2020

Procedural, pregnancy, and short-term outcomes after fetal aortic valvuloplasty

Procedural, pregnancy, and short-term outcomes after fetal aortic valvuloplasty Patel ND, Nageotte S, Ing FF, Armstrong AK, Chmait R, Detterich JA, Galindo A, Gardiner H, Grinenco S, Herberg U, Jaeggi E, Morris SA, Oepkes D, Simpson JM, Moon-Grady A, Pruetz JD. Catheter Cardiovasc Interv. 2020 Mar 26. doi: 10.1002/ccd.28846. [Epub ahead of print] PMID: 32216096 Similar articles Select item 32215649   Take Home Points: Fetal aortic valvuloplasty offers promise in patients with congenital aortic stenosis to prevent progression to hypoplastic left heart syndrome It is a high risk intervention and center experience is crucial to maximizing success Further investigation of outcomes in patients who undergo fetal aortic valvuloplasty but still require single ventricle palliation is warranted     Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch: Fetal aortic valvuloplasty (FAV) was first reported in 1991 as an option to treat congenital aortic stenosis in mid-gestation fetuses with the goal to prevent progression to hypoplastic left heart syndrome (HLHS). The process involves using ultrasound guidance to puncture through the maternal abdomen and uterus, then into the fetal thigh to deliver analgesia and a muscle relaxant. A different needle is then passed through the fetal chest and into the left ventricle. A wire is advanced through the needle across the aortic valve. A balloon is advanced over the wire and inflated across the aortic valve. Most centers performing fetal aortic valvuloplasty perform a small number of cases. Boston Children’s Hospital has performed the largest number of these cases and have previously reported their procedural outcomes (Freud LR, McElhinney DB, Marshall AC, et al. Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients. Circulation. 2014;130(8):638-645 and Friedman KG, Sleeper LA, Freud LR, et al. Improved technical success, postnatal outcome, and refined predictors of outcome for fetal aortic valvuloplasty. Ultrasound Obstet Gynecol. 2018;52(2):212-220), as have several other smaller single center studies. Procedure related fetal mortality rates are variable across centers (6.5-32.1%). The International Fetal Cardiac Interventions Registry (IFCIR) was created in 2010 to collect data on fetal interventions from multiple centers (19 centers actively entering data) and improve outcomes. The goal of this study was to look at several technical and procedural aspects of FAV (cannula size, balloon size, number of cardiac punctures) to determine if they were related to procedural and pregnancy outcomes.   Patel et al. report on their analysis of patients from 15 centers in the IFCIR who were candidates for fetal cardiac intervention from 2002-2018. The median center volume was 5 (range 1-21). Patients from Boston Children’s Hospital were excluded as data on those patients had already been presented in the previously mentioned studies. A total of 128 fetuses with a mean gestational age (GA) of 26.1 ± 3.4 weeks were deemed candidates. 108 of these fetuses underwent cardiac puncture and had adequate data in the database to be included for analysis. The indication for FAV was evolving HLHS in 103 fetuses (95.4%). The needle used to puncture the LV was 17 gauge in 15.7%, 18 gauge in 63.9%, and 19 gauge in 29.6%. A single puncture was needed in 77.1%, two punctures in 19.4%, and three punctures in 5.6%. 100 fetuses had an aortic balloon valvuloplasty performed, 90 (83.3%) of which were technically successful (defined as increased forward flow across aortic valve or new aortic regurgitation). The mean aortic valve Z-score was -2.5 ± 1.1 for all fetuses and the median balloon: aortic valve ratio was 1.1. 52 fetuses (48.1%) had at least one intraprocedural complication including bradycardia requiring treatment (37, 34.3%), pericardial effusion requiring treatment (24, 22.2%), pleural effusion (3, 2.8%), balloon rupture (6, 5.6%), and intraprocedural death (9, 8.3%). The overall procedural related mortality was 16.7% (9 intraprocedure deaths, 9 additional deaths within 48 hours). More than one puncture was associated with higher rates of procedural complications (specifically bradycardia, pleural effusion, and intraprocedural death). On multivariate analysis, later GA at intervention and technical success of the procedure were independently associated with live birth.   The outcomes of the pregnancies and infants born is shown below. The 81 fetuses born alive had a median GA of 38.1 weeks, with 26 of them being born prematurely (<37 weeks GA).     This study shows that fetal intervention is often technically successful and offers promise for a biventricular circulation. However, it has significant associated risk with a complication rate of nearly 50% and procedure related mortality of 16.7% (Boston Children’s Hospital’s most recent report had a mortality rate of 6.5%). The higher mortality in this series is multifactorial and likely involves the known learning curve that has been seen in performing this procedure. The authors highlight the importance of center experience and appropriate fetal positioning to minimize the number of punctures. Also, the authors discuss that later GA at the time of the procedure decreased risk likely due to larger fetus size. However, fetuses that were candidates later in gestation may also have less severe disease and waiting for many fetuses could lead to missing the window for prevention of HLHS. Interestingly, of the 81 patients born alive, 22 (27.1%) died prior to hospital discharge. Data from the Single Ventricle Reconstruction trial showed hospital mortality or need for transplantation at 18% (Pasquali SK, Ohye RG, Lu M, Kaltman J, Caldarone CA, Pizarro C, Dunbar-Masterson C, Gaynor JW, Jacobs JP, Kaza AK, Newburger J, Rhodes JF, Scheurer M, Silver E, Sleeper LA, Tabbutt S, Tweddell J, Uzark K, Wells W, Mahle WT, Pearson GD; Pediatric Heart Network Investigators. Variation in perioperative care across centers for infants undergoing the Norwood procedure. J Thorac Cardiovasc Surg. 2012; 144:915–921. doi: 10.1016/j.jtcvs.2012.05.021). While it is unknown if this difference is statistically significant and how many of those patients who died underwent attempted single ventricle palliation, the outcomes of patients that have had a fetal intervention and still require single ventricle palliation versus those that have not warrants further future investigation.    

