Pediatric Cardiology

What is the Significance of Elevated Troponin I in Children and Adolescents? A Diagnostic Approach

What is the Significance of Elevated Troponin I in Children and Adolescents? A Diagnostic Approach. Yoldaş T, Örün UA. Pediatr Cardiol. 2019 Dec;40(8):1638-1644. doi: 10.1007/s00246-019-02198-w. Epub 2019 Sep 4. PMID: 31485699   Take Home Points: A single-center evaluation of children with no known prior heart disease, who presented with elevated troponin I, showed association with diverse cardiac and noncardiac pathologies. The most frequent cause of elevated troponin I is myopericarditis (46%). Perimyocarditis is associated with higher peak troponin I levels and takes a long time to normalize. Most frequent non-cardiac causes include drug intoxication, carbon-monoxide poisoning. Intensive inhalation beta-agonist use in acute asthma and lower respiratory tract infections. A careful history, physical examination, Electrocardiogram, and Echocardiogram to evaluate function should form the basis for differentiating cardiac and noncardiac etiologies. CT angiography and Magnetic Resonance Imaging were performed in few patients. Unlike adults, coronary involvement is rare and cardiac catheterization is not routinely warranted except when rare pathology is suspected.   Commentary from Dr. Venu Amula MD (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: Troponin I is an important cardiac biomarker and its elevation in adult patients signifies coronary artery disease. Myocardial injury and elevated troponin, though rare in children, create considerable anxiety among pediatric providers. In the current study, Yoldas et al aim to evaluate the significance of elevated Troponin I in a retrospective cohort of children less than 18 years of age admitted to a single-center between 2007 – 2018. They excluded children with a history of congenital heart disease and those requiring or having undergone cardiac surgery within the previous year. Neonates with birth asphyxia and sepsis were also excluded. For those who met inclusion criteria demographics, clinical and other diagnostic data were abstracted from medical records and analyzed using descriptive statistics.   Troponin elevation was defined as >0.06 ng/ml per the institute's laboratory standard. Of the patients with elevated Troponin I, ECG findings, echocardiography and degree of troponin elevation were evaluated along with the final diagnosis sorted by cardiac and non-cardiac etiologies. The peak troponin levels were also compared between those with cardiac and noncardiac etiologies.   They screened 972 patient records and found 759 subjects meeting the study criteria. Evaluation of troponin was done for the most common indication of chest pain and syncope within 2 weeks of presentation. The most common cardiac causes of raised troponin were myopericarditis (46%), perimyocarditis (18%), cardiomyopathy (15%), followed by dysrhythmias (12%). The most common indication for performing troponin I test in the non-cardiac group was to investigate cardiac involvement in drug intoxications. The other reasons were carbon monoxide Poisoning and respiratory symptoms. Patients with myopericarditis and perimyocarditis had distinct characteristics of troponin elevation. Patients with perimyocarditis had higher peak troponins and also took a long time to negative troponin, not surprising given the predominance of myocardial inflammation.   The study provides a framework for differential diagnosis of elevated troponin I testing in children with no congenital heart disease. Providers should be wary of the implications of routine testing of troponin I levels. A careful review of history, indication for testing, physical examination findings should be corroborated by ECG and echocardiogram to interpret the elevated troponin I levels. Short of this, providers may produce an undue burden of anxiety for patients and themselves.         

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Human Milk Use in the Preoperative Period Is Associated with a Lower Risk for Necrotizing Enterocolitis in Neonates with Complex Congenital Heart Disease

Human Milk Use in the Preoperative Period Is Associated with a Lower Risk for Necrotizing Enterocolitis in Neonates with Complex Congenital Heart Disease. Cognata A, Kataria-Hale J, Griffiths P, Maskatia S, Rios D, O'Donnell A, Roddy DJ, Mehollin-Ray A, Hagan J, Placencia J, Hair AB. J Pediatr. 2019 Dec;215:11-16.e2. doi: 10.1016/j.jpeds.2019.08.009. Epub 2019 Sep 24. PMID: 31561958 Similar articles   Take Home Points: In infants with isolated critical congenital heart lesions, the incidence of preoperative necrotizing enterocolitis (NEC) was 3.3% and developed at a median of 5 days after initiation of feeds. Large volume feeding (>100ml/kg/d) of infants with critical heart disease in the preoperative period is associated with a 3-fold increase in the risk of NEC Infants who are exclusively fed with unfortified human milk diet had a significantly lower risk of preoperative NEC.   Commentary from Dr. Venu Amula (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: In this retrospective, single-center cohort study of infants of any gestational age with isolated critical congenital heart lesion between January 2010 and January 2016, the authors evaluated whether large feeding volumes (> 100ml/kg/d) and exposure to cow's milk formula increased the risk of preoperative Necrotizing Enterocolitis (NEC). NEC was defined based on the modified Bell criteria. The cardiac lesions included were those with a high risk of NEC - ductal dependent lesions, transposition of great arteries, truncus arteriosus, aortopulmonary window, severe Ebstein's anomaly and tetralogy of Fallot with absent PV. Infants were excluded if they were admitted at >72 hours of age or had heterotaxy, major gastrointestinal anomalies or hypoxic-ischemic encephalopathy. Infants with cardiac anomalies that did not require intervention were also excluded. Demographic data were collected for all the infants who met inclusion criteria. Feeding data included date feeds were initiated; type of feeds provided; the largest volume of feeds (mL/kg/d) provided; and the feeding route that was used (oral vs nasogastric).   The association of volume of feeds (> 100ml/kg/d or less) and type of formula (cow's milk) with the incidence of NEC in the preoperative period was evaluated in a multivariable logistic regression model that was built by including clinically relevant and all other risk factors found to be significantly associated with NEC (P = .05) in univariate analysis.   After screening 878 infants 546 were found to be eligible for the study. The incidence of NEC was 3.3% before the surgery and developed a median of 5 days after feeds were started. Large volume feeds defined as feeding volume more than 100 ml/kg/day was associated with an increased risk of NEC. Exclusive unfortified human milk diet was protective, with a significantly lower risk for NEC when controlled for other risk factors like feeding volume, birth weight small for gestational age, race, inotropic support preoperatively/pre-NEC, prematurity (gestational age <37 weeks), and cardiac lesion. Infants with biventricular ductal dependent blood flow had increased risk of NEC but were probably confounded by the fact that they were younger, had long wait times before surgery and were fed high volume, nonhuman milk diet.   The study is limited by being an evaluation of a single-center practice and outcomes and as such the results may not be generalizable, however, the sample size is large and contained decent case-mix severity. Like other studies on necrotizing enterocolitis, the definition of NEC and Bell staging, especially for stage 1 ( 8 out of 18) is subjective as acknowledged by the authors but every effort was taken to standardize. Whether these risk factors apply to severe stages of NEC is unclear due to the rarity of the outcomes in comparison to sample size when analyzed separately. The study suggests that caution should be exercised in the preoperative period when feeding infants with critical heart lesions of any gestational age regardless of the type and ductal dependency. Feeding volumes < 100ml/kg/d and using unfortified human milk diet seem to decrease the risk of this GI complication with major morbidity.        

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Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations

Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations. Frommelt PC, Hu C, Trachtenberg F, Baffa JM, Boruta RJ, Chowdhury S, Cnota JF, Dragulescu A, Levine JC, Lu J, Mercer-Rosa L, Miller TA, Shah A, Slesnick TC, Stapleton G, Stelter J, Wong P, Newburger JW. Circ Cardiovasc Imaging. 2019 Feb;12(2):e007865. doi: 10.1161/CIRCIMAGING.118.007865. PMID: 30755054 Similar Articles   Take Home Points: Shunt type has no significant impact on echocardiographic indices of right ventricle, neoaortic, and tricuspid valve size and function after the first palliation by 6 years of life. Decreases in RV function present in the RV to pulmonary artery shunt seen in the previous SVR trial were no longer present, suggesting ongoing RV remodeling with time. Future incorporation of this data with newer echo modalities, MRI results, and quality of life measures (self-reported or even exercise testing data as these patients get older) may give us even more beneficial data for this population.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: This is the most recent update from the Pediatric Heart Network SVR trial comparing 549 infants with single RV anomalies randomly assigned to a modified Blalock-Taussig shunt (MBTS) or RV to pulmonary artery shunt (RVPAS) at 15 centers in North America. The goal was to determine if shunt type was associated with better clinical outcome. Previous SVR trial articles showed a better 1 year transplant free survival in the RVPAS group, but this survivor benefit was no longer present at 3 years of life. Similarly, the early improved RV ejection fraction (RVEF) in the RVPAS group diminished over time, and in fact, at 3 years, the RVEF was worse in the RVPAS compared to the MBTS group.   This surveillance time point included those patients who had an echocardiogram performed within 6 months of Fontan palliation and at 6 years of life. 2D and Doppler indices of RV size and function were assessed, as well as neoaortic and tricuspid valve size and function. Exclusions included patients who underwent cardiac transplantation, a few who had biventricular repair, and those that did not consent to the extension trial. The specific echocardiographic and core lab procedures were described in earlier reports and unchanged for this study.   241 studies were analyzed with 116 MBTS and 125 RVPAS; 208 of those at undergone a pre-Fontan echo and 228 also had an acceptable echo at 14 months. In the overall cohort at the 6 year compared to the pre-Fontan echo, RV systolic and diastolic volumes and indexed areas decreased significantly, and RVEF, FAC, and peak tricuspid annular systolic velocity increased significantly (Table 2). When compared to the 14 month study, 6 year RV systolic and diastolic volume was significantly decreased, but RVEF increased nonsignificantly (P=0.06). When comparing interval changes by group, RVEF and FAC increased significantly in the RVPAS group vs. the MBTS group (Figure 2). There was an increase in MPI by PW Doppler (and not by tissue Doppler) over time (14 month to post-Fontan), but no difference at 6 years between groups. However, the increase was only significant for the MBTS group when comparing pre-Fontan and 6 years.   Neoaortic annular areas were larger in MBTS vs. RVPAS, but both were significant large compared to normal (z-score 6.4 and 5.4 respectively). Greater than mild regurgitation was rare and similar between groups. There was a significant decrease in size when comparing the 14 month and 6 year studies (no difference from pre-Fontan to 6 years). Indexed tricuspid annular areas and z-scores were similar in both groups at 6 years. Greater than moderate TR occurred in 17% of subjects in each group. There was significant decrease in annular size between 14 months and 6 years, but no difference from pre-Fontan to 6 years.   The authors also evaluated the 14 month RVEF and the relationship to late death/transplant. There were 43 deaths/transplants in the 337 patients who had 14 month echocardiograms. Decreased RVEF (<40%) had a hazard ratio of 3.18 for death/transplant. Shunt type had no effect.   This study at 6 years of follow up shows no difference in outcomes (echocardiographic and clinical) between shunt groups. Based on changes at this time point vs. the earlier time point, RV remodeling has occurred resulting in equivocal RV systolic and diastolic function regardless of shunt type. There have been concerns about the RV ventriculotomy and resultant injury/scarring potentially affecting RV performance, but this was not seen by 6 years. Additionally, the RVPAS group showed a similar survival/transplant free rate to the MBTS group at 6 years, despite the initial lower death/transplant-free survival benefit up to stage 2, and then an increase in death until the Fontan, showing that there was some beneficial change that occurred with time particularly in this group. The study confirms the lack of significant neoaortic and tricuspid valve issues in this younger age cohort, even with the marked increase in annular size compared to normal patients. Additionally, RVEF < 40% at 14 months remains a significant risk factor for death/transplant. It is important to note that the RVPAS group had a significantly higher incidence of catheter-based interventions, and it is possible that the improved hemodynamics may have contributed to the improved RV function. Multiple required interventions may have a “familial” and quality of life affect that has not been assessed.   Limitations of the study were clearly delineated. RV function is very difficult to evaluate in general by echo, and in particular in those with a single RV. Rudimentary LV mechanical effects on the single RV were also not assessed (and typically cannot be when only using echocardiographic tools). The addition of newer echocardiographic modalities such as strain and 3D assessment of the tricuspid valve, as well as MRI data, may be very helpful moving forward. There is some discussion that MRI data will be included in the next follow up report as the patients reach 10-12 years of age.   An accompanying editorial provides an additional question that is important to consider. Are there outcomes besides RV function that should be assessed? Health related quality of life measures and a better assessment of functional status (patient and parental reporting as well as future exercise data as these patients get older and are able to be tested on a cycle ergometer or treadmill) may provide a better functional insight into how these patients are over time.   Table 2 (cut):     

