing Trial for Prediction of Extubation Success in Pediatric Patients Following Congenital Heart Surgery: A Randomized, Controlled Trial.

Ferreira FV, Sugo EK, Aragon DC, Carmona F, Carlotti APCP.

Pediatr Crit Care Med. 2019 May 31. doi: 10.1097/PCC.0000000000002006. [Epub ahead of print]

PMID: 31162372

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  1. Characteristics and Outcomes of Pediatric Patients Supported With Ventricular Assist Device-A Multi-Institutional Analysis.

Puri K, Anders MM, Tume SC, Cabrera AG, Heinle JS, Causey JC, Wang Y, Moffett BS, Shekerdemian LS.

Pediatr Crit Care Med. 2019 May 31. doi: 10.1097/PCC.0000000000001966. [Epub ahead of print]

PMID: 31162368

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  1. Surgical Age and Morbidity After Arterial Switch for Transposition of the Great Arteries.

Ahlström L, Odermarsky M, Malm T, Ramgren JJ, Hanseus K, Liuba P.

Ann Thorac Surg. 2019 May 29. pii: S0003-4975(19)30711-8. doi: 10.1016/j.athoracsur.2019.04.033. [Epub ahead of print]

PMID: 31152730

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Select item 31153874

 

  1. Met-Hemoglobin Is a Biomarker for Poor Oxygen Delivery in Infants Following Surgical Palliation.

Swartz MF, Makhija P, Rubenstein J, Henrichs KF, Powers KS, Wang HY, Simon BV, Alfieris GM, Blumberg N, Cholette JM.

World J Pediatr Congenit Heart Surg. 2019 May 29:2150135119852327. doi: 10.1177/2150135119852327. [Epub ahead of print]

PMID: 31142197

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Select item 31136004

 

  1. The association between feeding protocol compliance and weight gain following high-risk neonatal cardiac surgery.

Furlong-Dillard JM, Miller BJ, Sward KA, Neary AI, Hardin-Reynolds TL, Jeffers G, Clay BA, Truong DT, Miller TA, Jones CE, Lambert LM, Bailly DK.

Cardiol Young. 2019 May 28:1-8. doi: 10.1017/S1047951119000222. [Epub ahead of print]

PMID: 31133078

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Select item 31136098

 

  1. Systemic atrioventricular valve replacement in patients with functional single ventricle.

Nakata T, Hoashi T, Shimada M, Ozawa H, Higashida A, Kurosaki K, Ichikawa H.

Semin Thorac Cardiovasc Surg. 2019 May 28. pii: S1043-0679(19)30009-7. doi: 10.1053/j.semtcvs.2019.05.019. [Epub ahead of print]

PMID: 31150828

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  1. Surgical repair of tetralogy of Fallot with absent pulmonary valve: favorable long term results.

Dorobantu DM, Stoicescu C, Tulloh RM, Stoica SC.

Semin Thorac Cardiovasc Surg. 2019 May 28. pii: S1043-0679(19)30166-2. doi: 10.1053/j.semtcvs.2019.05.022. [Epub ahead of print]

PMID: 31150827

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Select item 31147145

 

  1. Abnormal aortic flow profiles persist after aortic valve replacement in the majority of patients with aortic valve disease: how model-based personalized therapy planning could improve results. A pilot study approach.

Nordmeyer S, Hellmeier F, Yevtushenko P, Kelm M, Lee CB, Lehmann D, Kropf S, Berger F, Falk V, Knosalla C, Kuehne T, Goubergrits L.

Eur J Cardiothorac Surg. 2019 May 25. pii: ezz149. doi: 10.1093/ejcts/ezz149. [Epub ahead of print]

PMID: 31131388

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Select item 31129614

 

  1. Laparoscopic liver resection for hepatocellular carcinoma in Fontan-associated chronic liver disease. The first case report.

Angelico R, Lisignoli V, Monti L, Pariante R, Grimaldi C, Saffioti MC, Gagliardi MG, Spada M.

Int J Surg Case Rep. 2019 May 23;59:144-147. doi: 10.1016/j.ijscr.2019.05.029. [Epub ahead of print]

PMID: 31146196 Free PMC Article

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Select item 31038380

 

  1. Thyroid Hormone (Triiodothyronine) Therapy in Children after Congenital Heart Surgery: A Meta-analysis.

Flores S, Loomba RS, Checchia PA, Graham EM, Bronicki RA.

Semin Thorac Cardiovasc Surg. 2019 May 22. pii: S1043-0679(19)30168-6. doi: 10.1053/j.semtcvs.2019.05.020. [Epub ahead of print]

PMID: 31128253

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Select item 31112654

 

  1. Change in N-terminal pro B-type natriuretic peptide levels and clinical outcomes in children undergoing congenital heart surgery.

Gupta RK, Zheng H, Cui Y, Qu J, Li L, Liang H, Li K, Chen X, Xia H.

Int J Cardiol. 2019 May 15;283:96-100. doi: 10.1016/j.ijcard.2019.02.025. Epub 2019 Feb 14.

PMID: 30833104

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Select item 30826194

 

  1. Relief of anteriorly translocated pulmonary artery compression with a Nuss bar.

Kim H, Chan Sung S, Choi KH, Son BS, Doo Lee H, Kim G, Ko H.

Ann Thorac Surg. 2019 May 11. pii: S0003-4975(19)30686-1. doi: 10.1016/j.athoracsur.2019.04.015. [Epub ahead of print]

PMID: 31085169

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Select item 31079070

 

  1. Functional outcomes in children with anatomically repaired transposition of the great arteries with regard to congenital ventricular septal defect and coronary pattern.

Häcker AL, Reiner B, Oberhoffer R, Hager A, Ewert P, Müller J.

Arch Dis Child. 2019 May 11. pii: archdischild-2018-316444. doi: 10.1136/archdischild-2018-316444. [Epub ahead of print]

PMID: 31079070

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  1. Tracheobronchomalacia: Does One Size Fit All?

Beckerman Z, Mery CM.

Semin Thorac Cardiovasc Surg. 2019 May 8. pii: S1043-0679(19)30150-9. doi: 10.1053/j.semtcvs.2019.05.003. [Epub ahead of print] No abstract available.

PMID: 31077809

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Select item 31066078

 

  1. Efficacy and Safety of Tranexamic Acid in Pediatric Patients Undergoing Cardiac Surgery: A Single-Center Experience.

Zhang Y, Zhang X, Wang Y, Shi J, Yuan S, Duan F, Wang Y, Zhang Z, Jia Y, Gong J, Li L, Yan F.

Front Pediatr. 2019 May 7;7:181. doi: 10.3389/fped.2019.00181. eCollection 2019.

PMID: 31134172 Free PMC Article

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Select item 31130278

 

  1. RACHS – ANZ : A Modified Risk Adjustment in Congenital Heart Surgery Model for Outcome Surveillance in Australia and New Zealand.

McSharry B, Straney L, Alexander J, Gentles T, Winlaw D, Beca J, Millar J, Shann F, Wilkins B, Numa A, Stocker C, Erickson S, Slater A; Australian and New Zealand Intensive Care Society Paediatric Study Group and Centre for Outcomes and Resource Evaluation.

J Am Heart Assoc. 2019 May 7;8(9):e011390. doi: 10.1161/JAHA.118.011390.

PMID: 31039662 Free PMC Article

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  1. Health care-associated infections are associated with increased length of stay and cost but not mortality in children undergoing cardiac surgery.

Tweddell S, Loomba RS, Cooper DS, Benscoter AL.

Congenit Heart Dis. 2019 May 6. doi: 10.1111/chd.12779. [Epub ahead of print]

PMID: 31059210

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Select item 31071333

 

  1. Optimization of heart allocation: The transplant risk score.

Jasseron C, Legeai C, Jacquelinet C, Nubret-Le Coniat K, Flécher E, Cantrelle C, Audry B, Bastien O, Dorent R.

Am J Transplant. 2019 May;19(5):1507-1517. doi: 10.1111/ajt.15201. Epub 2019 Jan 7.

PMID: 30506840

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Select item 30106198

 

  1. Complicated coarctation repair: The importance of three-dimensional cross-sectional imaging in late postoperative assessment.