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Uncertainty of Prenatally Diagnosed Congenital Heart Disease: A Qualitative Study

Uncertainty of Prenatally Diagnosed Congenital Heart Disease: A Qualitative Study Kelly W Harris 1 2, Kathleen M Brelsford 2, Ann Kavanaugh-McHugh 3, Ellen Wright Clayton 1 2 4 JAMA Network Open. 2020 May 1;3(5):e204082.   doi:0.1001/jamanetworkopen.2020.4082. PMID: 32369178 PMCID: PMC7201310 Free PMC article   Take-Home Points:   The most significant contributor of stress to parents with the prenatal diagnosis of severe congenital heart disease is uncertainty – especially as it relates to the long term unknown variables of the cardiac diagnosis. The strategies adopted by referring physicians and fetal cardiologists before, during, and after appointments reduced or compounded stress surrounding uncertainty. Potential interventions to reduce parental anxiety may include expectation-setting before referral clinic appointment, communication in clinic both verbal and nonverbal and identity formation after the new diagnosis. Parallels can be drawn and counseling practices from other specialties – such as pediatric palliative care adopted in managing parental stress.     Commentary from Dr. Venugopal Amula (Salt Lake City UT), section editor of Pediatric Cardiology Journal Watch: Parents with a prenatal diagnosis of severe congenital heart disease experience stress and anxiety. Mitigation strategies should take into account the longitudinal emotional experience of the parents in the prenatal period. Prenatal diagnosis is known to improve neonatal outcomes with congenital heart disease by informing postdelivery care and decreasing hypoxia, metabolic acidosis, and end-organ damage. Little is known about the impact of prenatal diagnosis on the psychological health of parents. Harris et al. perform a qualitative analysis of parents' prenatal experience in their account using a thematic analysis approach of professionally transcribed audio-recorded telephone interviews. The coding of the transcripts was performed and revised iteratively by one author while evaluating intercoder reliability using a second author. The structured interview was performed on parents referred to and seen at the Fetal Cardiology Clinic at Vanderbilt Children’s Hospital from May 2019 to August 2019. Twenty-seven individuals from 17 families participated in 42 phone interviews during pregnancy, 27 after the first appointment, and 15 after follow up pediatric cardiology appointments. The authors identified “Uncertainty” as a pervasive central theme and were related both to concrete questions on scheduling, logistics, or next steps, and long-term unknown variables concerning the definitiveness of the diagnosis or overall prognosis. A portion of the uncertainty is solvable, especially concerning the process of care, but the portion that relates to details of anatomic diagnosis, the success of future interventions, etc. are unavoidable.   Although the report is limited by small sample size and may not represent a broad parental group with differing demographic, clinical, and cultural characteristics, it provides preliminary insight into the contributors to stress and potential interventions needed.    