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Mortality and Resource Use Following Cardiac Interventions in Children with Trisomy 13 and Trisomy 18 and Congenital Heart Disease

Mortality and Resource Use Following Cardiac Interventions in Children with Trisomy 13 and Trisomy 18 and Congenital Heart Disease. Domingo L, Carey JC, Eckhauser A, Wilkes J, Menon SC. Pediatr Cardiol. 2019 Feb;40(2):349-356. doi: 10.1007/s00246-018-2001-x. Epub 2018 Oct 5. PMID: 30291384 Similar Articles Select Item 30288600   Take Home Points: As expected, overall resource use is higher for patients with T13 and T18 who undergo surgery for cardiac lesions. T13 had a higher mortality, tracheostomies, and gastrostomies than T18. While there is increased risk for morbidity and mortality in this patient population, outcomes may be reasonable for a select cohort to undergo cardiac intervention.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Intervention, especially cardiac surgery, for patients with Trisomy 13 (T13) and Trisomy 18 (T18), has historically rarely been considered or offered due to a high 1 year mortality rate. There seems to be some change in practice over the past few years, possibly influenced by parental desire to “do everything” when these patients are carried to term. As a majority of patients will have CHD, cardiologists are often intimately involved beginning in fetal life. Recent surveys have shown practice variabilities amongst different groups of physicians (e.g. neonatologists vs. cardiologists), but also depending on the type of cardiac lesion. This study was done to describe the clinical outcomes and resource use in those patients who underwent cardiac intervention using a large multicenter database of 45 pediatric hospitals.   Data included demographics, ICD-9 coding, and resource utilization information. Children were excluded if they had additional genetic defects, admitted for non-cardiac indication, or older than age 5 at the time of first cardiac intervention. CHD was categorized as a shunt lesion, left-sided obstruction, conotruncal defect, or single ventricle physiology. Interventions including diagnostic and catheter based interventions, palliative procedures, or complete surgical repair. RACHS and other demographic and historical information were reviewed. Mortality and resource use (total hospital charges adjusted to 2014 US Dollars) were the primary outcomes.   189 patients with T13 and T18 underwent cardiac intervention between 1999-2015. 26% had T13 and 74% T18. There were 238 hospitalizations for cardiac interventions, with 18% of T13 and 14% of T18 undergoing more than one intervention. A higher number of T13 had a tracheostomy or G-tube than T18 (12% vs. 4% and 18% vs. 6%, respectively). Shunt and conotruncal lesions were most common in both groups, but more T13 patients had single ventricle physiology. The majority of interventions were complete surgical repairs. 40% of the interventions occurred in the south US region, with more than half on government insurance. (See Table 1).   T18 patients were significantly older than T13 patients at time of initial intervention (5 vs. 2.5 months). T13 had longer median hospital LOS, but this was not statistically significant. Total median hospital charge was $305,990 for T13 and $252,780 for T18 (also not statistically significant). Mortality rate following surgery was high for both groups, but significantly higher in T13 (29% vs. 12%). Overall in-hospital mortality between groups was not statistically significant (T13 33% and T18 22%). (See Table 2). Regression modeling showed improved survival for white race in T13 patients and older age at initial intervention for T18. Tracheostomy or other comorbidities, cardiopulmonary bypass, type of cardiac lesion, and type of intervention did not affect postprocedural survival.   The authors note that ~70% of patients overall were discharged home with improved symptoms, suggesting a clear clinical benefit for a majority of patients. Compared to previous studies, the age at initial intervention was younger, suggesting a shift towards more aggressive medical intervention. The data also show a clear trend towards complete repair than palliative repair in the US. However, median costs were 1.5-2 times more and hospital LOS 2-3 times longer than those without these trisomies. While type of lesion was not associated with survival, the numbers are probably too low for some lesions to affect current decision making processes in most institutions (e.g. not intervening or palliating for single ventricle lesions). This data though could possibly suggest a benefit to intervening for some lesions if the costs and morbidity are acceptable. Additionally, long-term survival and quality of life data in those who underwent intervention have not yet been elucidated, so individual treatment plans and ethics teams will likely be necessary as we move forward. However, this study should hopefully lead to more discussion at pediatric hospitals as to how best to manage this challenging patient population.       

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Factors associated with exercise capacity in patients with a systemic right ventricle

Gavotto A, Abassi H, Rola M, Serrand C, Picot MC, Iriart X, Thambo JB, Iserin L, Ladouceur M, Bredy C, Amedro P. Int J Cardiol. 2019 Jun 13. pii: S0167-5273(19)31187-8. doi: 10.1016/j.ijcard.2019.06.030. [Epub ahead of print] PMID: 31256996 Similar articles Select item 31242968 Take Home Points: NYHA functional class is a strong predictor of reduced exercise capacity in patients with a systemic right ventricle. Impaired right ventricular functional parameters are also associated with limited exercise capacity, while 2D global longitudinal strain correlated best with percent-predicted VO2max.   Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  This is a French multicenter study assessing factors related to exercise capacity in a cohort of patients with a systemic right ventricle. The authors included 111 patients with transposition of the great arteries after atrial switch operation (n= 94) and patients with congenitally corrected transposition of the great arteries (n= 17). The mean age was 37.2 ± 8.2 years. Patients who were included in this study had at least a physical examination, a cardiopulmonary exercise test (CPET), an ECG and an echocardiogram.   68.5% of the patients were in NYHA I functional class. A right ventricular assistance or cardiac transplantation project was considered in 10 patients. Conduction disorders were common (70 patients) and included sinus node dysfunction, complete right bundle branch block and complete atrioventricular block. 34 patients had supraventricular tachycardias including atrial flutter and atrial fibrillation. Echocardiographic parameters (TAPSE, S wave, TEI index, RV surface shortening fraction, global longitudinal 2D Strain) suggested RV dysfunction; 17% of the patients had severe tricuspid regurgitation.   The mean VO2max, the ventilatory anaerobic threshold and the VE/VCO2 slope were impaired (mean VO2max 23.3 ± 6.9 ml/kg/min; VO2 13.7 ± 5 ml/kg/min; VE/VCO2 slope 34 ± 8). In the univariate analysis professional status, NYHA functional class, BNP level, the type of systemic right ventricle, decreased right ventricular function values, tricuspid regurgitation, the presence of a pacemaker or an implantable defibrillator, the ventilatory anaerobic threshold, the maximum load, and the maximal heart rate during exercise correlated with VO2max (Table 4). In the multivariate analysis, VO2max correlated with NYHA functional class (Table 4). All RV functional parameters as assessed by echocardiography were related to exercise capacity (Table 4), while 2D global longitudinal strain analysis showed the best correlation.    

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Safety and Efficacy of Vasopressin After Fontan Completion: A Randomized Pilot Study

Bigelow AM, Ghanayem NS, Thompson NE, Scott JP, Cassidy LD, Woods KJ, Woods RK, Mitchell ME, Hraŝka V, Hoffman GM. Ann Thorac Surg. 2019 Dec;108(6):1865-1874. doi: 10.1016/j.athoracsur.2019.06.053. Epub 2019 Aug 7. PMID: 31400337 Similar articles Select item 31398358 Take Home Points:    Vasopressin infusion after Fontan completion was associated with reduced transpulmonary gradient and chest tube drainage in the early postoperative peri.od A larger multi-institutional study will be necessary to confirm safety and to see if this impacts length of stay and is cost-effective.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  While surgical mortality following Fontan palliation is low, postoperative morbidity is common, most often from prolonged pleural fluid drainage. This occurs in 15-40% of patients and is thought to be related to the specific physiologic changes present in the single ventricle population. Many strategies have been attempted to try to reduce chest tube (CT) output with nearly all being unsuccessful on a consistent basis. The authors hypothesized that vasopressin, through its effect on increasing systemic vascular resistance, decreasing capillary leakage, and increasing coronary and pulmonary vasodilation, may be quite suitable in this patient population. This was a small pilot study primarily done to evaluate the safety and efficacy of reducing CT output in a small prospective group of Fontan patients.   This was a randomized, double blinded, placebo controlled trial that included all planned Fontan completions except for those with a history of Fontan takedown, planned AV valvuloplasty, or arch reconstruction, or history of renal replacement therapy. All patients underwent non-fenestrated extracardiac conduit, with standard monitoring and use of inotropes (epinephrine and milrinone) post-operatively. The study drug was initiated at 0.3 mU/kg/min in the OR and continued for 21 hours. It was weaned 0.1 mU/kg/min each hour until discontinued at 24 hours. Additional fluids and titration of inotropes (including open label vasopressin) were at the discretion of the cardiac intensivist. Chest tubes were removed when draining less than 2 ml/kg for 24 hours. 20 patients were studied after a few were excluded or did not consent, with 10 in each group.   There were no differences in the vasopressin and placebo groups in terms of patient characteristics, ventricular morphology, pre-Fontan O2 saturation or hemodynamics, or surgical technique and CPB factors. CT drainage was significantly lower in the vasopressin group during the first postoperative night, POD 1, and at 48 hours after surgery. Median CT duration was 92 hours in the vasopressin group vs 114 hours in the control group. Urine output and fluid balance was similar in both groups. The vasopressin group received significantly higher milrinone dose and had a lower transpulmonary gradient on the first postoperative night. Two patients (one in each arm) received open label vasopressin due to hypotension. Median duration of vasoactive support was the same in both groups. Median hospital stay was 180 hours in the vasopressin group compared to 203 hours in the control group, but this did not meet statistical significance. See table 2, Figure 1, and Table 4.   This was an interesting small single-center RCT that suggests improved transpulmonary gradient and reduced CT output using vasopressin, with no significant safety concerns or deleterious hemodynamic consequences. The vasopressin group did receive a higher dose of milrinone likely due to increased BP; however, the authors think this is likely not a significant confounding variable based on prior studies of milrinone. As a pilot study and as mentioned by the authors, this study was not powered to detect many important clinical outcomes. While CT duration is predicated on CT output, and is likely a main factor in length of hospital stay, statistical significance in this study was not reached. A larger multicenter trial would be recommended.      