Garcia AF, Ahmed R, Nyktari E, Daubeney P, Voges I.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):178-181. doi: 10.4103/apc.APC_62_18.

PMID: 31143052 Free PMC Article

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  1. Postoperative Acute Kidney Injury in Young Adults With Congenital Heart Disease.

Fuhrman DY, Nguyen LG, Sanchez-de-Toledo J, Priyanka P, Kellum JA.

Ann Thorac Surg. 2019 May;107(5):1416-1420. doi: 10.1016/j.athoracsur.2019.01.017. Epub 2019 Feb 11.

PMID: 30763561

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Select item 30982719

 

  1. Prosthetic pulmonary valve and conduit endocarditis in congenital heart disease.

Rebollal-Leal F, Felipe-Abella R, Gutierrez-García F, A Mestres C, Bautista-Hernandez V.

Asian Cardiovasc Thorac Ann. 2019 May;27(4):265-270. doi: 10.1177/0218492319832769. Epub 2019 Feb 21.

PMID: 30791694

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Select item 30340821

 

  1. Long-term outcome after atrial correction for transposition of the great arteries.

Couperus LE, Vliegen HW, Zandstra TE, Kiès P, Jongbloed MRM, Holman ER, Zeppenfeld K, Hazekamp MG, Schalij MJ, Scherptong RWC.

Heart. 2019 May;105(10):790-796. doi: 10.1136/heartjnl-2018-313647. Epub 2018 Nov 10.

PMID: 30415204

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Select item 30377261

 

  1. National trends in Fontan operation and in-hospital outcomes in the USA.

Akintoye E, Miranda WR, Veldtman GR, Connolly HM, Egbe AC.

Heart. 2019 May;105(9):708-714. doi: 10.1136/heartjnl-2018-313680. Epub 2018 Oct 30.

PMID: 30377261

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Select item 30368486

 

  1. Palliative Operations for Cyanotic Congenital Heart Disease with Severely Asymmetrical Pulmonary Arteries.

He Y, Yang Y, Zhu Y, Li Z, Su J, Xu Y, Fan X, Liu Y, Fan X.

Heart Lung Circ. 2019 May 1. pii: S1443-9506(19)30349-X. doi: 10.1016/j.hlc.2019.04.010. [Epub ahead of print]

PMID: 31147189

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Select item 30528449

 

  1. Biomarkers associated with 30-day readmission and mortality after pediatric congenital heart surgery.

Parker DM, Everett AD, Stabler ME, Vricella L, Jacobs ML, Jacobs JP, Thiessen-Philbrook H, Parikh CR, Brown JR.

J Card Surg. 2019 May;34(5):329-336. doi: 10.1111/jocs.14038. Epub 2019 Apr 3.

PMID: 30942505

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Select item 30924563

 

  1. An alternative technique for completion of the total cavopulmonary connection.

Talwar S, Siddharth CB, Rajashekar P, Sengupta S, Sharma S, Gharde P, Choudhary SK, Airan B.

J Card Surg. 2019 May;34(5):236-238. doi: 10.1111/jocs.14037. Epub 2019 Mar 29.

PMID: 30924563

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Select item 30868648

 

  1. Early results of ring-reinforced conduit and curved porcine patch in Sano-Norwood procedure.

Yıldırım Ö, Bakhshaliyev S, Kilercik H, Balaban İ, Zübarioğlu U, Konukoğlu O, Sever K, Özyüksel A.

J Card Surg. 2019 May;34(5):279-284. doi: 10.1111/jocs.14016. Epub 2019 Mar 14.

PMID: 30868648

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Select item 30868639

 

  1. Anomalous coronary sinus: A predictor of challenging redo mitral surgery.

Prestipino F, D’Ascoli R, Miele M, Luzi G.

J Card Surg. 2019 May;34(5):348-349. doi: 10.1111/jocs.14017. Epub 2019 Mar 14.

PMID: 30868639

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Select item 30866121

 

  1. Surgical repair of aortic coarctation in pediatric age: A single center two decades experience.

Padalino MA, Bagatin C, Bordin G, Tua L, Francescato A, Pradegan N, Piperata A, Vida VL, Castaldi B, Boccuzzo G, Stellin G.

J Card Surg. 2019 May;34(5):256-265. doi: 10.1111/jocs.14019. Epub 2019 Mar 13.

PMID: 30866121

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Select item 30795967

 

  1. A Propensity-Score Matched Analysis on Outcomes Using Recombinant Activated Factor VII in Pediatric Cardiac Surgery.

Li Y, Zhao W, Luo Q, Wu X, Ding J, Yan F.

J Cardiothorac Vasc Anesth. 2019 May;33(5):1269-1275. doi: 10.1053/j.jvca.2018.12.016. Epub 2018 Dec 12.

PMID: 30795967

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Select item 30527630

 

  1. Optimal Position of Inferior Vena Cava Cannula in Pediatric Cardiac Surgery: A Prospective, Randomized, Controlled, Double-Blind Study.

Seino Y, Ohashi N, Imai H, Baba H.

J Cardiothorac Vasc Anesth. 2019 May;33(5):1253-1259. doi: 10.1053/j.jvca.2018.10.023. Epub 2018 Oct 23.

PMID: 30527630

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Select item 30883033

 

  1. Adrenal insufficiency in neonates undergoing cardiopulmonary bypass and postoperative hypothalamic-pituitary-adrenal function after prophylactic glucocorticoids.

Tang A, Rosenfeld CR, Mikhael M, McPhaul MJ, Koch JD.

J Perinatol. 2019 May;39(5):640-647. doi: 10.1038/s41372-019-0344-7. Epub 2019 Mar 12.

PMID: 30867544

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Select item 30741756

 

  1. [Three-dimensional virtual and printed models improve preoperative planning and promote patient-safety in complex congenital and pediatric cardiac surgery].

Király L.

Orv Hetil. 2019 May;160(19):747-755. doi: 10.1556/650.2019.31393. Hungarian.

PMID: 31055963

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Select item 30758099

 

  1. Pediatric ventricular assist devices: Bridge to a new era of perioperative care.

Navaratnam M, Maeda K, Hollander SA.

Paediatr Anaesth. 2019 May;29(5):506-518. doi: 10.1111/pan.13609.

PMID: 30758099

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Select item 30714261

 

  1. Recent achievements and future developments in neonatal cardiopulmonary bypass.

Bojan M.

Paediatr Anaesth. 2019 May;29(5):414-425. doi: 10.1111/pan.13597. Epub 2019 Mar 21.

PMID: 30714261

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Select item 30710405

 

  1. Impact of Partnership Between Children’s HeartLink and IQIC Database With a Pediatric Cardiology and Cardiovascular Surgery Center in Brazil.

Croti UA, Murakami AN, De Marchi CH, Borim BC, Dearani JA, Overman D, Hickey P, Jenkins K.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):270-275. doi: 10.1177/2150135118825151.

PMID: 31084314

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Select item 31084310

 

  1. Perfusion Study Helps in the Management of the Intraseptal Course of an Anomalous Coronary Artery.

Agati S, Secinaro A, Caldaroni F, Calvaruso D, Manuri L, Gitto P, Ferro G, Anderson R, Vouhè PR, Zanai R, Campanella I.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):360-363. doi: 10.1177/2150135119829004.

PMID: 31084306

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Select item 31084302

 

  1. Autologous Patch Angioplasty of a Giant Right Coronary Artery Aneurysm and Coronary Stenosis in Kawasaki Disease.

Ganta S, Kavarana M.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):351-354. doi: 10.1177/2150135118825265.

PMID: 31084302

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Select item 31084300

 

  1. Micro-Costing Estimation of Workforce Needs: Will That Work for Cardiac Services in Kenya?

Nguyen NW.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):328-329. doi: 10.1177/2150135119843067. No abstract available.

PMID: 31084300

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Select item 30832541

 

  1. Retroesophageal Infracardiac Total Anomalous Pulmonary Venous Drainage.

Rodriguez ML, Caldarone CA, Toeg H, Maharajh G.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):367-369. doi: 10.1177/2150135117731893. Epub 2017 Nov 9.