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Implementation of a statewide, multisite fetal tele-echocardiography program: evaluation of more than 1100 fetuses over 9 years

Implementation of a statewide, multisite fetal tele-echocardiography program: evaluation of more than 1100 fetuses over 9 years. Bolin EH, Collins RT 2nd, Best TH, Zakaria D, Lang SM, Boushka MG, Renno MS, Heil LL, Lowery CL, Bornemeier RA.J Perinatol. 2020 May 7. doi: 10.1038/s41372-020-0677-2. Online ahead of print. PMID: 32382116   Take Home Points:   Fetal tele-echo identified all types of CHD with a sensitivity of 74% and specificity of 97%. For ductal dependent lesions, sensitivity and specificity were 100%. With an experienced sonographer willing to travel, fetal tele-echo may be a viable option for more remote locales.     Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: This was a study of fetal tele-echo (FTE) on 1164 fetuses in 6 satellite sites in Arkansas. The main campus is located centrally in the state, and they opened 6 offices throughout the state, many over 100-150 miles away. All studies were interpreted in real time by a fetal cardiologist as they were transmitted over a broadband IP network. Most of the studies were performed by a single sonographer with 20+ years experience. A positive study, defined as whether a postnatal echo was recommended (excluding a normal FTE in a poorly controlled diabetic mother) prompted a next available referral to the main campus with (in-person) face to face consultation with a pediatric cardiologist. FTE was not recommended in those with a high risk of carrying a fetus with an arrhythmia or structural heart disease (e.g. suspected cardiac abnormality or arrhythmia on OB ultrasound or hydrops) and these patients were initially referred to the main campus.   Table 1 shows the population characteristics. 1086 FTEs were normal and 78 abnormal. There were 15 false negative studies, but only 2 of those required cardiac intervention in the first 6 months of life. Both were perimembranous VSDs. There were 36 false positives of which the authors state were mostly small VSDs that may have closed prior to delivery. Of the 42 true positives, 18 required intervention in the first 6 months of life. This resulted in 74% sensitivity and 97% specificity. When subanalyzing ductal dependent lesions, FTE was 100% sensitive and 100% specific. With neonatal intervention within the first 6 months, FTE was 91% sensitive and 100% specific. See table 2. There was 1 patient who had a normal FTE, but subsequently developed flail TV and functional PV atresia; however since the disease developed after FTE, the case was not included in the analysis.   Per the authors, this was the largest study of FTE in terms of number of patients and sites. Previous studies have also shown good accuracy of FTE as well as community acceptance and patient preference of local FTE instead of traveling to quite distant sites. Additionally, there may be some economic benefits to patients and empowerment of local providers. The authors did not specifically discuss delivery planning based on FTE which would have been helpful given the variations in regional and state-wide hospital systems and medical transport. They showed an increase in rate of prenatal detection of CHD as well as financial benefit with implementation of the FTE program.   There were a few caveats. First 26% of abnormal FTEs were lost to follow up, which is quite high and concerning. The authors state that none of these cases were ductal dependent lesions. Additionally, the feasibility of the program most likely hinges on the experience of the sonographer; however, the studies were reviewed in real-time and theoretically, discussion and requests for more/different views could be done before the patient left. Based on the data, I would wonder if real-time review is actually necessary; this would result in even greater cost-effectiveness and financial benefit and likely be more applicable nation-wide or even world-wide.      

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Umbilical Cord Blood Gas in Newborns with Prenatal Diagnosis of Congenital Heart Disease: Insight into In-Utero and Delivery Hemodynamics