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Parental reactions, distress, and sense of coherence after prenatal versus postnatal diagnosis of complex congenital heart disease

Bratt EL, Järvholm S, Ekman-Joelsson BM, Johannsmeyer A, Carlsson SÅ, Mattsson LÅ, Mellander M. Cardiol Young. 2019 Sep 16:1-7. doi: 10.1017/S1047951119001781. [Epub ahead of print] PMID: 31522698 Similar articles Select item 31529177 Take Home Points: Parents with babies born with congenital heart disease have a high level of anxiety and depression than their matched control groups. There was no difference between the anxiety scores of parents with prenatal vs postnatal diagnosis. Life Satisfaction was lower in the parents in the prenatal group and it decreased further during follow up testing after birth, suggesting this group remained vulnerable to poor psychological health. Parents in the prenatal group had a lower sense of coherence when compared to controls and upon follow up testing continued to remain so. Parents in the postnatal group scored satisfaction with the relationship significantly higher than parents in the control group. The factors underlying this are not clear but may relate to parents coming together and making their life comprehensible, manageable and meaningful.( as evidenced by an increased sense of coherence ). Given the risk of adverse psychological health, efforts should focus on improving counseling and support during pregnancy for parents with a fetal diagnosis of congenital heart disease.   Commentary from Dr. Venugopal Amula (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch:  It is well known that parental stress during pregnancy negatively affects neonatal outcomes and can hamper infant development. In the current study, investigators from the University of Gothenburg, Sweden,  study the impact of timing of diagnosis of Congenital Heart Disease (prenatal vs postnatal ) on the psychological health of pregnant women and their partners. Specifically, depression and anxiety, sense of coherence, life satisfaction and satisfaction with partner relationship were studied. Three study groups were included those with a prenatal diagnosis of CHD in offspring (prenatal group), those with a postnatal diagnosis (post-natal group), and a group with uncomplicated pregnancies and deliveries (controls). Patients in the prenatal group were recruited after 22 completed weeks of pregnancy so as not to interfere with the decision to terminate the pregnancy ( the upper limit of GA to allow termination being 22 completed weeks in Sweden). Matching of the groups based on parental age, sex and parity and complexity of CHD was done. Previously validated instruments such as Hospital Anxiety and Depression scale, Sense of Coherence Scale, Life Satisfaction scale and Dyadic Adjustment scale were used. Testing at Time 1 included prenatal group and healthy controls. All three groups were tested at follow up (Time 2) between 2-6 months after delivery.      

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Examining the Utility of Coronary Artery Lack of Tapering and Perivascular Brightness in Incomplete Kawasaki Disease

Rabinowitz EJ, Rubin LG, Desai K, Hayes DA, Tugertimur A, Kwon EN, Dhanantwari P, Misra N, Stoffels G, Blaufox AD, Mitchell E. Pediatr Cardiol. 2019 Jan;40(1):147-153. doi: 10.1007/s00246-018-1971-z. Epub 2018 Sep 8. PMID: 30196380 Similar articles Select item 30196379 Take Home Points: Lack of tapering of the coronary arteries (LT) and perivascular brightness (PB) are subjective, poorly reproducible, and not specific for Kawasaki disease (KD).. Guidelines should ideally be based on data and consensus panel recommendations should be subsequently tested for validity when possible.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  In 2017, the AHA published revised guidelines for the diagnosis of KD, including more extensive recommendations for incomplete KD. Both LT, defined as absence of the normal tapering expected in the distal coronary, and PB, defined as echogenicity/brightness of the coronary wall, which had been used in the previous guidelines, were removed due to the absence of data, with the only “suggestive of KD” coronary artery criteria being 2D measurements of at least a z-score of 2-2.5. The inclusion of these criteria were initially based on expert panels. The thought was that LT or PB may represent arteritis prior to true dilation or aneurysm formation.   This was a single center retrospective study that began prior to the new revisions. They hypothesized that both LT and PB were unreliable. 117 de-identified parasternal short axis echo clips of the coronary arteries (CA) from patients ages 0-10 years old were interpreted blindly by 6 pediatric cardiologists with at least 5 years of experience outside of training. Subjects were grouped as 1) healthy, afebrile, with benign murmurs, 2) KD s/p IVIG treatment, 4-5 clinical criteria at presentation based on chart review, 3) incomplete KD (iKD) s/p IVIG, 1-3 clinical criteria, 4) febrile > 3 days, no IVIG, KD not suspected. The KD and iKD patients were identified though a list of all patients receiving IVIG and subsequent chart review for symptoms and diagnoses. Each of the 6 reading cardiologists interpreted the same clips independently and recorded the presence or absence of LT and PB, and measured the diameters of the LMCA, LAD and RCA. At least 10 duplicate cases were selected to assess intra-observer reliability. Since the reading cardiologists were blinded, they could not calculate z-scores based on the measurements obtained.   Median ages of the groups ranged from 1.7-3.2 years. Chart review showed subungual peeling and/or CA aneurysm (z-score > 2.5) were noted in 21/30 KD and 19/32 iKD patients. CA z-score measurements showed good intra-observer agreement (reliability coefficients 0.52-0.6). In contrast, LT and PB showed poor agreement (reliability coefficients 0.36 and 0.13 respectively). See table 2. The prevalence of positive LT and PB in the healthy and febrile groups outnumbered the total in the KD and iKD groups from some of the echo readers, with the median frequency for LT 11% healthy, 53% KD, 44% iKD, and 24% febrile groups, and for PB 4% healthy, 17% KD, 14% iKD, 11% febrile groups. The rate of agreement for positive LT reads in a majority of cardiologists (e.g. 4-6) was very low. For PB, in 92% of the patients there was good agreement for a negative read, and only a single patient had a majority agreeing on a positive read (see figure 1). The intra-observer variability was low for both LT and PB (see Table 2). At least one cardiologist had a high percentage of LT and PB on all clips, suggesting “over-diagnosis” likely due to the unreliability of these findings. Review of the medical records showed that the presence of LT and/or PB played a role in the diagnosis of iKD and subsequent IVIG treatment in some patients. One of the four did have periungual peeling and none had late aneurysms. Additionally, none of the 10 iKD patients with LT or PB had refractory symptoms requiring another dose of IVIG or other medication.   Limitations of this study included its retrospective nature and a statistical difference in age between the groups. However, this study showed that LT and PB are poorly reliable, poorly reproducible, and not specific to iKD or KD. Additionally, these findings were seen in normal and febrile patients without KD, a known issue amongst cardiologists who read many echocardiograms for suspected KD (usually iKD) without enough clinical criteria to meet even incomplete diagnostic criteria, but with the pediatricians or ID folks hoping we can help them “make the diagnosis”.  As the authors note, guidelines without evidence can often be flawed. There should be future prospective studies especially in KD looking at the utility of the current guidelines and use of echocardiography in the diagnosis.    

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Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein’s Anomaly

Meierhofer C, Kühn A, Müller J, Shehu N, Hager A, Martinoff S, Stern H, Ewert P, Vogt M. Pediatr Cardiol. 2019 Apr;40(4):792-798. doi: 10.1007/s00246-019-02066-7. Epub 2019 Feb 6. PMID: 30726509 Take Home Points Exercise capacity is used to prognosticate in patients with Ebstein’s anomaly (EA) and cardiac magnetic resonance (CMR) is used to assess volumetric and functional parameters Functional parameters measured by CMR have been shown to correlate with exercise capacity and can be used in follow-up for patients with EA, possibly to predict the need for surgical intervention prior to development of symptoms   Comment from Dr. Shelby White (Tucson AZ), section editor of Pediatric Cardiology Journal Watch:  Ebstein’s anomaly (EA) results in volume overload and dysfunction of the right ventricle due to tricuspid regurgitation and atrialization of the ventricle.  Exercise capacity is used as a prognostic variable, specifically % predicted peak oxygen uptake (peak VO2%), for event free survival in EA.  CMR is used for right ventricular assessment in congenital heart disease, this study sought to evaluate variables assessed by CMR and their correlation to peak VO2%.   As seen in table 4, the majority of the parameters that correlated significantly with functional status as determined by peak VO2% were physiologic parameters rather than volumetric measurements.  These parameters which influence cardiac output may be followed over time to predict a decline in function in patients with EA.  

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Mental health care for parents of babies with congenital heart disease during intensive care unit admission: Systematic review and statement of best practice.