PMID: 29121836

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Select item 28825386

 

  1. Neonatal Tracheal and Intracardiac Repair in a High-Risk Premature Infant Requiring Preoperative ECMO Transport.

Martin BJ, Holinski P, Noga M, El-Hakim H, Aklabi MA.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):380-383. doi: 10.1177/2150135117696490. Epub 2017 Aug 20.

PMID: 28825386

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Select item 31104665

 

  1. Characteristics and Outcomes of Pediatric Patients Supported With Ventricular Assist Device-A Multi-Institutional Analysis.

Puri K, Anders MM, Tume SC, Cabrera AG, Heinle JS, Causey JC, Wang Y, Moffett BS, Shekerdemian LS.

Pediatr Crit Care Med. 2019 May 31. doi: 10.1097/PCC.0000000000001966. [Epub ahead of print]

PMID: 31162368

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Select item 31145527

 

  1. Surgical Age and Morbidity After Arterial Switch for Transposition of the Great Arteries.

Ahlström L, Odermarsky M, Malm T, Ramgren JJ, Hanseus K, Liuba P.

Ann Thorac Surg. 2019 May 29. pii: S0003-4975(19)30711-8. doi: 10.1016/j.athoracsur.2019.04.033. [Epub ahead of print]

PMID: 31152730

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Select item 31138335

 

  1. The association between feeding protocol compliance and weight gain following high-risk neonatal cardiac surgery.

Furlong-Dillard JM, Miller BJ, Sward KA, Neary AI, Hardin-Reynolds TL, Jeffers G, Clay BA, Truong DT, Miller TA, Jones CE, Lambert LM, Bailly DK.

Cardiol Young. 2019 May 28:1-8. doi: 10.1017/S1047951119000222. [Epub ahead of print]

PMID: 31133078

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Select item 31133077

 

  1. Systemic atrioventricular valve replacement in patients with functional single ventricle.

Nakata T, Hoashi T, Shimada M, Ozawa H, Higashida A, Kurosaki K, Ichikawa H.

Semin Thorac Cardiovasc Surg. 2019 May 28. pii: S1043-0679(19)30009-7. doi: 10.1053/j.semtcvs.2019.05.019. [Epub ahead of print]

PMID: 31150828

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Select item 31134437

 

 

  1. Impact of Long-Term Support with Berlin Heart EXCOR® in Pediatric Patients with Severe Heart Failure.

Kanaya T, Ueno T, Taira M, Kido T, Okuda N, Araki K, Watanabe T, Toda K, Kuratani T, Sawa Y.

Pediatr Cardiol. 2019 May 24. doi: 10.1007/s00246-019-02108-0. [Epub ahead of print]

PMID: 31123765

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Select item 31124590

 

  1. Renal injury and recovery in pediatric patients after ventricular assist device implantation and cardiac transplant.

Hollander SA, Cantor RS, Sutherland SM, Koehl DA, Pruitt E, McDonald N, Kirklin JK, Ravekes WJ, Ameduri R, Chrisant M, Hoffman TM, Lytrivi ID, Conway J.

Pediatr Transplant. 2019 May 24:e13477. doi: 10.1111/petr.13477. [Epub ahead of print]

PMID: 31124590

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Select item 31118113

 

  1. Bidirectional Glenn for residual outflow obstruction in Tetralogy of Fallot.

Saleem K, Ahmed I, Sultan M, Haq IU, Younus U, Novick WM.

Cardiol Young. 2019 May 23:1-5. doi: 10.1017/S1047951119000866. [Epub ahead of print]

PMID: 31118113

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Select item 31120630

 

  1. A Quality Bundle to Support High-Risk Pediatric Ventricular Assist Device Implantation.

Knoll C, Chen S, Murray JM, Dykes JC, Yarlagadda VV, Rosenthal DN, Almond CS, Maeda K, Shin AY.

Pediatr Cardiol. 2019 May 13. doi: 10.1007/s00246-019-02123-1. [Epub ahead of print]

PMID: 31087144

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Select item 31087143

 

  1. Variability in donor selection among pediatric heart transplant providers: Results from an international survey.

Godown J, Kirk R, Joong A, Lal AK, McCulloch M, Peng DM, Scheel J, Davies RR, Dipchand AI, Miera O, Gossett JG.

Pediatr Transplant. 2019 May 13:e13417. doi: 10.1111/petr.13417. [Epub ahead of print]

PMID: 31081171

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  1. Adams-Stokes attack with delayed heart block after ventricular septal repair.

Chaiyakulsil C, Sirichongkolthong B.

Asian Cardiovasc Thorac Ann. 2019 May 9:218492319851380. doi: 10.1177/0218492319851380. [Epub ahead of print]

PMID: 31072104

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Select item 31070841

 

  1. Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review †.

Sinha R, Gooty V, Jang S, Dodge-Khatami A, Salazar J.

Children (Basel). 2019 May 4;6(5). pii: E67. doi: 10.3390/children6050067. Review.

PMID: 31060236 Free Article

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Select item 31085083

 

  1. Hospital Costs Related to Early Extubation After Infant Cardiac Surgery.

McHugh KE, Mahle WT, Hall MA, Scheurer MA, Moga MA, Triedman J, Nicolson SC, Amula V, Cooper DS, Schamberger M, Wolf M, Shekerdemian L, Burns KM, Ash KE, Hipp DM, Pasquali SK; Pediatric Heart Network Investigators.

Ann Thorac Surg. 2019 May;107(5):1421-1426. doi: 10.1016/j.athoracsur.2018.10.019. Epub 2018 Nov 17.

PMID: 30458158 Free PMC Article

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Select item 30359594

 

  1. Bilateral Pulmonary Artery Banding in Ebstein’s Anomaly With Circular Shunting.

Hasegawa M, Iwai S, Yamauchi S, Kugo Y, Kayatani F, Takahashi K, Kawata H.

Ann Thorac Surg. 2019 May;107(5):e317-e319. doi: 10.1016/j.athoracsur.2018.08.079. Epub 2018 Oct 22.

PMID: 30359592

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Select item 30315797

 

  1. Valve-Sparing Aortic Root Replacement in an 8-Month-Old Infant With Loeys-Dietz Syndrome.

Wisniewski K, Singer S, Kehl HG, Nawrocki P, Januszewska K, Malec E.

Ann Thorac Surg. 2019 May;107(5):e321-e323. doi: 10.1016/j.athoracsur.2018.07.094. Epub 2018 Oct 10.

PMID: 30315797

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Select item 30413485

 

  1. Extracorporeal membrane oxygenation in a low-weight infant after cardiac surgery.

Fuchigami T, Nagata N, Nishioka M, Akashige T, Fujiwara N, Nakayashiro M.

Asian Cardiovasc Thorac Ann. 2019 May;27(4):304-306. doi: 10.1177/0218492319833268. Epub 2019 Feb 24.

PMID: 30798610

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Select item 30791694

 

  1. Biomarkers associated with 30-day readmission and mortality after pediatric congenital heart surgery.

Parker DM, Everett AD, Stabler ME, Vricella L, Jacobs ML, Jacobs JP, Thiessen-Philbrook H, Parikh CR, Brown JR.

J Card Surg. 2019 May;34(5):329-336. doi: 10.1111/jocs.14038. Epub 2019 Apr 3.

PMID: 30942505

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Select item 30932229

 

  1. Physiological Responses to Exercise in Pediatric Heart Transplant Recipients.

Bovard JM, DE Souza AM, Harris KC, Human DG, Hosking MCK, Potts JE, Armstrong K, Sandor GGS, Cote AT.

Med Sci Sports Exerc. 2019 May;51(5):850-857. doi: 10.1249/MSS.0000000000001889.

PMID: 30629048

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Select item 31043758

 

  1. Pediatric ventricular assist devices: Bridge to a new era of perioperative care.

Navaratnam M, Maeda K, Hollander SA.

Paediatr Anaesth. 2019 May;29(5):506-518. doi: 10.1111/pan.13609.

PMID: 30758099

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Select item 30414333

 

  1. Cefoxitin Prophylaxis During Pediatric Cardiac Surgery: Retrospective Exploration of Postoperative Trough Levels.

Ricci Z, Benegni S, Cies JJ, Marinari E, Haiberger R, Garisto C, Cairoli S, Di Chiara L, Goffredo B.