Umbilical Cord Blood Gas in Newborns with Prenatal Diagnosis of Congenital Heart Disease: Insight into In-Utero and Delivery Hemodynamics. Adams AD, Aggarwal N, Iqbal SN, Tague L, Skurow-Todd K, McCarter R, Donofrio MT. Pediatr Cardiol. 2019 Dec;40(8):1575-1583. doi: 10.1007/s00246-019-02189-x. Epub 2019 Aug 30. PMID: 31471626 Similar articles   Take Home Points: Newborns with a prenatal diagnosis of congenital heart disease are not at increased risk of acidosis at the time of delivery when compared to gestational age-matched controls. No difference was noticed in the umbilical arterial (UA) pH between those with single ventricle vs two-ventricle disease and those with and without aortic arch obstruction. In pregnancies complicated by congenital heart disease, spontaneous vaginal delivery with prolonged labor seems to impact the fetal outcome as noted by the significant decline in UA pH with increasing duration of labor in this group when compared to other modes such as induced vaginal delivery and C-Section post-labor. Commentary from Dr. Venu Amula (Salt Lake City), section editor of Fetal Cardiology Journal Watch: The American College of Obstetricians and Gynecologists and the American Academy of Pediatrics recommend performing umbilical artery blood acid-base analysis after certain high-risk deliveries in which a fetal metabolic abnormality is suspected to assess fetal well-being. Adams et al conducted this single-center, retrospective observational study to determine if newborns with congenital heart disease are at a higher risk for acidosis at delivery as determined by the umbilical cord blood analysis. The control group included singleton deliveries without CHD matched by date of birth and gestational age. The authors also sought to determine if specific fetal cardiac diagnosis, type, and duration of labor are associated with acidosis. The cases included all forms of complex congenital heart disease. Given the unique physiological challenges faced by single ventricle heart lesions and those with systemic outflow obstruction – the lesions were divided into 4 categories – Single Ventricle lesions with and without aortic arch obstruction, and Biventricular lesions with and without aortic arch obstruction. Class, I was defined as patients with two ventricles without aortic arch obstruction Class II as patients with two ventricles with arch obstruction, Class III as patients with a single ventricle without arch obstruction and Class IV as patients with a single ventricle with arch obstruction. The study cohort consisted of 134 cases with an equal number of gestational and calendar year matched healthy newborns. Overall there was no difference in the median UA pH in the cases with congenital heart disease versus the control group. There was also no difference by physiological class nor by single ventricle vs two ventricle type nor by the presence or absence of aortic arch obstruction. The authors conclude that fetuses with congenital heart disease have well-compensated hemodynamics and inutero oxygen delivery owing to fetoplacental circulation regardless of the subtype of congenital heart disease. They also evaluated the effect of mode of delivery on UA pH and found that in the congenital heart disease group there was a significant decline in the median UA pH with increasing duration of labor in those with spontaneous vaginal delivery when compared to those with induced vaginal and C-Section Post-Labor. However, it is to be noted that even in this group pathological fetal acidemia, a practical pH threshold where neonatal morbidity increases i.e. umbilical artery pH <7, was rare. The study is limited by a sampling bias given no umbilical arterial blood analysis data was present for those with hemodynamically unstable neonates with congenital heart lesions. Maternal characteristics were also not completely matched given the placental health may impact umbilical cord gas analysis even though venous sampling would be more reflective of that. Duration of labor in fetuses with the diagnosis of congenital heart disease planned for spontaneous vaginal delivery may impact the outcome as evidenced by declining UA pH and needs planned perinatal management.      

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Isolated ventricular septal defects demonstrated by fetal echocardiography: prenatal course and postnatal outcome