Kasparian NA, Kan JM, Sood E, Wray J, Pincus HA, Newburger JW. Early Hum Dev. 2019 Aug 24:104837. doi: 10.1016/j.earlhumdev.2019.104837. [Epub ahead of print] PMID: 31455569 Take Home Points: Congenital heart disease is associated with psychological stress for mothers, fathers and their infants. In four of the five trials included, mental health interventions demonstrated some efficacy in reducing maternal anxiety. Mixed results were found for maternal depression and infant feeding. Mental health interventions for parents of infants admitted to the intensive care unit (ICU) did not improve quality of life, physical health or infant length of stay. An integrated approach is needed to address mental health issues in parents of infants with CHD admitted to the intensive care unit.   Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch:  This is a systematic literature review and statement of best practice addressing  mental health interventions and resources for parents of children born with congenital heart disease admitted to the intensive care unit (ICU). The objective of this review was to identify and critically appraise evidence on the efficacy and cost-effectiveness of mental health interventions delivered in the ICU as well as to develop guidelines for advancing health policy and practice. There are many recognized psychologic and social stressors experienced by parents and infants within the ICU. Up to 30% of parents of infants with complex CHD experience symptoms consistent with post-traumatic stress disorder, another 25-50% experience depression or anxiety and up to 80% report severe psychological distress. Parents with higher distress report poorer physical health, high health service use, more suicidal ideation, and poorer quality of life. Methods included a search of 6 electronic databases. A total of 289 articles were identified but only 5 were included in this review.   Results: A total of 339 participants (311 mothers, 28 fathers) were included. Interventions varied widely across the studies included. There was high risk of bias in the 3 non-randomized controlled trials. Discussion: This systematic review of 289 articles resulted in a limited database for analysis and therefore should be approached with caution. Integrated psychotherapeutic interventions within the ICU environment are hypothesized to prevent or minimize severe psychosocial distress in parents of medically fragile infants. Four of the five identified trials demonstrated efficacy in reducing maternal anxiety; however, only a single trial demonstrated  a positive effect of psychological intervention for parental depression. Further analysis of the 5 studies included in this review did demonstrate preliminary results showing positive effects  of psychosocial interventions on maternal coping, mother-infant attachment, parenting confidence, family function, and communication. In this small review, there was no evidence for efficacy for family quality of life or intensive care unit length of stay. A single trial demonstrated some benefit for infant mental development at 6-months; however, this is difficult to generalize in a complex CHD population given the risks for neurodevelopmental abnormalities. Limitations: There were multiple limitations to include in this systematic review including small sample size, single center recruitment, and lack of blinding. Some of the included studies relied on self-administered, symptom-based measures without reference to established cut-offs to provide indications of psychological change resulting in the risk for serious bias. There was also substantial variation in the interventions provided across groups. Next steps: Mental health interventions are needed for parents of infants with CHD and should begin in the ICU. Structured psychosocial screening and a mental health assessment using validated tools is an important first step. This should be followed by an integrated and multidisciplinary approach to improve parental mental health care and therefore outcomes of this vulnerable population.  

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mDixon ECG-gated 3-dimensional cardiovascular magnetic resonance angiography in patients with congenital cardiovascular disease

Kourtidou S, Jones MR, Moore RA, Tretter JT, Ollberding NJ, Crotty EJ, Rattan MS, Fleck RJ, Taylor MD. J Cardiovasc Magn Reson. 2019 Aug 8;21(1):52. doi: 10.1186/s12968-019-0554-3. PMID: 31391061 Free PMC Article Similar articles Select item 31402138 Take Home Points: modified Dixon (mDixon) 3D cardiovascular magnetic resonance angiography (CMRA) demonstrates reduced artefact burden and better delineation of head and neck vessels as well as pulmonary veins compared to conventional 3D balanced steady state free precession CMRA. mDixon acquisitions might help improving diagnostics in pediatric and adult congenital heart disease patients.   Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:   This is a very interesting retrospective cardiovascular magnetic resonance imaging study comparing conventional contrast-enhanced cardiovascular magnetic resonance angiography (CE-CMRA) and non-contrast angiographic techniques (1. conventional 3D balanced steady state free precession CMRA; 3D bSSFP and 2. modified Dixon CMRA; mDixon). As reported by the authors, Dixon-based methods are less susceptible to field inhomogeneities. 24 cardiovascular magnetic resonance (CMR) scans were included. Each patient underwent CE-CMRA followed by 3D bSSFP and mDixon acquisitions. The water images of the mDixon sequence were used for the final analysis. CMR studies were reviewed by two independent observers who assessed the overall image quality and compared the contrast-to-noise ratio (CNR) and signal-to-noise ratio (SNR) in the myocardium and blood pool. Furthermore, vascular measurements on multiplanar reformatted images were performed and the scan time was recorded. CE-CMRA data were not included in the analysis of SNR, CNR, vascular measurements and scanning time. The authors found that the image quality of the water images of the mDixon sequence were comparable and for some structures better compared to 3DSSFP images. Bland-Altman analysis revealed acceptable agreement for image quality scores between all three techniques. mDixon images were better in depicting the entire field of view compared to 3D bSSFP and CE-CMRA and demonstrated a better vessel wall sharpness whereas 3D bSSFP images allowed a better intracardiac anatomy and myocardium-blood pool definition. There was no difference for CNR and SNR between 3D bSSFP and mDixon acquisitions. Artefact burden was less in mDixon compared to 3D bSSFP and CE-CMRA. Eight different vessel cross-sectional diameters were measured on 3D bSSFP and mDixon sequences and the analysis showed less than 10% measurement difference between both techniques (Table 3). The scanning time for 3D SSFP and mDixon acquisitions was comparable. In general, this nice study demonstrates that mDixon might be a superior to the conventionally used 3D SSFP sequence as it also offers an excellent depiction of the cardiovascular anatomy even in complex anatomies with the advantage of reduced artefact burden.    

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Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles

Biko DM, Gaynor JW, Partington SL, Harris MA, Whitehead KK, Trusty P, Yoganathan AP, Fogel M. Ann Thorac Surg. 2019 Apr 5. pii: S0003-4975(19)30496-5. doi: 10.1016/j.athoracsur.2019.03.019. [Epub ahead of print] PMID: 30959013 Similar articles Select item 30948702 Take Home Points: Single ventricle patients with previous aortic reconstruction have higher aortic pulse wave velocity (PWV) compared to single ventricle (SV) patients without aortic reconstruction. There is an inverse relationship between aortic distensibility and exercise stress test parameters in SV patients who underwent aortic reconstruction.   Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  In this very interesting prospective study, the authors examined the relationship between parameters of aortic stiffness and exercise performance in SV patients after Fontan completion. 48 SV patients underwent real-time exercise cardiovascular magnetic resonance (CMR) imaging. 18 out of 48 patients had previous aortic reconstruction. Aortic pulse wave velocity PWV, aortic distensibility, ventricular volumes as well as metabolic exercise stress test parameters were measured. Patients with previous aortic reconstruction had a higher aortic PWV compared to those without aortic reconstruction (figure 1). In addition, the authors found an inverse relationship between PWV and indexed end-diastolic volume, indexed end-systolic volume and stroke volume (at rest and during exercise) in the group of patients with aortic reconstruction. There was no significant difference in aortic distensibility between patients with a reconstructed aorta and those without aortic reconstruction. In patients with previous aortic reconstruction the authors found inverse correlations between aortic distensibility and peak O2 pulse, peak oxygen consumption (figure 2), oxygen consumption at the anaerobic threshold as well as peak work, respectively. These results might improve our understanding of decreased exercise capacity in single ventricle patients who had previous aortic reconstruction. Figure 1   Figure 2

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2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT

Hansmann G, Koestenberger M, Alastalo TP, Apitz C, Austin ED, Bonnet D, Budts W, D'Alto M, Gatzoulis MA, Hasan BS, Kozlik-Feldmann R, Kumar RK, Lammers AE, Latus H, Michel-Behnke I, Miera O, Morrell NW, Pieles G, Quandt D, Sallmon H, Schranz D, Tran-Lundmark K, Tulloh RMR, Warnecke G, Wåhlander H, Weber SC, Zartner P. J Heart Lung Transplant. 2019 Sep;38(9):879-901. doi: 10.1016/j.healun.2019.06.022. Epub 2019 Jun 21. PMID: 31495407 Similar articles Select item 31324444 Take Home Points: Pulmonary hypertension mortality has been decreasing over the last 2 decades in children and adults, likely secondary to increased awareness, more accurate diagnoses, better risk stratification and early initiation of combination pharmacotherapy. The lower limit of normal mean pulmonary arterial pressure (mPAP) was decreased from 24 mmHg to 20 mmHg. A subgroup of children with idiopathic pulmonary arterial hypertension (IPAH) are positive responders to acute vasoreactivity testing (AVT) and would be classified as PAH long-term responders to calcium channel blockers (CCB). PAH and pulmonary veno-occlusive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH) are now considered a spectrum of pulmonary vascular disease (PVD). Diagnostic methods and variables and their application to pediatric PH have been updated including echocardiography, cardiac MRI, CT and cardiac catheterization. New treatment algorithms, including medications for pediatric PH and PAH-CHD as well as drug-drug interactions are available.     Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch:  This is an updated, comprehensive and practical guideline for healthcare providers providing care to children and young adults with pulmonary hypertension (PH) and pulmonary vascular disease (PVD). Methodology included an executive writing group consisting of 22 pediatricians, 7 adult congenital heart disease physicians, 1 adult pulmonologist and 1 thoracic surgeon. Specific features of this guideline include new patient groups such as PH associated with congenital heart disease (PAH-CHD), persistent PH of the newborn period (PPHN) as well as PH associated with bronchopulmonary dysplasia (BPD) or chronic lung disease (CLD). The guidelines also include new recommendations for the treatment of acute PH in the intensive care unit (ICU). This is also the first time the challenges of addressing PH in middle to low income regions are addressed. The following class of recommendation (COR) and level of evidence (LOE) grading were used in this guideline. The most recent classification of PH according to the WSPH (Nice, 2018). The lower limit of normal mean pulmonary arterial pressure (mPAP) was decreased to 20 mmHg following a study demonstrating poor survival in adults with mildly elevated mPAP (20-24 mmHg). The diagnostic algorithm for a child or young adult with suspected PH. Treatment algorithm for pediatric PAH, including idiopathic and hereditary PAH (Figure 4 and Table 11). In acute care settings, treatment of acute PAH crises should include application of supplemental oxygen, minimization of aggravating factors (agitation, pain), treatment of triggering factors (acidosis, hypovolemia, anemia) and use of pharmacotherapy to increase myocardial perfusion, reduce right ventricular pressure afterload and counteract the right-to-left interventricular septal shift (Table 10). There are several findings to distinguish lower versus higher risk in pediatric PH and/or suspected pulmonary hypertensive vascular disease (PVHD); however, these only have a LOE C because of sparse pediatric data.   Algorithm for the management of patients with congenital shunt lesions associated with PAH or PHVD (Figure 3, Table 8). The diagnostics, monitoring (including transthoracic echocardiography, cardiac catheterization, cardiac magnetic resonance imaging, computed tomography), and genetic testing are summarized in Table 3-7. The management of special PAH populations including children and adults with CHD as well as infants with persistent bronchopulmonary dysplasia or chronic lung disease are summarized in Tables 8 & 9. The diagnosis and management of PH in middle and low income regions are summarized in Table 12.    