Pediatr Infect Dis J. 2019 May;38(5):484-489. doi: 10.1097/INF.0000000000002179.

PMID: 30169486

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Select item 31090165

 

  1. Airway compression after pulmonary artery banding.

Nakashima K, Kurosaki K, Ichikawa H, Hoashi T, Shiraishi I.

Pediatr Int. 2019 May;61(5):509-511. doi: 10.1111/ped.13825. Epub 2019 May 14. No abstract available.

PMID: 31087585

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Select item 30719878

 

  1. Gestational Age, Birth Weight, and Outcomes Six Years After the Norwood Procedure.

Miller TA, Ghanayem NS, Newburger JW, McCrindle BW, Hu C, DeWitt AG, Cnota JF, Tractenberg FL, Pemberton VL, Wolf MJ, Votava-Smith JK, Fifer CG, Lambert LM, Shah A, Graham EM, Pizarro C, Jacobs JP, Miller SG, Minich LL; Pediatric Heart Network Investigators.

Pediatrics. 2019 May;143(5). pii: e20182577. doi: 10.1542/peds.2018-2577. Epub 2019 Apr 12.

PMID: 30979811

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Select item 30648314

 

  1. Impact of Regional Cerebral Perfusion on Outcomes Among Neonates Undergoing Norwood Operation.

Migally K, Rettiganti M, Gossett JM, Reemtsen B, Gupta P.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):261-267. doi: 10.1177/2150135118825274.

PMID: 31084315

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Select item 31084314

 

  1. Long-Term Fate of the Neoaortic Root After Neonatal Ross Operation: A Case Series.

Pradegan N, Castaldi B, Azzolina D, Stellin G, Vida VL.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):364-366. doi: 10.1177/2150135119840023.

PMID: 31084313

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Select item 31084312

 

  1. Hepatic Vein Incorporation Into the Azygos System in Heterotaxy and Interrupted Inferior Vena Cava.

Montesa C, Karamlou T, Ratnayaka K, Pophal SG, Ryan J, Nigro JJ.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):330-337. doi: 10.1177/2150135119842869.

PMID: 31084312

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Select item 31084310

 

  1. Epicardial Echocardiography in Pediatric and Congenital Heart Surgery.

Stern KWD, Emani SM, Peek GJ, Geva T, Kutty S.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):343-350. doi: 10.1177/2150135119838414.

PMID: 31084307

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Select item 31084303

 

  1. Arteriovenous Hemangioma of the Mitral Valve: Successful Surgical Removal in an Infant.

Perez-Brandão C, Carvalho R, Pinto FF, Fragata J.

World J Pediatr Congenit Heart Surg. 2019 May;10(3):355-356. doi: 10.1177/2150135119833338. No abstract available.

PMID: 31084303

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Select item 31084300

 

  1. Successful management of a neonate with antenatally detected mature intrapericardial teratoma.

Arvind A, Rajeshkumar R, Thakur D, Sridhar A, Sivaprakasm MC.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):182-184. doi: 10.4103/apc.APC_77_18.

PMID: 31143053 Free PMC Article

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Select item 31143052

 

  1. Complicated coarctation repair: The importance of three-dimensional cross-sectional imaging in late postoperative assessment.

Garcia AF, Ahmed R, Nyktari E, Daubeney P, Voges I.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):178-181. doi: 10.4103/apc.APC_62_18.

PMID: 31143052 Free PMC Article

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Select item 31143051

 

  1. Postoperative cerebral oximetry monitoring helps in early detection of diminished flow in Blalock-Taussig shunt.

Rauf A, Joshi RK, Aggarwal N, Joshi R.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):169-171. doi: 10.4103/apc.APC_182_18.

PMID: 31143049 Free PMC Article

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Select item 31143048

 

  1. Improvement of cardiopulmonary function after minimally invasive surgical repair of pectus excavatum (Nuss procedure) in children.

Das BB, Recto MR, Yeh T.

Ann Pediatr Cardiol. 2019 May-Aug;12(2):77-82. doi: 10.4103/apc.APC_121_18.

PMID: 31143030 Free PMC Article

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Select item 31143029

 

 

Fetal Cardiology Featured Articles

Fetal Cardiology May 2019

 

  1. Single cell expression analysis reveals anatomical and cell cycle-dependent transcriptional shifts during heart development.

Li G, Tian L, Goodyer W, Kort EJ, Buikema JW, Xu A, Wu J, Jovinge S, Wu SM.

Development. 2019 May 29. pii: dev.173476. doi: 10.1242/dev.173476. [Epub ahead of print]

PMID: 31142541

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Select item 31144180

 

  1. Strain and dyssynchrony in fetuses with congenital heart disease compared to normal controls using speckle tracking echocardiography (STE).

Drop MV, Möllers M, Hammer K, Oelmeier de Murcia K, Falkenberg MK, Braun J, Eveslage M, Köster HA, Klockenbusch W, Steinhard J, Schmitz R.

J Perinat Med. 2019 May 29. pii: /j/jpme.ahead-of-print/jpm-2019-0073/jpm-2019-0073.xml. doi: 10.1515/jpm-2019-0073. [Epub ahead of print]

PMID: 31141490

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Select item 31142197

 

  1. Application of the INTERGROWTH-21st chart compared to customized growth charts in fetuses with left heart obstruction: late trimester biometry, cerebroplacental hemodynamics and perinatal outcome.

Graupner O, Helfrich F, Ostermayer E, Lobmaier SM, Ortiz JU, Ewert P, Wacker-Gussmann A, Haller B, Axt-Fliedner R, Enzensberger C, Abel K, Karge A, Oberhoffer R, Kuschel B.

Arch Gynecol Obstet. 2019 May 28. doi: 10.1007/s00404-019-05198-6. [Epub ahead of print]

PMID: 31139902

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Select item 31133078

 

  1. Developmental origins for semilunar valve stenosis identified in mice harboring congenital heart disease-associated GATA4 mutation.

LaHaye S, Majumdar U, Yasuhara J, Koenig SN, Matos-Nieves A, Kumar R, Garg V.

Dis Model Mech. 2019 May 28. pii: dmm.036764. doi: 10.1242/dmm.036764. [Epub ahead of print]

PMID: 31138536 Free Article

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Select item 31150828

 

  1. Inhibitor of DNA binding in heart development and cardiovascular diseases.

Hu W, Xin Y, Hu J, Sun Y, Zhao Y.

Cell Commun Signal. 2019 May 24;17(1):51. doi: 10.1186/s12964-019-0365-z. Review.

PMID: 31126344 Free PMC Article

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Select item 31019158

 

  1. The Impact of Epigenetics on Cardiovascular Disease.

Prasher D, Greenway SC, Singh RB.

Biochem Cell Biol. 2019 May 21. doi: 10.1139/bcb-2019-0045. [Epub ahead of print]

PMID: 31112654

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Select item 31112369

 

  1. Characteristics and outcomes of fetuses with laterality defects are the current outcomes better? A single center study.

Fesslova V, Pluchinotta F, Brankovic J, Boschetto C, Piazza L, Inversetti A, Sigismondi C, Giorgione V, Frigiola A.

J Matern Fetal Neonatal Med. 2019 May 20:1-8. doi: 10.1080/14767058.2019.1610737. [Epub ahead of print]

PMID: 31106637

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Select item 31107995

 

  1. [The roles and regulation mechanism of transcription factor GATA6 in cardiovascular diseases].

Sun ZQ, Yan B.

Yi Chuan. 2019 May 20;41(5):375-383. doi: 10.16288/j.yczz.19-044. Chinese.

PMID: 31106773

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Select item 31102479

 

  1. Imbalanced mitochondrial function provokes heterotaxy via aberrant ciliogenesis.

Burkhalter MD, Sridhar A, Sampaio P, Jacinto R, Burczyk MS, Donow C, Angenendt M, Investigators CNFCHD, Hempel M, Walther P, Pennekamp P, Omran H, Lopes SS, Ware SM, Philipp M.

J Clin Invest. 2019 May 16;130. pii: 98890. doi: 10.1172/JCI98890.