Isolated ventricular septal defects demonstrated by fetal echocardiography: prenatal course and postnatal outcome. Raucher Sternfeld A, Sheffy A, Tamir A, Mizrachi Y, Assa S, Shohat M, Berger R, Lev D, Gindes L. J Matern Fetal Neonatal Med. 2020 Jan 12:1-5. doi: 10.1080/14767058.2020.1712710. [Epub ahead of print] PMID: 31928261 Similar articles Select item 31940858   Take Home Points: Small muscular VSDs are common in fetal life with almost 50% closing before birth and over 90% closing by 2 years of life Muscular VSDs were not associated with any chromosomal abnormalities or genetic mutations on microarray analysis, but only a small number of patients underwent testing The high incidence and frequency of spontaneous closure along with no genetic abnormality may suggest that the presence of small muscular VSDs may be a delayed physiological process as opposed to something pathologic, but this premise should be studied further Perimembranous VSDs are less likely to close and more likely to be associated with a genetic abnormality Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  VSDs are the most common congenital cardiac malformation and often diagnosed prenatally. Most VSDs will have little hemodynamic effect prenatally due to equal pressure in both ventricles and limited shunting and many close during fetal life or within a few years after birth. This was a retrospective study looking at prenatal course and postnatal outcomes in fetuses diagnosed with VSDs over a 4 year study period of 7466 fetal echocardiograms. 59 cases of complicated CHD was seen (0.79%). 86 isolated VSDs were diagnosed (1.17%) with 11 cases excluded due to loss of follow up. 64/75 patients had muscular VSDs with a mean size of 1.1 mm and median maximal size of 1 mm. In comparison, perimembranous VSDs had a mean size of 2.5 mm and a median size of 3.5 mm. The authors note that all fetuses with a VSD are referred for genetic consultation at this study center and each family can decide on testing. 19 fetuses subsequently had a karyotype and 4 fetuses had a complete microarray. All fetuses had normal testing except for 1 with Down Syndrome who had a perimembranous VSD. Spontaneous closure occurred in 92% of the muscular VSDs during the study period (up to 2 years) and 48.4% closed before birth. This contrasted with perimembranous VSDs, with 45.5% closing over 2 years and 27% before birth (see Table 1). Amongst all VSDs, those that closed spontaneously were significantly smaller than those that remained open (1.48 +/- 0.68 mm vs. 2.6 +/- 1.25 mm). The authors conclude that muscular VSDs are common, are not associated with chromosomal aberrations, and often close spontaneously. They suggest that the presence of these VSDs may actually represent a delayed physiologic process rather than something pathologic. A significant caveat to this would be that very few patients in their study actually underwent genetic testing (specifically microarray) and it is possible that there may be some genetic mutations that would be associated with VSDs. However, invasive genetic testing such as amniocentesis should likely not be recommended for this patient population. Additionally, the overall number of patients was small and while they mention size as a significant factor for spontaneous closure, nearly all of those patients had muscular VSDs anyway. I do think that this study adds to the objective ability of fetal cardiologists to reassure patients when small muscular VSDs are seen even though most of us have recognized this by experience.  

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Coronary Intimal Thickening Begins in Fetuses and Progresses in Pediatric Population and Adolescents to Atherosclerosis

Coronary Intimal Thickening Begins in Fetuses and Progresses in Pediatric Population and Adolescents to Atherosclerosis. Guerri-Guttenberg R, Castilla R, Cao G, Azzato F, Ambrosio G, Milei J. Angiology. 2020 Jan;71(1):62-69. doi: 10.1177/0003319719849784. Epub 2019 May 14. PMID: 31088126 Similar articles Select item 31475425   Take Home Points: Coronary intimal thickening (IT) can be found in infants, children and adolescents. The prevalence of IT is higher with older age. IT is rarely seen in fetuses, suggesting that it is probably not related to embryological or fetal development. Comment from Dr. Inga Voges (Kiel, Germany), section editor of Fetal Cardiology Journal Watch: In this autopsy study the authors assessed the prevalence of coronary intimal thickening (IT) and types of coronary alterations in 63 hearts from fetuses, infants, older children (1-11 y) and adolescents (11-18 y) without structural heart disease. None of the deaths were related to coronary artery alterations. Histomorphometric and planimetric analyses were performed and maximal intima thickness, media thickness, as well as intima to media ratio were measured. IT was defined as musculoelastic thickening characterized by proliferation of smooth muscle cells, scarce monocytes and lymphocytes embedded by amorphous deposits within the internal elastic membrane and morphologically intact endothelium above the lesion. In addition, immunophenotyping of cells in the vessel wall was performed with monoclonal antibodies against macrophages, smooth muscle cells, transforming growth factor b1 (TGF-b1), apolipoprotein B and endothelial cells. An automated immunohistochemical system was used for stem cells(CD34/CD117), fibroblasts (vimentin), endothelial cells (CD34/vimentin), endothelial cells and monocytes/macrophages (vascular endothelial growth factor), and cellular proliferation (Ki67). In the group of fetuses (n=20) only two cases were found to have IT. In the group of infants (n=18), older children (n=15) and adolescents (n=10) the authors found an increasing frequency of coronary alterations with increasing age (see Table I and figure I). The extent of IT correlated with patient age (see figure 3) but was not related to gender or causes of death. Intimal thickening was more commonly found near bifurcation sites in the left anterior descending coronary artery and in zones free of bifurcation in the right coronary artery. The authors also found that the thicker the intima, the more disrupted the internal elastic membrane. Immunohistochemical studies with a-SMC actin could demonstrate, that in IT, smooth muscle cells lose polarity and orient in a perpendicular manner towards the internal elastic membrane or in a disarranged form. The authors nicely summarize that their results suggest that 1) IT is not a “remnant” of changes derived from embryological development, 2) IT cannot be a consequence of alterations in fetal circulation/oxygenation and 3) the effects of maternal risk factors or other maternal conditions during pregnancy are not evident during fetal life.