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Echocardiographic Assessment of Cardiac Function in Pediatric Survivors of Anthracycline-Treated Childhood Cancer

Slieker MG, Fackoury C, Slorach C, Hui W, Friedberg MK, Fan CS, Manlhiot C, Dillenburg R, Kantor P, Mital S, Liu P, Nathan PC, Mertens L. Circ Cardiovasc Imaging. 2019 Dec;12(12):e008869. doi: 10.1161/CIRCIMAGING.119.008869. Epub 2019 Dec 12. PMID: 31826678 Similar articles Select item 31766863 Take Home Points: Longitudinal strain is only mildly reduced in pediatric childhood cancer survivors (CCSs) and associated with age at the time of the study and higher body surface area. Left ventricular ejection fraction is preserved in the majority of CCSs.   Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  This large multicenter study included 546 pediatric childhood cancer survivors (CCSs)and assessed if echocardiographic strain parameters are of utility for the identification of patients at risk for cardiac dysfunction. Echocardiographic examinations included assessment and calculation of cardiac chamber size, left ventricular (LV) systolic and diastolic function and ventricular mass. Speckle tracking echocardiography was used to measure LV strain parameters. Patients were compared to 134 healthy controls.   The median age and median time from last anthracycline dose were 13.8 years and 7.9 years respectively; all patients were asymptomatic. Compared to controls, CCSs had lower LV ejection fraction (LVEF) and fractional shortening. LV dilatation was only found in 1 patient. Reduced EF with values below 50% were found in 0.8% (n=3) of all patients and 4.2% had values between 51% and 55%. Regarding parameters of diastolic function, the authors found an increased isovolumetric relaxation time in CCS compared to controls.   Global longitudinal strain (GLS) was significantly lower (Figure 1) and circumferential strain (CS) was significantly higher in patients, but the absolute difference was small for both parameters. Lower mean longitudinal strain was associated with lower LVEF in both, patients and controls (Figure 2). Patients with low LS Z-scores had significantly lower CS values.   In multivariable regression analysis age and body surface area were significantly associated with lower mean LS Z-scores. Patients who received a cumulative anthracycline dose of <150 mg/m2 compared to those with a dose >150 mg/m2, there was no difference between patients who received a dose between 150 mg/m2 -300 mg/m2 and those who received a cumulative dose >300 mg/m2.   Overall, the results are interesting, especially for the pediatric cohort of childhood cancer survivors. Figure 1 Figure 2

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Determinants of Exercise Performance in Children and Adolescents with Repaired Tetralogy of Fallot Using Stress Echocardiography

Bhatt SM, Elci OU, Wang Y, Goldmuntz E, McBride M, Paridon S, Mercer-Rosa L. Pediatr Cardiol. 2019 Jan;40(1):71-78. doi: 10.1007/s00246-018-1962-0. Epub 2018 Aug 18. PMID: 30121867 Similar articles Select item 30121864 Take Home Points: Among patients with repaired tetralogy of Fallot (rTOF), normal chronotropic response to exercise, defined as HR > 185 bpm, and those with greater HR reserve had better exercise performance and less pulmonary insufficiency at peak exercise. RV function at PI severity at rest were not associated with exercise performance. Further longitudinal studies using exercise testing should be done to determine if improved chronotropic response leads to less morbidity and mortality in this population.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Exercise performance in patients with rTOF is known to decline with age. Metabolic parameters from cardiopulmonary exercise testing (CPET) and diminished exercise capacity may predict morbidity and mortality in adults with rTOF. However, specific factors associated with this decline have not yet been elucidated, with many possibilities including pulmonary insufficiency (PI), ventricular dysfunction, chronotropic impairment, pulmonary artery stenosis/distortion, and abnormal lung function.   This was a small, but prospective, study that looked at CPET and stress echo findings in children/teenagers with rTOF. Those with pacemakers and on beta-blockers were excluded. 29 patients had CPET using a cycle ergometer or 1-min incremental treadmill protocol. Maximal exercise was achieved in all patients, based on an RER >1.10. Exercise factors such as VO2, O2 pulse, VE/VCO2 slope, peak heart rate (pHR) and heart rate reserve (HRR) were measured. Stress echo was performed immediately prior to exercise and at peak exercise, with peak RV global longitudinal strain (RV GLS), tricuspid inflow and myocardial velocity (TDI), and ratio of the diastolic to systolic time-velocity integral (DSTVI) for pulmonary insufficiency documented. 2 groups were compared and determined based on maximum VO2 (%mVO2), with VO2>80% predicted defined as high performers, and VO2<80% predicted as low performers.   The median %mVO2 in the high performers was 97.5%, with a max VO2 of 41.2 ml/kg/min, while the low performers had a median %mVO2 of 68% and a max VO2 of 30 ml/kg/min. The high performers were younger and had lower BSA. There was no difference based on genetic syndrome, type of TOF, type of repair, age at complete repair, or history of pulmonary valve replacement. See table 1. RVGLS was abnormal in both groups at baseline, and there was no difference in change or in the diastolic parameters from rest to peak exercise. There was a significant decrease in PI/DSTVI from rest to peak exercise in both groups, but the high performers had a greater reduction despite greater PI at rest. See figures 1 and 2. High performers had a greater peak HR and HRR (median 114) compared to the low performers (median 100). O2 pulse and VE/VCO2 were comparable.   As both groups had maximal effort and that O2 pulse (estimate of stroke volume) was equivalent, the authors noted that the ability to increase the HR may be a more significant contributor to augmentation of cardiac output and by extension, exercise performance. They also speculate that the decrease in PI may be due to a shorter diastolic time with increased heart rates, which is why baseline PI did not predict response. Additionally, the lack of change in strain suggests ventricular performance is not a factor in exercise performance. However, it is known that chronotropy is often a greater contributor to cardiac output in younger children than stroke volume, so it would important to know if this effect would continue with an older population.   Based on these observations, it is possible that exercise training/rehab may improve exercise capacity and potentially morbidity in this population by improving chronotropic response. As earlier studies have shown that the lowest VO2 (<15) and highest VE/VCO2 groups (>38) show a marked increase in mortality, early interventions may prevent these changes/declines. Limitations of this study included small numbers, low age and minimal residual pulmonary outflow abnormalities, so these findings may not be generalizable. Additionally, younger children are more likely to be active than older teens/young adults, and the authors did not attempt to qualitatively determine activity via parental reports or exercise logs. Further longitudinal studies should be done in this population, but it is refreshing to see good quality studies using a still somewhat underused testing modality, and one that may have more “real-world” applicability.      

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Impact of Right Ventricular Geometry and Left Ventricular Hypertrophy on Right Ventricular Mechanics and Clinical Outcomes in Hypoplastic Left Heart Syndrome

Rösner A, Bharucha T, James A, Mertens L, Friedberg MK. J Am Soc Echocardiogr. 2019 Jul 24. pii: S0894-7317(19)30767-9. doi: 10.1016/j.echo.2019.06.003. [Epub ahead of print] PMID: 31351794 Similar articles Select item 31339575 Take Home Points: In patients with hypoplastic left heart syndrome, right ventricular function is a key determinant of long-term transplant free survival. The impact of geometry of the systemic right ventricle and hypoplastic left ventricle on right ventricular mechanics is poorly understood. Right ventricular apical bulging resulting from the hypertrophied and hypoplastic left ventricle negatively impact both regional and global right ventricular systolic function (figure 2 and 3). Although left ventricular hypertrophy is often seen in the setting of mitral stenosis and aortic atresia, apical bulging was present in all anatomic subtypes. There was suggestion of association between reduced right ventricular strain and death or transplant specifically in the subset with apical bulging (figure 6).   Commentary from Dr. Shaji Menon (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: This is a retrospective cohort study of 48 hypoplastic left heart syndrome patient from the Hospital for Sick Children, Toronto with the median age of 2.2 years.  Right ventricle apical bulging defined as a sigmoid shaped septum with the right ventricle leftward apical segment contiguous with the left ventricle lateral wall.  Regional and global right ventricular strain were measured using speckle tracking echocardiography and comparisons were made between those with apical bulging and dose without apical bulging. RV global (–7.3% 6 2.8% vs –11.2% 6 4.4%; P = .001), basal septal (–3.8% 6 3.2% vs –11.4% 6 5.8%; P = .0001) and apicolateral (–5.1% 6 3.5% vs –8.0% 6 5.8%, P = .001) longitudinal strain were lower in patients with versus without apical bulging, respectively. Apical bulging was equally prevalent in all HLHS anatomical variants. Figure 1: Figure 2:

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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 Similar articles Select item 30801777 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.  

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Multicentre reference values for cardiac magnetic resonance imaging derived ventricular size and function for children aged 0-18 years.

van der Ven JPG, Sadighy Z, Valsangiacomo Buechel ER, Sarikouch S, Robbers-Visser D, Kellenberger CJ, Kaiser T, Beerbaum P, Boersma E, Helbing WA. Eur Heart J Cardiovasc Imaging. 2019 Jul 5. pii: jez164. doi: 10.1093/ehjci/jez164. [Epub ahead of print] PMID: 31280290 Similar articles Select item 31278142 Take Home Points: Cardiovascular magnetic resonance (CMR) imaging is now considered the “gold standard” imaging tool for assessment of cardiac volume and function in pediatric cardiac disease. This relatively large multicenter study provides reference values from steady-state free precession (SSFP) CMR for biventricular volumes and mass.   Commentary from Dr. Shaji Menon (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: A total of 141 healthy children (68 boys) from three European centres underwent cine-SSFP CMR imaging. Cardiac structures were manually contoured for end-diastolic and end-systolic phases in the short-axis orientation according to current standardized CMR post-processing guidelines. Volumes and masses were derived from these contours. Age-related reference curves were constructed using the lambda mu sigma method. Median age was 12.7 years (range 0.6–18.5).