PMID: 31094706 Free Article

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Select item 30885417

 

  1. Fetal cardiac abnormalities: genetic etiologies to be considered.

Petracchi F, Sisterna S, Igarzabal L, Wilkins-Haug L.

Prenat Diagn. 2019 May 13. doi: 10.1002/pd.5480. [Epub ahead of print]

PMID: 31087396

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Select item 31092183

 

  1. Fetal whole-heart 4D imaging using motion-corrected multi-planar real-time MRI.

van Amerom JFP, Lloyd DFA, Deprez M, Price AN, Malik SJ, Pushparajah K, van Poppel MPM, Rutherford MA, Razavi R, Hajnal JV.

Magn Reson Med. 2019 May 12. doi: 10.1002/mrm.27798. [Epub ahead of print]

PMID: 31081250

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Select item 31085169

 

  1. EPAS 1, congenital heart disease, and high altitude: disclosures by genetics, bioinformatics, and experimental embryology.

Sergi C.

Biosci Rep. 2019 May 10;39(5). pii: BSR20182197. doi: 10.1042/BSR20182197. Print 2019 May 31.

PMID: 31015364 Free PMC Article

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Select item 31109778

 

  1. Navigating the non-coding genome in heart development and Congenital Heart Disease.

Chahal G, Tyagi S, Ramialison M.

Differentiation. 2019 May 8;107:11-23. doi: 10.1016/j.diff.2019.05.001. [Epub ahead of print] Review.

PMID: 31102825 Free Article

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Select item 31069588

 

  1. Mortality and morbidity of major congenital heart disease related to general prenatal screening for malformations.

Lytzen R, Vejlstrup N, Bjerre J, Petersen OB, Leenskjold S, Dodd JK, Jørgensen FS, Søndergaard L.

Int J Cardiol. 2019 May 7. pii: S0167-5273(18)36295-8. doi: 10.1016/j.ijcard.2019.05.017. [Epub ahead of print]

PMID: 31130278

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Select item 31047007

 

  1. Maternal Viral Infection and Risk of Fetal Congenital Heart Diseases: A Meta-Analysis of Observational Studies.

Ye Z, Wang L, Yang T, Chen L, Wang T, Chen L, Zhao L, Zhang S, Zheng Z, Luo L, Qin J.

J Am Heart Assoc. 2019 May 7;8(9):e011264. doi: 10.1161/JAHA.118.011264.

PMID: 30995883 Free PMC Article

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Select item 31076139

 

  1. Maternal Occupational Oil Mist Exposure and Birth Defects, National Birth Defects Prevention Study, 1997⁻2011.

Siegel M, Rocheleau CM, Johnson CY, Waters MA, Lawson CC, Riehle-Colarusso T, Reefhuis J; National Birth Defects Prevention Study.

Int J Environ Res Public Health. 2019 May 4;16(9). pii: E1560. doi: 10.3390/ijerph16091560.

PMID: 31060207 Free PMC Article

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Select item 31064677

 

  1. Brain and CSF Volumes in Fetuses and Neonates with Antenatal Diagnosis of Critical Congenital Heart Disease: A Longitudinal MRI Study.

Claessens NHP, Khalili N, Isgum I, Ter Heide H, Steenhuis TJ, Turk E, Jansen NJG, de Vries LS, Breur JMPJ, de Heus R, Benders MJNL.

AJNR Am J Neuroradiol. 2019 May;40(5):885-891. doi: 10.3174/ajnr.A6021. Epub 2019 Mar 28.

PMID: 30923087

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Select item 30797558

 

  1. Response to the letter about the article “Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?”

Amedro P, Vincenti M.

Arch Cardiovasc Dis. 2019 May;112(5):365-366. doi: 10.1016/j.acvd.2019.04.001. Epub 2019 Apr 11. No abstract available.

PMID: 30982719

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Select item 30979689

 

  1. Letter in response to the article entitled “Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?” by Vincenti et al.

Séguéla PE, Thomas J, Thambo JB.

Arch Cardiovasc Dis. 2019 May;112(5):363-364. doi: 10.1016/j.acvd.2019.03.002. Epub 2019 Apr 9. No abstract available.

PMID: 30979689

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Select item 30971399

 

  1. Early fetal echocardiography.

McBrien A, Hornberger LK.

Birth Defects Res. 2019 May 1;111(8):370-379. doi: 10.1002/bdr2.1414. Epub 2018 Nov 14. Review.

PMID: 30430770

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Select item 30957841

 

  1. Lessons from exome sequencing in prenatally diagnosed heart defects: A basis for prenatal testing.

Westphal DS, Leszinski GS, Rieger-Fackeldey E, Graf E, Weirich G, Meitinger T, Ostermayer E, Oberhoffer R, Wagner M.

Clin Genet. 2019 May;95(5):582-589. doi: 10.1111/cge.13536. Epub 2019 Mar 28.

PMID: 30868567

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Select item 30947872

 

  1. Identification of clinically actionable variants from genome sequencing of families with congenital heart disease.

Alankarage D, Ip E, Szot JO, Munro J, Blue GM, Harrison K, Cuny H, Enriquez A, Troup M, Humphreys DT, Wilson M, Harvey RP, Sholler GF, Graham RM, Ho JWK, Kirk EP, Pachter N, Chapman G, Winlaw DS, Giannoulatou E, Dunwoodie SL.

Genet Med. 2019 May;21(5):1111-1120. doi: 10.1038/s41436-018-0296-x. Epub 2018 Oct 8.

PMID: 30293987

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Select item 30661039

 

  1. Downregulation of microRNA-592 protects mice from hypoplastic heart and congenital heart disease by inhibition of the Notch signaling pathway through upregulating KCTD10.

Pang XF, Lin X, Du JJ, Zeng DY.

J Cell Physiol. 2019 May;234(5):6033-6041. doi: 10.1002/jcp.27190. Epub 2018 Nov 27.

PMID: 30478832

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Select item 30760879

 

  1. Novel mutations of AXIN2 identified in a Chinese Congenital Heart Disease Cohort.

Zhu MJ, Ma XY, Ding PC, Tang HF, Peng R, Lu L, Li PQ, Qiao B, Yang XY, Zheng YF, Wang HY, Gao YQ, Chen FS.

J Hum Genet. 2019 May;64(5):427-435. doi: 10.1038/s10038-019-0572-x. Epub 2019 Feb 13.

PMID: 30760879

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Select item 31034441

 

  1. Fetal echocardiography (ECHO) in assessment of structural heart defects in congenital diaphragmatic hernia patients: Is early postnatal ECHO necessary for ECMO candidacy?

Style CC, Olutoye OO, Verla MA, Lopez KN, Vogel AM, Lau PE, Cruz SM, Espinoza J, Fernandes CJ, Keswani SG, Lee TC.

J Pediatr Surg. 2019 May;54(5):920-924. doi: 10.1016/j.jpedsurg.2019.01.022. Epub 2019 Feb 20.

PMID: 30954228

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Select item 30826117

 

  1. Association of NKX2-5, GATA4, and TBX5 polymorphisms with congenital heart disease in Egyptian children.

Behiry EG, Al-Azzouny MA, Sabry D, Behairy OG, Salem NE.

Mol Genet Genomic Med. 2019 May;7(5):e612. doi: 10.1002/mgg3.612. Epub 2019 Mar 4.