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Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study. Freud LR, McElhinney DB, Kalish BT, Escobar-Diaz MC, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Selamet Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, Tworetzky W.J Am Heart Assoc. 2020 Nov 3;9(21):e016684. doi: 10.1161/JAHA.120.016684. Epub 2020 Oct 20.PMID: 33076749 Free PMC article.   Take Home Points Patients with fetal diagnosis of Ebstein anomaly were managed heterogeneously post-natally. Those patients that underwent a neonatal intervention, mortality differed by procedure with right ventricular exclusion procedures having no mortality. Low tricuspid valve regurgitation jet and lack of antegrade flow across the pulmonary valve was associated with a higher mortality. Commentary from Dr. Clifford Cua (Columbus Ohio USA), section editor of Pediatric/Fetal Cardiology Journal Watch: CUA This was a retrospective multicenter follow up study on fetuses that had a fetal diagnosis of Ebstein anomaly (EA) or tricuspid valve dysplasia (TVD) who survived to birth. The goal of this study was to describe the management strategies post-natally for these patients as well as to define any clinical or echocardiographic parameters associated with mortality and, among survivors, a palliated versus biventricular circulation outcome.   Retrospective multicenter (23 institutions) database query from 2005 to 2011 was performed. Singleton fetuses without other associated anomalies, no fetal interventions, and who survived to birth were reviewed. Clinical and echocardiographic data were reviewed at various time points. 176 live births occurred and 22 patients were excluded, therefore the cohort evaluated consisted of 154 patients. Earlier gestational age at birth, lower birth weight, lower APGAR scores, increased medical management, and > 1 neonatal surgeries were associated with increased mortality (Table 1 and Figure 1). 83/154 patients did not undergo any intervention. 66/83 of these patients survived to discharge (Figure 2). Various interventions were performed on the 71 patients that underwent a procedure (Table 2). All patients who underwent a right ventricular exclusion survived. Mortality was highest for RV-PA conduit placement (50%), shunt or ductal stent placement (48%), and TV repair (42%). Multiple echocardiographic parameters were significantly different between survivors and non-survivors with univariate analysis, but only lower TR velocity (OR 2.3 [1.1 – 5.0]) and lack of antegrade flow across the pulmonary valve (OR 4.5 [1.3 – 14.2]) was associated with neonatal mortality. When including clinical data in the model, lower birth weight (OR 4.5 [1.8 – 11.1]) and lower TR jet (OR 4.2 [1.9 – 9.1]) were significantly associated with mortality. All patients who underwent a palliation with right ventricular exclusion and a TR velocity < 3.0 m/s and no antegrade pulmonary blood flow. Those that underwent a TV repair as part of a biventricular pathway, patients with a TR velocity < 2.5 m/s had 50% mortality whereas patients with a TR velocity > 3/0 m/s all survived. In this large multisite study of patients with a fetal diagnosis of TV abnormalities who survived > 24 hours after birth, birth weight, TV velocity, and pulmonary blood flow was associated with mortality. This study also showed the wide variability in medical and surgical management across institutions. The limitations of the study include its retrospective nature as well as the inability to control for various biases present in the selection of treatment options. Despite this, the study benefits from the large number of patients evaluated considering the rarity of the disease and is useful from a descriptive nature. In addition, with variables identified as being associated with mortality, more informed decisions may be made in the future for this patient population.  