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2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT

Hansmann G, Koestenberger M, Alastalo TP, Apitz C, Austin ED, Bonnet D, Budts W, D'Alto M, Gatzoulis MA, Hasan BS, Kozlik-Feldmann R, Kumar RK, Lammers AE, Latus H, Michel-Behnke I, Miera O, Morrell NW, Pieles G, Quandt D, Sallmon H, Schranz D, Tran-Lundmark K, Tulloh RMR, Warnecke G, Wåhlander H, Weber SC, Zartner P. J Heart Lung Transplant. 2019 Sep;38(9):879-901. doi: 10.1016/j.healun.2019.06.022. Epub 2019 Jun 21. PMID: 31495407 Similar articles Select item 31324444 Take-Home Points: Based on the World Symposium of Pulmonary Hypertension WSPH ( Nice, 2018) PH is now defined as mean Pulmonary Artery Pressure (mPAP) > 20mm in children > 3 months of age at sea level. The limit was decreased from 24 to 20mm hg based on adult studies where even mild elevation of mPAP between 20-24mm Hg was found to be independent predictor of poor survival. Children with suspected PH should be evaluated, treated and monitored in specialized pediatric centers. The initial evaluation should include a comprehensive medical history, physical examination, assignment to the functional class, assessment of cardiac function by ECG, Echocardiogram. Further testing and advance imaging is guided by the key findings in the history and include but are not limited to cardiac catheterization, HR-Chest CT with angiography, V/Q scan, Sleep study, Blood gas analysis, Lung function test, etc. Some determinants of higher risk in pediatric PH include clinical evidence of RV failure, progressive symptoms, presence of syncope, growth failure, severely elevated Serum NT-proBNP (> 1200 pg/ml (>1 yr. old). Transthoracic Echocardiogram (TTE) cannot establish a definite diagnosis of PH or determine WHO PH group. PH diagnosed by TTE should be confirmed by catheterization before initiation of targeted PH therapy except in infants with PPHN, BPD and those with high risk. We should rely on a multiparametric approach other than single parameters. Echo or CMR variables that are determinants of high risk include evidence of severe RA/RV enlargement, RV systolic dysfunction, RV/LV end systolic ratio of >1.5, TAPSE ¯¯ (Z-score , -3). Systolic/Diastolic ratio > 1.4 (TR jet ), Pulmonary Artery Acceleration Time PAAT < 70ms and  Pericardial effusion.   Cardiac catheterization is indicated in all pediatric patients with PH to confirm a diagnosis and determine severity, especially when considering pharmacotherapy except in infants and Low body weight or those presenting critically ill where it can be omitted until more stable. The cardiac catheterization should include Acute Vasoreactivity testing. In children with IPAH/HPAH, the hemodynamic change that defines a positive response to AVT in PH without CV shunt is > 20% fall in mPAP and PVR/SVR ratio without a decrease in CI. Invasive hemodynamics that portend high risk include Cardiac Index < 2.5. mRAP> 15, mPAP/mSAP> 0.75, PVRi> 15 Wu-m In centers equipped to perform advanced pediatric cardiac magnetic resonance imaging, CMR without anesthesia /sedation is recommended as a part of diagnostic evaluation and follow up to assess changes in ventricular function and chamber dimensions. Several Pulmonary Arterial Hypertension (PAH) associated genes have been identified and genetic testing can allow the definition of PAH etiology, estimation of prognosis and identification of families at risk. Hence genetic counseling by trained personnel before genetic testing is recommended for families with children diagnosed with Idiopathic PAH or Heritable PAH. Children who are asymptomatic PAH mutation carriers, asymptomatic first-degree relatives of patients with HPAH without an associated gene mutation should be screened by serial echocardiograms every 1-3 yrs. for the presence of elevated RV pressure and undergo additional diagnostic evaluation if clinically indicated. In patients with PAH associated with congenital heart disease a complete diagnostic workup should be performed to determine whether PAH is associated with or causally related to concomitant CHD. Children with simple shunt defects beyond the typical timing of surgery (> 6 months old ) and with severe cyanosis should undergo comprehensive right and left heart catheterization before any intervention or surgery. Standard management is adopted for PVR1< 6 Wu-m2 and PVR/SVR < 0.3. For patients in gray zone PVRi 6-8 Wu -m2 - a treat and repair approach is reasonable using PAH targeted pharmacotherapy with 1-2 medications. A shunt defect must not be closed in when PVRi > 8 Wu- m2 in children. The term and preterm newborn infant with PPHN should receive oxygen, ventilatory support, surfactant with a goal preductal sat of 91-95 %. Inhaled Nitric Oxide is indicated to improve oxygenation and to reduce the need for ECMO if PaO2 < 100 on 100% oxygen or OI index > 25. In the presence of right heart failure preterm and term infants with PPHN should receive PGE infusion to maintain ductal patency in the absence of post tricuspid unrestrictive shunt. In the pediatric ICU, oxygen is administered when the SaO2 < 95% in children with PH and normal anatomy. iNO may be considered for postoperative PH treatment in mechanically ventilated patients to improve oxygenation and reduce PHT crises. Concomitant sildenafil should be administered to prevent rebound PH while weaning iNO. Inhaled iloprost can be as effective as iNO in children with postoperative PH. The diagnosis and management of PH in middle and low-income regions should be done with specialized centers and endemic diseases should be considered in etiology. Schistosomiasis, Rheumatic heart disease, HIV infection, high altitude, chronic hepatitis, etc. should be considered. Treatment should be based on individual case assessment and PAH targeted medications may help especially when pursued at centers specialized in PH. Treatment of PH should be under expert guidance. Supportive therapy with oxygen, Diuretic therapy, anticoagulation should be based on a specific type of PH, CCB should be considered in those patients who are acutely reactive to iNO with or without oxygen. They should be avoided in AVT nonresponders and children with right heart failure regardless of AVT response.   Commentary from Dr. Venugopal Amula (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch:  To provide a specific, comprehensive, detailed and practical framework for the clinical care of children and young adults with Pulmonary Hypertension, the European Pediatric Pulmonary Vascular Disease Network constituted an executive writing group. The executive writing group members conducted a thorough scientific review and generated tables with graded recommendations based on the European Society of Cardiology/ American Heart Association definitions. They included studies with pediatric data only,  adult studies that included> 10% of children or studies that enrolled adults with CHD. Although the majority of recommendations on pediatric PH are based on the level of evidence B or C,  much effort has been made to obtain pediatric data from registries and clinical studies. A summary of such recommendations is provided below.  Updated Clinical Classification of Pulmonary Hypertension, WSPH, Nice, 2018 Diagnostic algorithm for a child or young adult with suspected pulmonary hypertension  Algorithm for management of patients with CHD associated with PAH/Pulmonary Hypertensive Vascular Disease and congenital shunt lesions.  Treatment algorithm for Pediatric PAH       

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Cardiomegaly on chest radiographs as a predictor of heart disease in the pediatric population.

Dasgupta S, Kelleman M, Slesnick T, Oster ME. Am J Emerg Med. 2019 Jun 25. pii: S0735-6757(19)30426-7. doi: 10.1016/j.ajem.2019.06.045. [Epub ahead of print] PMID: 31272753 Similar articles Select item 31275480 Take Home Points: Cardiomegaly on chest x-ray (CXR) has a relatively poor positive predictive value (PPV) for subsequent diagnosis of true heart disease. The addition of an EKG and BNP > 100 pg/ml improves the PPV, especially in patients < 1 year of age but does not negate the need for a subsequent echocardiogram.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Cardiomegaly on chest x-ray is not an infrequent reason for pediatric cardiology referral and consultation. False positive diagnoses may be common, with enlargement of the cardiac silhouette often due to other factors such as thoracic wall abnormalities, mediastinal factors (thymus), or technical issues. A small study in 2001 showed that CXR had pretty high specificity and negative predictive value (NPV), but poor sensitivity and PPV. The goal of this study was to determine the PPV of cardiomegaly on CXR in predicting true heart disease, and whether the addition of an EKG and BNP would have utility. They also stratified their results based on patient age. This was a retrospective chart review of all pediatric patients with cardiomegaly on initial CXR and who had a subsequent echocardiogram within 30 days. A study investigator confirmed the finding of cardiomegaly by recording the cardio-thoracic ratio (CTI) in a blinded fashion. A cut-off of CTI > 0.55 was used as the definition of cardiomegaly, with only those confirmed included in the study. If obtained, EKG findings and BNP levels were recorded, with a BNP > 100 pg/ml considered abnormal. PPV and NPV were calculated for all patients with cardiomegaly on CXR alone, + abnormal EKG, + BNP > 100, + both abnormal EKG and abnormal BNP, and those with normal EKG and normal BNP.  A subgroup analysis excluding those with an abnormal cardiac exam was also performed. 1544 patients out of 282,618 had cardiomegaly on CXR. 1055 were excluded since they did not have a subsequent echocardiogram or they had a known diagnosis of congenital disease, and 37 of those did not meet CXR criteria for cardiomegaly when reanalyzed. 489 patients met the inclusion criteria, with an EKG done in 275 patients, BNP in 176, and both in 122. 31% of patients were diagnosed with structural or functional heart disease or pericardial effusion. The median age of those with heart disease was significantly lower as compared to those without, with patients < 1 year of age most likely to have heart disease diagnosed by echo (see figure 1). See Table 2 for types of heart disease diagnosed. 59% of patients with heart disease had an abnormal CV exam. PPV of CXR alone was 31%, + abnormal EKG was 67.1%, + BNP > 100 was 59%, and if both was 76.7%. In patients < 1 year of age, the PPV for all categories was higher whether or not the physical exam was included (see figure 2). CXR alone as expected did not have a good PPV, and while the addition of an EKG and BNP improved the PPV, 19% of patients still had some type of heart disease and would thus not eliminate the need for an echocardiogram. This is of course clinically relevant, as an echo typically is not done easily in the ER setting, and cost-relevant, as the cost of an echocardiogram is quite expensive as compared to the other tests. Additionally, the authors report that 41% of patients with heart disease had a normal physical exam and did little to increase the PPV. This should probably be taken with some caution, as the expertise in physical exam has been known to have decreased in general, and the ER setting is sometimes not ideal to elicit subtle findings.  Overall, while adding these tests to the patient with cardiomegaly on CXR may help, many will still likely need to be referred for a full cardiology evaluation and echocardiogram.      

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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 Similar articles Select item 31165593 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.  

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Neurovascular findings in children and young adults with Loeys-Dietz syndromes: Informing recommendations for screening

LoPresti MA, Ghali MZ, Srinivasan VM, Morris SA, Kralik SF, Chiou K, Du RY, Lam S. J Neurol Sci. 2019 Dec 12;409:116633. doi: 10.1016/j.jns.2019.116633. [Epub ahead of print] PMID: 31862516 Similar articles Select item 31834244 Take Home Points: Cerebrovascular arterial tortuosity is a common finding in patients with Loeys-Dietz syndrome. Neurovascular findings mostly remain stable over years in pediatric patients.   Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  The authors retrospectively analysed clinical, genetic and imaging data in children and young adults with Loeys-Dietz syndrome. 47 patients were included of whom 39 had magnetic resonance angiography or computed tomography angiography of the head and neck. 21 patients had follow-up neurovascular imaging; average follow-up was 607 days (range 123–3070 days). Arterial tortuosity index of the left and right internal carotid artery (LICA, RICA) and vertebral arteries (LVA, RVA) were measured (Figure 1). Furthermore, the percent change in Arterial Tortuosity Index over time was calculated. All patients had genetic testing, most common mutations were those in TGFBR2 and TGFBR1. Neurovascular imaging data demonstrated intracranial neurovascular tortuosity in 79.5% and cervical neurovascular tortuosity in 64.1% of patients. Involvement of both, anterior and posterior circulation was common (Table 1). Three patients had intracranial aneurysms. None of the patients had intracranial hemorrhage or vascular rupture and no interventions were performed. Only three patients with follow-up imaging data were subjectively found to have new or progressive anomalies; all of them had a TGFBR2 mutation. Based on their findings, the authors nicely discuss the frequency of neurovascular screening in pediatric Loeys-Dietz syndrome patients.    