PMID: 30834692 Free PMC Article

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Select item 30896870

 

  1. Maternal and fetal genetic effects on birth weight and their relevance to cardio-metabolic risk factors.

Warrington NM, Beaumont RN, Horikoshi M, Day FR, Helgeland Ø, Laurin C, Bacelis J, Peng S, Hao K, Feenstra B, Wood AR, Mahajan A, Tyrrell J, Robertson NR, Rayner NW, Qiao Z, Moen GH, Vaudel M, Marsit CJ, Chen J, Nodzenski M, Schnurr TM, Zafarmand MH, Bradfield JP, Grarup N, Kooijman MN, Li-Gao R, Geller F, Ahluwalia TS, Paternoster L, Rueedi R, Huikari V, Hottenga JJ, Lyytikäinen LP, Cavadino A, Metrustry S, Cousminer DL, Wu Y, Thiering E, Wang CA, Have CT, Vilor-Tejedor N, Joshi PK, Painter JN, Ntalla I, Myhre R, Pitkänen N, van Leeuwen EM, Joro R, Lagou V, Richmond RC, Espinosa A, Barton SJ, Inskip HM, Holloway JW, Santa-Marina L, Estivill X, Ang W, Marsh JA, Reichetzeder C, Marullo L, Hocher B, Lunetta KL, Murabito JM, Relton CL, Kogevinas M, Chatzi L, Allard C, Bouchard L, Hivert MF, Zhang G, Muglia LJ, Heikkinen J; EGG Consortium, Morgen CS, van Kampen AHC, van Schaik BDC, Mentch FD, Langenberg C, Luan J, Scott RA, Zhao JH, Hemani G, Ring SM, Bennett AJ, Gaulton KJ, Fernandez-Tajes J, van Zuydam NR, Medina-Gomez C, de Haan HG, Rosendaal FR, Kutalik Z, Marques-Vidal P, Das S, Willemsen G, Mbarek H, Müller-Nurasyid M, Standl M, Appel EVR, Fonvig CE, Trier C, van Beijsterveldt CEM, Murcia M, Bustamante M, Bonas-Guarch S, Hougaard DM, Mercader JM, Linneberg A, Schraut KE, Lind PA, Medland SE, Shields BM, Knight BA, Chai JF, Panoutsopoulou K, Bartels M, Sánchez F, Stokholm J, Torrents D, Vinding RK, Willems SM, Atalay M, Chawes BL, Kovacs P, Prokopenko I, Tuke MA, Yaghootkar H, Ruth KS, Jones SE, Loh PR, Murray A, Weedon MN, Tönjes A, Stumvoll M, Michaelsen KF, Eloranta AM, Lakka TA, van Duijn CM, Kiess W, Körner A, Niinikoski H, Pahkala K, Raitakari OT, Jacobsson B, Zeggini E, Dedoussis GV, Teo YY, Saw SM, Montgomery GW, Campbell H, Wilson JF, Vrijkotte TGM, Vrijheid M, de Geus EJCN, Hayes MG, Kadarmideen HN, Holm JC, Beilin LJ, Pennell CE, Heinrich J, Adair LS, Borja JB, Mohlke KL, Eriksson JG, Widén EE, Hattersley AT, Spector TD, Kähönen M, Viikari JS, Lehtimäki T, Boomsma DI, Sebert S, Vollenweider P, Sørensen TIA, Bisgaard H, Bønnelykke K, Murray JC, Melbye M, Nohr EA, Mook-Kanamori DO, Rivadeneira F, Hofman A, Felix JF, Jaddoe VWV, Hansen T, Pisinger C, Vaag AA, Pedersen O, Uitterlinden AG, Järvelin MR, Power C, Hyppönen E, Scholtens DM, Lowe WL Jr, Davey Smith G, Timpson NJ, Morris AP, Wareham NJ, Hakonarson H, Grant SFA, Frayling TM, Lawlor DA, Njølstad PR, Johansson S, Ong KK, McCarthy MI, Perry JRB, Evans DM, Freathy RM.

Nat Genet. 2019 May;51(5):804-814. doi: 10.1038/s41588-019-0403-1. Epub 2019 May 1.

PMID: 31043758

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Select item 31053406

 

  1. Recessive mutations in proximal I-band of TTN gene cause severe congenital multi-minicore disease without cardiac involvement.

Ge L, Fu X, Zhang W, Wang D, Wang Z, Yuan Y, Nonaka I, Xiong H.

Neuromuscul Disord. 2019 May;29(5):350-357. doi: 10.1016/j.nmd.2019.03.007. Epub 2019 Mar 14.

PMID: 31053406

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Select item 30796469

 

  1. An integrated in utero MR method for assessing structural brain abnormalities and measuring intracranial volumes in fetuses with congenital heart disease: results of a prospective case-control feasibility study.

Griffiths PD, Mousa HA, Finney C, Mooney C, Mandefield L, Chico TJA, Jarvis D.

Neuroradiology. 2019 May;61(5):603-611. doi: 10.1007/s00234-019-02184-2. Epub 2019 Feb 22.

PMID: 30796469 Free PMC Article

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Select item 31055963

 

  1. The role of GPCR signaling in cardiac Epithelial to Mesenchymal Transformation (EMT).

Nebigil CG, Désaubry L.

Trends Cardiovasc Med. 2019 May;29(4):200-204. doi: 10.1016/j.tcm.2018.08.007. Epub 2018 Aug 22. Review.

PMID: 30172578

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Select item 30381848

 

  1. Dynamic fetal cardiac magnetic resonance imaging in four-chamber view using Doppler ultrasound gating in normal fetal heart and in congenital heart disease: comparison with fetal echocardiography.

Tavares de Sousa M, Hecher K, Yamamura J, Kording F, Ruprecht C, Fehrs K, Behzadi C, Adam G, Schoennagel BP.

Ultrasound Obstet Gynecol. 2019 May;53(5):669-675. doi: 10.1002/uog.20167.

PMID: 30381848

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Select item 31084317

 

  1. Application of the INTERGROWTH-21st chart compared to customized growth charts in fetuses with left heart obstruction: late trimester biometry, cerebroplacental hemodynamics and perinatal outcome.

Graupner O, Helfrich F, Ostermayer E, Lobmaier SM, Ortiz JU, Ewert P, Wacker-Gussmann A, Haller B, Axt-Fliedner R, Enzensberger C, Abel K, Karge A, Oberhoffer R, Kuschel B.

Arch Gynecol Obstet. 2019 May 28. doi: 10.1007/s00404-019-05198-6. [Epub ahead of print]

PMID: 31139902

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Select item 31133078

 

  1. Aortic Coarctation: A Comprehensive Analysis of Shape, Size, and Contractility of the Fetal Heart.

DeVore GR, Jone PN, Satou G, Sklansky M, Cuneo BF.

Fetal Diagn Ther. 2019 May 27:1-11. doi: 10.1159/000500022. [Epub ahead of print]

PMID: 31132773

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Select item 31147145

 

  1. Fetal XCMR: a numerical phantom for fetal cardiovascular magnetic resonance imaging.

Roy CW, Marini D, Segars WP, Seed M, Macgowan CK.

J Cardiovasc Magn Reson. 2019 May 23;21(1):29. doi: 10.1186/s12968-019-0539-2.

PMID: 31118056 Free PMC Article

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Select item 31120904

 

  1. Characteristics and outcomes of fetuses with laterality defects are the current outcomes better? A single center study.

Fesslova V, Pluchinotta F, Brankovic J, Boschetto C, Piazza L, Inversetti A, Sigismondi C, Giorgione V, Frigiola A.

J Matern Fetal Neonatal Med. 2019 May 20:1-8. doi: 10.1080/14767058.2019.1610737. [Epub ahead of print]

PMID: 31106637

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Select item 31112709

 

  1. Isl1-expressing non-venous cell lineage contributes to cardiac lymphatic vessel development.

Maruyama K, Miyagawa-Tomita S, Mizukami K, Matsuzaki F, Kurihara H.

Dev Biol. 2019 May 18. pii: S0012-1606(18)30183-0. doi: 10.1016/j.ydbio.2019.05.002. [Epub ahead of print]

PMID: 31112709 Free Article

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Select item 31133431

 

  1. Prenatal Detection, Comorbidities, and Management of Vascular Rings.

Young AA, Hornberger LK, Haberer K, Fruitman D, Mills L, Noga M, Tham E, McBrien A.

Am J Cardiol. 2019 May 15;123(10):1703-1708. doi: 10.1016/j.amjcard.2019.02.030. Epub 2019 Feb 23.

PMID: 30876659

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Select item 31102384

 

  1. Fetal heart rhabdomyomatosis: a single-center experience.

Pavlicek J, Klaskova E, Kapralova S, Prochazka M, Vrtel R, Gruszka T, Kacerovsky M.

J Matern Fetal Neonatal Med. 2019 May 15:1-7. doi: 10.1080/14767058.2019.1613365. [Epub ahead of print]

PMID: 31032681

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Select item 30892143

 

  1. Coronary Intimal Thickening Begins in Fetuses and Progresses in Pediatric Population and Adolescents to Atherosclerosis.