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Necrotizing Enterocolitis and Associated Mortality in Neonates With Congenital Heart Disease: A Multi-Institutional Study

Spinner JA, Morris SA, Nandi D, Costarino AT, Marino BS, Rossano JW, Shamszad P. Pediatr Crit Care Med. 2019 Sep 20. doi: 10.1097/PCC.0000000000002133. [Epub ahead of print] PMID: 31568264 Similar articles Select item 31546957 Take-Home Points: The prevalence of NEC in neonates with congenital heart disease is 3.7 %. Hypoplastic Left Heart Syndrome and TA were the two cardiac lesions associated with the highest frequency of NEC. The odds of developing NEC were 2.7 and 2.6 times when compared to the lesion with the lowest incidence of NEC, that is Transposition of great arteries. Adjusted risk factors associated with the development of NEC included prematurity, male sex, AA race, GI abnormality, and additional birth defects. The overall hospital mortality of neonates with CHD and NEC was significantly higher than compared to neonates with CHD  and no NEC. NEC was also associated with increased total hospital charges, longer ICU LOS and hospital LOS when compared to those without NEC and the association remained significant when analyzed by the individual cardiac defect. No association was found with a 30-day readmission rate.   Commentary from Dr. Venugopal Amula (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch:  This is a retrospective multi-institutional study describing the prevalence and mortality of necrotizing enterocolitis (NEC) in neonates with congenital heart disease. The Pediatric Health Information System database was queried for all index hospitalizations involving neonates with CHD between 2004 to 2015 and the primary outcome was the presence of NEC during the index hospitalization. Secondary outcomes studied included in-hospital mortality, hospital LOS, ICU LOS, hospital charges and 30-day readmission. Multivariable analysis for the presence of NEC and in-hospital mortality were performed for each heart defect category utilizing generalized estimating equations to adjust for hospital clustering. The heart defect categories included Single ventricle, Tricuspid atresia (TA), Truncus arteriosus, Transposition of great arteries, Tetralogy of Fallot and isolated arch obstruction. Variables included in the multivariable model for the presence of NEC were prematurity, African American race, male sex, additional birth defect or GI anomaly.                            

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Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome

Karaca-Altintas Y, Laux D, Gouton M, Bensemlali M, Roussin R, Hörer J, Raisky O, Bonnet D. Eur J Cardiothorac Surg. 2019 Jun 10. pii: ezz167. doi: 10.1093/ejcts/ezz167. [Epub ahead of print] PMID: 31180449 Similar articles Select item 31230936 Take Home Points: In absent pulmonary valve syndrome (APVS), a Nakata index (NI) of > 1500 mm2/m2 is a risk factor for death, even after pulmonary artery (PA) plasty. Whether repair at an earlier age (e.g. lower NI) would affect outcome remains to be seen, but this study does provide some prognostic and clinical information that could help providers counsel families and provide good post-operative care.             Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  APVS, a rare congenital abnormality often associated with an outlet VSD and overriding aorta (e.g. Tetralogy of Fallot with absent pulmonary valve), is typically characterized by massive dilation of the pulmonary arteries. Respiratory symptoms can develop due to tracheal and bronchial compression, with the clinical presentation often dependent on the severity of those symptoms. High morbidity and mortality is present in neonates with symptoms soon after birth, but others can have more mild symptoms, with surgery being delayed for a few months until elective repair is undertaken. The study aims were to report clinical and surgical outcomes of patients with APVS, investigate predictors of mortality and prolonged postoperative ventilation, to find a threshold PA size predictive of negative outcomes, and to evaluate if PA plasty or the LeCompte maneuver would influence airway relief. There were 3 groups: neonates < 28 days at repair, infants 28 days to 1 year old, children > 1 year old. Preoperative airway compression was evaluated with chest x-rays or CT scan and bronchoscopy when available, and graded as mild, moderate or severe. PA size was evaluated by echo and the Nakata index (sum of cross-sectional area of the left and right PAs in mm2 divided by BSA). Figure 1 shows the flowchart for surgical technique used. Prolonged mechanical ventilation (PMV) was defined as > 7 days. There were 68 total patients with a median age at repair of 3.9  months and weight of 5 kg. Airway compression was more frequent at a younger age and more severe in the neonatal group as would be expected. Median NI was 938 mm2/m2, and those that had surgery at a younger age, had pre- or postoperative airway compression, or required PMV had a significantly higher NI (see figure 2). The mortality rate at discharge was 12% and overall was 19%, with most early and late deaths due to respiratory failure. Table 2 lists the preoperative univariate and multivariable risk factors for mortality, with only the NI and basal SpO2 % being significant. Figure 3 shows the ROC curves of the ability of the NI to predict outcomes. The AUC was 0.92. A cut off of 1500  mm2/m2 had a sensitivity of 98% and a specificity of 82%. Figure 4 shows the Kaplan Meier survival curves according to NI.   The median duration of ventilation was 15 days and only lower weight at repair was a risk factor in multivariable analysis for PMV. The accuracy of the NI for PMV was not as good as for survival (AUC 0.76). Patients with a PA plasty +/- LeCompte had an increased risk of death and neither had an effect on duration of ventilation.  The NI for this subgroup remained the only risk factor for mortality, but the sensitivity/specificity of a cut-off of 1637 mm2/m2 for those with  a LeCompte was not as high (75% and 80% respectively). As the authors note, PA dilation can not only cause external compression of major airways, but can also be associated with intrinsic abnormal lung function due to distal bronchial compression. Increased small airway resistance has been shown in this population, even higher than those with RSV. This may be a reason why a PA plasty and LeCompte did not show significant benefit, as it only helped the more proximal airway compression. A limitation of this study was the use of echo to calculate the NI in 40% of the cohort, as most centers use CT or MRI for this. This may affect the NI cut-off for prognosis. Additionally, given the small overall sample size, it may be difficult to fully extrapolate the results.

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Right and Left Ventricular Strain Patterns After the Atrial Switch Operation for D-Transposition of the Great Arteries-A Magnetic Resonance Feature Tracking Study

Burkhardt BEU, Kellenberger CJ, Franzoso FD, Geiger J, Oxenius A, Valsangiacomo Buechel ER. Front Cardiovasc Med. 2019 Apr 9;6:39. doi: 10.3389/fcvm.2019.00039. eCollection 2019. PMID: 31024933 Free PMC Article Similar articles Select item 30947912 Take Home Points: In patients with transposition of the great arteries (TGA) after atrial switch operation the longitudinal strain of the systemic right ventricle is impaired compared to both a normal subpulmonary right ventricle and a systemic left ventricle. Free wall ventricular strain values for both ventricles were higher compared to global strain values in TGA patients.   Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  This is a Magnetic Resonance feature tracking study in adult patients with TGA after atrial switch operation. The authors included 29 adult patients with TGA after atrial switch operation and 19 healthy controls. Cardiac Magnetic Resonance (CMR) cine images were used for the feature tracking analysis and to measure ventricular volumes, ejection fraction (EF) as well as mitral and tricuspid annular plane systolic excursion. Cine phase contrast imaging was applied for internal validation of stroke volumes. The feature tracking analysis included the measurement of longitudinal and circumferential strain of both ventricles. Regional strain values for the left ventricle (LV) were measured using the American Heart Association 17-segment model; for the right ventricle (RV) a “mirror imaged” model for defining the RV segments was used. Cardiopulmonary exercise (CPEX) data were also collected.   The main findings include that the RV in the systemic position had larger volumes and lower EF than the systemic LV in healthy controls. Furthermore, the subpulmonary LV in patients was smaller and had a higher EF than the RV in healthy controls. In TGA patients, annular plane systolic excursion of the systemic and subpulmonary ventricles were lower compared to the corresponding systemic and subpulmonary ventricle in the control group. Global longitudinal strain of the systemic RV in patients was lower compared to the RV in controls (Figure 2). Compared to the systemic LV in healthy subjects the systemic RV in patients had both, lower global circumferential and longitudinal strains (Figure 3). The subpulmonary LV had a lower global circumferential strain compared to the systemic LV in controls (Figure 2). However, when compared to the subpulmonary RV in controls global circumferential strain of the subpulmonary LV was increased (Figure 3). Regional strain values in TGA patients after atrial switch were higher in the free wall compared to global strain values (Table 3). CPEX data did not correlate with ventricular strain values. Overall this study increases our knowledge about the global and regional function of the systemic RV in TGA patients after atrial switch operation and opens the way for further studies in this field.          

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Increased extracellular volume in the liver of pediatric Fontan patients

de Lange C, Reichert MJE, Pagano JJ, Seed M, Yoo SJ, Broberg CS, Lam CZ, Grosse-Wortmann L. J Cardiovasc Magn Reson. 2019 Jul 15;21(1):39. doi: 10.1186/s12968-019-0545-4. PMID: 31303178 Free PMC Article Similar articles Select item 31302712 Take Home Points: In Fontan survivors, elevated systemic venous congestion, reduced cardiac output and a multitude of other hepatic insults including often multiple prior cardiac surgeries results in Fontan associated liver disease (FALD) characterized by hepatic congestion, and fibrosis. In Fontan patients, varying degrees of FALD is universal and progressive. Cirrhosis and hepatocellular carcinoma are known morbidities of advanced FALD. The natural history of FALD and factors influencing its progress are unclear. Traditional serological markers of hepatic function fail to provide adequate insight into severity and progression of FALD until late in the disease process. Cardiac magnetic resonance imaging T1 mapping of liver and young Fontan patient is feasible. Fontan survivors showed elevated native T1 and ECV values compared to both bidirectional cavopulmonary anastomosis subjects and controls suggesting diffuse hepatic congestion and/or fibrosis. Subjects with Fontan as well as bidirectional cavopulmonary anastomosis showed elevated native T1 compared to control indicating hepatic congestion even in subjects with bidirectional cavopulmonary anastomosis. In Fontan subjects, T1 values correlated with exposure to cardiopulmonary bypass time but not to central venous pressure or age. Native T1 and ECV values may be useful imaging biomarkers to evaluate hepatic congestion and fibrosis in FALD. ECV does not provide clinically significant additional information compared to native T1 analysis. Relying on native T1 analysis obviates the need for gadolinium and reduces the postprocessing and scan time. Commentary from Dr. Shaji Menon (Salt Lake City), section editor of Pediatric Cardiology Journal Watch: This is a retrospective cross-sectional cohort study of pediatric patients with bidirectional cavopulmonary anastomosis and Fontan procedures. Hepatic native T1 times and ECV was measured using cardiac short axis modified Look-Locker inversion recovery sequence retrospectively from images displaying the liver.  A multivariate regression model was performed for evaluating factors associated with native T1 times and ECV.  Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan. Figure 1 Figure 2        

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2-Year Outcomes After Complete or Staged Procedure for Tetralogy of Fallot in Neonates

Savla JJ, Faerber JA, Huang YV, Zaoutis T, Goldmuntz E, Kawut SM, Mercer-Rosa L. J Am Coll Cardiol. 2019 Sep 24;74(12):1570-1579. doi: 10.1016/j.jacc.2019.05.057. PMID: 31537267   Take-Home Points: • Complete repair has a higher mortality than a staged approach in neonates with tetralogy. • Postoperative cardiac complications are the most likely mediator for this relationship. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Symptomatic/cyanotic infants with TOF require neonatal intervention. There has been debate as to the better strategy: palliation with a shunt or complete repair. Both have advantages and disadvantages. Shunt palliation will prolong cyanosis and require a 2nd surgery. However, complete repair is a more complex procedure with a longer hospital course and less patients would be able to have a valve-sparing repair, which likely has much better long term consequences. It is known that neonatal repair has a higher morbidity and mortality than later complete repair, but it has not yet been clear which approach may result in the best outcomes in the neonatal population. This was a retrospective cohort study using the Pediatric Health Information System (PHIS) database between 2004-2015. Primary outcome was death during the 2 year follow up after initial procedure. Somewhat complex statistics were necessary to control for patient and hospital based characteristics, as both could influence the choice of surgical approach. An inverse probability weighted Cox model was used to estimate the effect of the initial procedure on late mortality and a logistic regression was used to analyze mortality within the first hospitalization. To address unmeasured confounding variables and their relative strength, E values were calculated for the estimated hazard and odds ratios. 2363 patients were studied; 1032 had a complete repair and 1331 were staged. Table 1 showed good balance in the weighted sample, with no demographic or patient characteristics being significantly different (all < 10%). Surgeries were performed at 45 hospitals; 24% performed greater than ⅔ complete repairs, 33% performed greater than ⅔ staged repairs, and 42% had equal proportions. Median length of initial hospital stay was 16 days for the complete repair and 15 days for the staged repair. There were 239 deaths, 92 in the complete and 147 in the staged groups. Figure 1 shows the breakdown of each cohort. Complete repair had a greater risk for mortality within the 2 year follow up (HR 1.51) and is shown graphically below. There was no difference within the first week, but from day 7-30 (early), the complete group had an increased risk of mortality (HR 2.29). E-value calculations indicated that only the presence of very strong unmeasured confounders could explain the association of type of repair and survival. See Table 2. At least one in-hospital cardiac complication was observed in 36.1% of the complete group and 15.4% of the staged group. This was shown to be the primary mediator between surgical approach and mortality, with 87% of the total effect attributable to early mortality and 80% to late mortality. Non-cardiac complications did not mediate the treatment-mortality relationship. However, subgroup analysis did not show a difference in mortality for patients who received a BT shunt versus complete repairs, nor were there differences in those that received an RV to PA conduit within the complete repair group. The authors write that the trend was similar for benefit of BT shunt over complete repair, but the study was possibly underpowered to detect a significant relationship. As an accompanying editorial suggests, given the limitation of an administrative dataset, the most likely influence of this study will be on those considered to be higher risk. If there is a way to identify those that are more likely to have a cardiac complication, or a way to prevent that complication from occurring, then complete repair risk may be mitigated. Additionally, of those patients in the staged group that did not receive a BT shunt, was it improved catheter based therapy such as RVOT stenting or PDA stenting that led to the improved mortality? Overall, it does seem that a less-invasive initial approach should be favored, especially in the highest risk patients, and decision-making should be individualized to achieve the best possible results. A risk-stratification model may be the next logical step to help determine who may benefit the most from a given therapy.

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Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium)

Oster ME, McCracken C, Kiener A, Aylward B, Cory M, Hunting J, Kochilas LK. Am J Cardiol. 2019 Jun 6. pii: S0002-9149(19)30626-5. doi: 10.1016/j.amjcard.2019.05.047. [Epub ahead of print] PMID: 31272703 Similar articles Select item 31130285 Take Home Points: The long-term survival in patients with aortic coarctation who underwent surgery before the age of 12 months is excellent. Weight <2.5 kg at the time of CoA repair, presence of a genetic syndrome and surgery before 1990 seems to be associated with increased late mortality.   Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  It has been shown that patients with aortic coarctation (CoA) have an increased cardiovascular morbidity and mortality despite successful surgical repair. This retrospective study evaluated a large cohort of CoA patients who underwent surgical repair before 12 months of age between 1982 and 2003 using data from the Pediatric Cardiac Care Consortium (US-based registry for interventions for pediatric heart diseases) as well as the National Death Index and the Organ Sharing Procurement Network. Long-term survival and risk factors associated with late mortality were assessed. Patients who had lesions other than atrial and ventricular septal defect, bicuspid aortic valve, aortic stenosis and hypoplastic aortic arch were excluded. The CoA type was categorized as follows: 1) CoA with genetic syndrome, 2) CoA with VSD, 3) CoA with left-sided obstruction including hypoplastic aortic arch, bicuspid aortic valve, aortic stenosis and 4) simple CoA with no accompanying defects other than a patent ductus arteriosus or atrial septal defect. 2424 CoA patients from 43 centers met the inclusion criteria. Of them 57 died postoperatively during the admission for CoA repair. 2367 patients were discharged alive and of them 1913 patients had adequate identifiers to be submitted to National death Index and Organ Procurement and Transplant Network. The 1-year and 20-year survival of these patients was 97.5% and 95.6%, respectively. Those patients who were discharged alive were likely to be older at the time of surgery, to have a greater weight at the time of surgical repair, to have undergone surgical repair outside the neonatal period and to have a simple CoA. Those patients who died during the postoperative course after CoA repair were likely to have a genetic syndrome and CoA with VSD. The overall long-term survival of all patients included in this study was 94.5% (Figure 2A) and 95.8% of those who were discharged alive (Figure 2B). CoA repair as a neonate was associated with worse survival (Figure 3A) and patients with a genetic syndrome had worse survival compared to simple CoA (Figure 3B). Furthermore, patients who underwent patch angioplasty and subclavian flap repair showed decreased survival compared to patients who had surgical repair with end-to-end anastomosis (Figure 3C). Weight <2.5 kg at the time of repair, presence of a genetic syndrome and surgery in the 1980’s was associated with increased late mortality. More than half of the deaths were related to the cardiovascular system. Overall, this interesting study increases our knowledge about the long-term survival in CoA patients who underwent surgical repair in the neonatal period and infancy. Figure 2 Figure 3          

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Pharmacologic stress cardiovascular magnetic resonance in the pediatric population: A review of the literature, proposed protocol, and two examples in patients with Kawasaki disease.

Fares M, Critser PJ, Arruda MJ, Wilhelm CM, Rattan MS, Lang SM, Alsaied T. Congenit Heart Dis. 2019 Sep 9. doi: 10.1111/chd.12840. [Epub ahead of print] Review. PMID: 31498562   Take-Home Points: Pharmacologic stress cardiovascular MRI (PSCMR) is an underused but potentially very helpful modality in assessing certain types of pediatric CHD, especially those with coronary abnormalities or coronary reimplantation. Further focused research as well as cost-analysis may be useful, but integrating this with a larger stress perfusion testing center may be most effective. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  A large trial recently published in JACC on MRI stress perfusion in adults with coronary artery disease received a lot of attention due to the high sensitivity and cost-effectiveness of what is a clearly underused and under-available modality. This was a review article on its use in the pediatric/adult congenital population along with proposed protocols.  Exercise stress testing is of course not feasible for all patients, but the sensitivity and specificity may be low, especially when trying to assess perfusion of the right ventricle. This is also a known limitation of nuclear testing. Often times, pediatric cardiologists need to make decisions based on echo findings, standard stress testing, or sometimes resort to an invasive cardiac catheterization, but which still do not always provide the prognostic information necessary to decide whether surgery or other management needs to occur.   Table 1 lists high risk cohorts who could theoretically benefit from PSCMR which can be categorized as surgical coronary reimplantation, anomalous coronary artery (AAOCA), acquired disease (such as Kawasaki disease (KD)), or systemic right ventricle. Inotropic agents (dobutamine) and coronary vasodilator agents can be used to mimic physical exercise. Dobutamine may be better utilized when there is concern for dynamic obstruction such as in AAOCA or myocardial bridging. A newer vasodilating agent, Regandenoson, has a better side effect profile and only requires a single bolus instead of a continuous infusion as needed in older agents (adenosine/dipyridamole). The authors list the few, relatively small, studies done using PSCMR, and highlight the fact that there is data to suggest higher sensitivity and specificity, along with the advantages of no radiation exposure and not being limited by acoustic windows (as stress echo may be). They also provide a suggested protocol along with dosage when using dobutamine, adenosine, and regadenoson and provide a conceptual framework for imaging (Figure 1). Finally, they discuss 2 case reports of patients with KD and the clear utility of PSCMR. The first case was a patient with a history of giant aneurysms that had mostly resolved and with a normal treadmill stress test. PSCMR showed perfusion defects, and the patient ultimately underwent cutting balloon dilation. The 2nd case was a young patient (4 years old), too young to undergo standard stress testing, with giant aneurysms and perfusion defects. A cath showed near occlusion of the LAD and he underwent CABG.   Hopefully, there will be further published studies looking at PSCMR in pediatric patients, as this is a modality that can be very helpful in certain subgroups for which current testing is limited.          

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Outcomes After Extracorporeal Cardiopulmonary Resuscitation of Pediatric In-Hospital Cardiac Arrest: A Report From the Get With the Guidelines-Resuscitation and the Extracorporeal Life Support Organization Registries

Outcomes After Extracorporeal Cardiopulmonary Resuscitation of Pediatric In-Hospital Cardiac Arrest: A Report From the Get With the Guidelines-Resuscitation and the Extracorporeal Life Support Organization Registries. Bembea MM, Ng DK, Rizkalla N, Rycus P, Lasa JJ,...

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Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting

Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting. Qureshi AM, Goldstein BH, Glatz AC, Agrawal H, Aggarwal V, Ligon RA, McCracken C, McDonnell...

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