Roberto GG, Rocío C, Gabriel C, Francisco A, Giuseppe A, José M.

Angiology. 2019 May 14:3319719849784. doi: 10.1177/0003319719849784. [Epub ahead of print]

PMID: 31088126

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Select item 31076408

 

  1. The non-genetic paternal factors for congenital heart defects: A systematic review and meta-analysis.

Peng J, Meng Z, Zhou S, Zhou Y, Wu Y, Wang Q, Wang J, Sun K.

Clin Cardiol. 2019 May 10. doi: 10.1002/clc.23194. [Epub ahead of print]

PMID: 31073996 Free Article

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Select item 31109778

 

  1. Prenatal diagnosis of left pulmonary artery sling and review of literature.

Sezer S, Acar DK, Ekiz A, Kaya B, Bornaun H, Aslan H.

Echocardiography. 2019 May;36(5):1001-1004. doi: 10.1111/echo.14325. Epub 2019 Apr 9.

PMID: 30968436

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Select item 30934142

 

  1. Fetal and postnatal echocardiographic imaging of transposition of the great arteries with the aortic valve posterior to the pulmonary valve.

Castellanos DA, McKenzie ED, Morris SA.

Echocardiography. 2019 May;36(5):1005-1008. doi: 10.1111/echo.14292. Epub 2019 Feb 23.

PMID: 30801777

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Select item 30988757

 

  1. Evaluation of Fetal Left Ventricular Size and Function Using Speckle-Tracking and the Simpson Rule.

DeVore GR, Klas B, Satou G, Sklansky M.

J Ultrasound Med. 2019 May;38(5):1209-1221. doi: 10.1002/jum.14799. Epub 2018 Sep 23.

PMID: 30244474

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Select item 30208238

 

  1. Speckle Tracking of the Basal Lateral and Septal Wall Annular Plane Systolic Excursion of the Right and Left Ventricles of the Fetal Heart.

DeVore GR, Klas B, Satou G, Sklansky M.

J Ultrasound Med. 2019 May;38(5):1309-1318. doi: 10.1002/jum.14811. Epub 2018 Sep 12.

PMID: 30208238

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Select item 30553676

 

 

Congenital Cardiovascular Anesthesia and Critical Care Featured Articles

CHD Anesthesia May 2019

 

  1. Use of Continuous Dexmedetomidine at Home for Nearly Three Years in a Young Woman with End-Stage Cyanotic Congenital Heart Disease and Cyclic Vomiting.

Thienprayoon R, Meyer M, Flint H, Weidner N, Hirsch R.

J Palliat Med. 2019 May 6. doi: 10.1089/jpm.2018.0516. [Epub ahead of print]

PMID: 31058565

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Select item 30252317

 

  1. Risk associated with anesthesia for noncardiac surgery in children with congenital heart disease.

Taylor D, Habre W.

Paediatr Anaesth. 2019 May;29(5):426-434. doi: 10.1111/pan.13595. Epub 2019 May 2.

PMID: 30710405

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Select item 30614162

 

  1. Tetralogy of Fallot: Everything you wanted to know but were afraid to ask.

Wise-Faberowski L, Asija R, McElhinney DB.

Paediatr Anaesth. 2019 May;29(5):475-482. doi: 10.1111/pan.13569. Epub 2019 Apr 15.

PMID: 30592107

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Select item 30414333

 

  1. Current understanding and perioperative management of pediatric pulmonary hypertension.

Latham GJ, Yung D.

Paediatr Anaesth. 2019 May;29(5):441-456. doi: 10.1111/pan.13542. Epub 2018 Dec 19.

PMID: 30414333

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Select item 30365221

 

  1. Use of antifibrinolytics in pediatric cardiac surgery: Where are we now?

Faraoni D, Rahe C, Cybulski KA.

Paediatr Anaesth. 2019 May;29(5):435-440. doi: 10.1111/pan.13533. Epub 2018 Nov 18.

PMID: 30365221

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Select item 31058784

 

  1. Modified Lung Ultrasound Examinations in Assessment and Monitoring of Positive End-Expiratory Pressure-Induced Lung Reaeration in Young Children With Congenital Heart Disease Under General Anesthesia.

Wu L, Hou Q, Bai J, Zhang J, Sun L, Tan R, Zhang M, Zheng J.

Pediatr Crit Care Med. 2019 May;20(5):442-449. doi: 10.1097/PCC.0000000000001865.

PMID: 31058784

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Select item 30676491

 

  1. Therapeutic Hypothermia After Perinatal Asphyxia in Infants With Severe, Ductal-Dependent Congenital Heart Disease.

Boos V, Tietze A, Berger F, Bührer C.

Pediatr Crit Care Med. 2019 May;20(5):457-465. doi: 10.1097/PCC.0000000000001878.

PMID: 30676491

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Select item 30652195

 

  1. Perioperative echocardiography in children: Is there a role for pediatric cardiac anesthesiologists?

Ramamoorthy C, Lopez C.

Paediatr Anaesth. 2019 May;29(5):401-402. doi: 10.1111/pan.13622. No abstract available.

PMID: 31099468

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Select item 30934152

 

  1. Extubation Failure Rates After Pediatric Cardiac Surgery Vary Across Hospitals.

Rooney SR, Donohue JE, Bush LB, Zhang W, Banerjee M, Pasquali SK, Gaies MG.

Pediatr Crit Care Med. 2019 May;20(5):450-456. doi: 10.1097/PCC.0000000000001877.

PMID: 30807544

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Select item 30676491

 

  1. Reduction in Unintended Extubations in a Level IV Neonatal Intensive Care Unit.

Galiote JP, Ridoré M, Carman J, Zell L, Brant K, Gayle C, Short BL, Klugman D, Soghier L.

Pediatrics. 2019 May;143(5). pii: e20180897. doi: 10.1542/peds.2018-0897.

PMID: 31028159

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Select item 31023830

 

 

 

The CHIP Network Journal Watch Team

Konstantin Averin, MD, MS is an Associate Professor of Pediatrics at the University of Alberta and an Interventional Pediatric Cardiologist at the Stollery Children’s Hospital in Edmonton, Alberta. He received his medical degree from the Feinberg School of Medicine at Northwestern University in Chicago, IL. After medical school, he completed pediatrics residency, pediatric cardiology fellowship and sub-specialty training in pediatric and adult interventional cardiology at the Cincinnati Children’s Hospital in Cincinnati, OH. His clinical and research interests are focused on the percutaneous treatment of pediatric patients with congenital and acquired heart disease with a focus on patients with single ventricle physiology, transcatheter pulmonary valves, and pulmonary hypertension.

Dr. Leong Ming Chern “MC” is an adult congenital heart disease specialist at the National Heart Institute, Kuala Lumpur, Malaysia. He received his medical training at the University of Malaya and pediatric cardiology training at the National Heart Institute. His area of interest includes treatment of adult patients with congenital heart disease and pulmonary hypertension in congenital heart disease.

Dr. Damien Cullington, MBChB MRCP MD FESC is a consultant adult congenital cardiologist who works at Leeds General Infirmary, UK. In summer 2019, he will move over to the newly commissioned North West ACHD Service based in Liverpool, UK. Damien qualified in 2002 from the University of Liverpool and became a substantive consultant in 2016. Prior to this, he worked throughout the North of England and Yorkshire as a cardiology trainee. Damien was awarded a doctorate in medicine (MD) from the University of Hull in 2013 for his work in heart rate in patients with heart failure. He is a member of the Royal College of Physicians (London), Fellow of the European Society of Cardiology and honorary senior lecturer for the University of Leeds. His ACHD subspecialty clinical interests are heart failure, imaging and palliative care. He is regional organiser for ACHD training at Leeds and clinical governance lead for the Leeds congenital cardiac unit. His research interests and wide and far but particularly epidemiology, chronic heart failure in ACHD patients in all its guises, the systemic RV and the univentricle

Dr. Blanche Cupido is a consultant adult cardiologist working at Groote Schuur Hospital, UCT Private Academic Hospital and the University of Cape Town(UCT), South Africa. She completed her physician training in 2009 and her cardiology subspecialist training in 2013. She recently returned to SA after doing a Fellowship in Adult Congenital Heart Disease in Leeds, United Kingdom under the guidance of Dr Kate English. She has established a dedicated unit for Grown Up Congenital Heart Disease in Cape Town, South Africa. Her aim is to grow ACHD services in Sub-Saharan Africa and embark on GUCH research on the African continent.

Dr. Jeremy L. Herrmann is an Assistant Professor of Surgery in the Division of Thoracic and Cardiovascular Surgery at Indiana University. He specializes in pediatric and adult congenital cardiac surgery, and his clinical interests also include heart transplantation and mechanical circulatory support. His hospital affiliations include Indiana University Health Riley Hospital for Children and Methodist Hospital as well as Peyton Manning Children’s Hospital at St. Vincent Hospital in Indianapolis

Maan Jokhadar is a cardiologist and associate professor of medicine at Emory University in Atlanta, Georgia. He is board certified in internal medicine, cardiovascular disease, advanced heart failure/transplantation, adult congenital heart disease, and echocardiography. He is fellowship director for the Emory Adult Congenital Heart Disease training program and Core Curriculum Director for the Emory general cardiology fellowship program. Dr Jokhadar graduated from the University Damascus School of Medicine in Syria and then went to Mayo Clinic in Rochester, Minnesota for internal medicine residency. He then completed cardiology and subspecialty training at Emory University, where he currently on faculty. Dr. Jokhadar has been the recipient of numerous teaching awards. He is married with 3 children.

Michael Ma, MD is an Assistant Professor in Cardiothoracic Surgery in the Division of Pediatric Cardiac Surgery at Stanford University. He specializes in pediatric and adult congenital cardiac surgery, with an emphasis on neonates, complex biventricular repair, and pulmonary artery reconstruction. His research lab investigates ex and in vivo translational models for complex congenital heart disease, to optimize future surgical and endovascular repair strategies.

Jeremy P. Moore MD MS FHRS is the Director of Clinical Research and faculty in Pediatric Cardiology and Adult Congenital Heart Disease at the University of California, Los Angeles. Dr. Moore received his medical degree from the Medical College of Virginia in 2003. He completed residency and fellowship at UCLA before pursuing his subspecialty training in Pediatric Electrophysiology at Vanderbilt University in 2009. Dr. Moore has been faculty at UCLA since 2010 and has since published numerous research manuscripts dealing with electrophysiologic aspects pertinent to congenital heart disease. Dr. Moore’s primary interest is the study of mechanisms of arrhythmia, and the development of novel electrophysiologic techniques for management of the adult patient with congenital heart disease

Dr. Mehul Patel MD, is a structural and adult congenital heart disease specialist and an interventional cardiologist. Dr. Patel earned his undergraduate and medical education from the Mumbai University, India. After completing his post-graduate training in Internal Medicine and Cardiology, he further trained in interventional cardiology at the Mount Sinai Medical Center, NYC, NY and Adult Congenital Heart Disease at the Texas Children’s Hospital, Texas Heart Institute, Baylor College of Medicine, Houston, TX. Dr. Patel worked as Chief of adult congenital heart disease, Assistant Professor at the Michigan State University, Grand Rapids, MI where he not only expanded the program, performing complex interventions and device implantations but also established the percutaneous pulmonary valve implantation (Melody Valve) Program. Due to his passion for treating structurally abnormal hearts, he did a dedicated year of Structural Heart Disease fellowship at Henry Ford Hospital, Detroit, MI where he worked with pioneers in this field before moving to North Carolina. He is proficient in performing transcatheter aortic valve replacement (TAVR), MitraClip, Watchman device implantation, percutaneous Mitral, Tricuspid and Pulmonary valve replacements along with a variety of interventions on congenital heart disease and pulmonary hypertension. Dr. Patel is ABIM board certified in Internal Medicine, Cardiology, Interventional cardiology and Adult Congenital Heart Disease. Dr. Patel has more than 50 peer reviewed publications and numerous abstracts to his credit. He serves as a Co-Editor-in-Chief for the CHiP Network journal watch. His wife, Khyati is a pediatric cardiac Electrophysiologist and they have a 9-year-old daughter. Dr. Patel is also an artist, likes music, yoga and plays badminton. His clinical areas of expertise and interests are: 1) Transcatheter therapies for Structural and Adult Congenital heart disease. 2) Complex device implantations.

Dr. Timothy Pirolli is an Assistant Professor of Surgery in the Division of Pediatric Cardiothoracic Surgery at University of Texas Southwestern Medical Center. He specializes in pediatric and adult congenital cardiac surgery. His hospital affiliations include Children’s Medical Center in Dallas, Parkland Memorial Hospital, and Clements University Hospital (UTSW).

Dr. Inga Voges, M.D. Consultant in Pediatric Cardiology, Lead Consultant Cardiovascular MRI. I trained in General Pediatrics and Pediatric Cardiology in Rostock (Germany) and Kiel (Germany) from 2002-2010 and did additional training in Adult Congenital Heart Disease (ACHD) in Kiel (Germany) which I finished in 2014. I specialized in Cardiovascular Magnetic Resonance Imaging (CMR) and have a further special interest in cardiomyopathies. Currently, I am working as a consultant pediatric and ACHD cardiologist at the University Hospital Schleswig-Holstein (Kiel, Germany) where I am responsible for the CMR imaging program in pediatric and ACHD patients and contribute to the care of patients with acquired and congenital heart disease. I received my MD from the Medical University Luebeck in 2006 (Germany) and finished my “Habilitation” (PhD equivalent; University Hospital Kiel, Germany) in 2014. Since 2016, I am the secretary of the Association for European Paediatric and Congenital Cardiology Imaging Working Group.

Dr. Gary Webb, M.D. is an Emeritus Professor of Pediatrics and Internal Medicine at the University of Cincinnati College of Medicine and,
from 2009-2016, the Director of the Adult Congenital Heart Program at Cincinnati Children’s Hospital Heart Institute. A graduate of McGill
University in Montréal, he interned at the Royal Victoria Hospital, and then trained in internal medicine and cardiology at the University of Toronto. From 1980-2004, he was co-director and then director of the Toronto Congenital Cardiac Center for Adults at Toronto General Hospital. He is a Fellow of the Royal College of Physicians and Surgeons of Canada in both internal medicine and cardiology. From 2004-2009, he was director of the Philadelphia Adult Congenital Heart Center at the University of Pennsylvania. Since 2016, he has been a consultant to Cincinnati Children’s Hospital, and has been responsible for the ACHD Learning Center, the Cardiology Fellow Testing Center, and the Congenital Heart Professionals International (CHIP) Network. Since 2017, he has resumed seeing patients on a part-time basis at the Toronto Congenital Cardiac Centre for Adults.

Wendy Whiteside, MD Wendy is Assistant Professor of Pediatrics and Associate Director of Interventional Pediatric Cardiology at the University of Michigan Congenital Heart Center, C.S. Mott Children’s Hospital. She obtained her medical degree from Albert Einstein College of Medicine in 2006 then completed pediatric residency at Children’s Hospital Oakland in Oakland, CA in 2009. She received
both her categorical and interventional pediatric cardiology training at the University of Michigan in Ann Arbor, MI. Her clinical and research interests include single ventricle physiology, transcatheter
pulmonary valves, and quality improvement within the cardiac catheterization laboratory.

About the Congenital Heart and Pediatric Cardiac Journal Watch

Congenital heart and pediatric cardiac Journal Watch was designed to make it easier for congenital heart and pediatric cardiac professionals to keep up with the literature in 6 subsections of congenital heart disease abstracts on a monthly basis: pediatric cardiology; congenital heart surgery; congenital  heart interventions; congenital/pediatric electrophysiology; fetal cardiology; and adult congenital heart disease.

We encourage your continued support as well as your valuable comments and feedback.

Learn More

Sincerely,

Dr. Gary Webb, MD and Dr. Mehul Patel
CHiP Network
Congenital Heart and Pediatic Cardiac Journal Watch Editorial Board

The Chip Network, the Congenital Heart International Professionals Network, aims to develop a single global list of all congenital and pediatric cardiac professionals.

We invite you to share this with your colleagues.

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