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Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study. Freud LR, McElhinney DB, Kalish BT, Escobar-Diaz MC, Komarlu R, Puchalski MD, Jaeggi ET, Szwast AL, Freire G, Levasseur SM, Kavanaugh-McHugh A, Michelfelder EC, Moon-Grady AJ, Donofrio MT, Howley LW, Selamet Tierney ES, Cuneo BF, Morris SA, Pruetz JD, van der Velde ME, Kovalchin JP, Ikemba CM, Vernon MM, Samai C, Satou GM, Gotteiner NL, Phoon CK, Silverman NH, Tworetzky W. J Am Heart Assoc. 2020 Nov 3;9(21):e016684. doi: 10.1161/JAHA.120.016684. Epub 2020 Oct 20. PMID: 33076749 Free article.   Take Home Points: In this large multicenter study, the neonatal mortality in Ebstein anomaly and tricuspid valve dysplasia patients is high and risk factors include earlier gestational age at birth and lower birth weight. Echocardiographic risk factors for neonatal mortality include tricuspid valve jet velocity and a lack of antegrade flow across the pulmonary valve. Among those patients who underwent right ventricular exclusion, none died. Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric/Fetal Cardiology Journal Watch: The authors present outcome and management data from a large retrospective multicenter cohort study in fetuses with Ebstein anomaly (EA) or tricuspid valve dysplasia (TVD). From 2005 to 2011 243 fetuses with EA/TVD were included. Pre- and postnatal clinical as well as echocardiographic data were assessed in live-borns. Out of the 176 live-borns, eleven patients died within 24 hours, 7 received comfort care and 4 were excluded due to incomplete data. In the group of the remaining 154 patients, 38 patients died before hospital discharge. Figure 2 shows the management and outcome at the time of hospital discharge. The authors could show that nonsurvivors were born at an earlier gestational age and had a lower birth weight compared to survivors (please see Figure 1). In addition, they received more intense medical management. 18 patients were treated with extracorporeal membrane oxygenation and among them only 3 survived. The surgical procedures performed together with the neonatal survival, are shown in Figure 3. Patients who had more than 1 surgery in the neonatal period were more likely to die. Interestingly, all 15 patients who underwent right ventricular exclusion survived. Multilogistic regression analysis of echocardiographic data revealed that a lower tricuspid jet velocity and a lack of antegrade flow across the pulmonary valve was associated with neonatal mortality. In a multivariable analysis with echocardiographic indexes, gestational age at birth or birth weight, lower birth weight, and lower TR jet velocity were significantly associated with neonatal mortality. Overall, this study describes in detail the medical, surgical and interventional management and the outcome of this large cohort of EA and TVD patients. The presented data are helpful for clinical practice and might support to guide therapy in this complex and heterogenous group of patients with EA/TVD.  

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Anatomical Survey Versus Fetal Echocardiograms for Diagnosis of Cardiac Defects with a Single Umbilical Artery Cases: A Retrospective Cohort Study and Diagnostic Meta-analysis

Anatomical Survey Versus Fetal Echocardiograms for Diagnosis of Cardiac Defects with a Single Umbilical Artery Cases: A Retrospective Cohort Study and Diagnostic Meta-analysis View Article Tiffany Tonismae 1, Jessica H Kline 1, Jennifer J Choe 1, Frank Schubert 1, Methodius Tuuli 1, Anthony Shanks 1 J Ultrasound Med. 2020 Sep 4.  doi: 10.1002/jum.15483. Online ahead of print. PMID: 32885858.  DOI: 10.1002/jum.15483 Take-Home Points: In a fetus with a Single Umbilical Artery, a standard anatomic survey with recommended cardiac screening highly predicts congenital heart disease. Addition of fetal echocardiogram does not yield more when cardiac views are normal on anatomic survey. Commentary from Dr. Venu Amula (Salt Lake City, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Single Umbilical Artery ( SUA) in the fetus is associated with cardiac and extracardiac anomalies. The American Institute of Ultrasound in Medicine recommends a fetal anatomic survey for prenatal detection of significant abnormalities. Standard cardiac screening includes a four-chamber view, left ventricular, and right ventricular outflow tract views at a minimum.  A question arises whether those with isolated SUA would benefit from a fetal echocardiogram to rule out congenital heart disease in addition to anatomic ultrasound. To answer this question, Tonismae et al. performed this retrospective cohort study of prenatally diagnosed SUA over ten years at a single institution. The authors hypothesized that in isolated SUA, there is no additional benefit to performing a fetal echocardiogram. The study reviewed patients with documented SUA who had both an anatomical survey and a fetal echocardiogram available.  They compared cardiac anatomy on a detailed anatomy survey with a fetal echocardiogram in the fetuses with SUA.  Additionally, they report a diagnostic meta-analysis of studies with similar comparison during the period 2010 and 2019. Predictive characteristics of anatomical ultrasound were estimated with a fetal echocardiogram to detect congenital heart disease as a “gold standard.” Of the 22,666 anatomical surveys during the study period, the authors identified SUA in 320 cases. The analysis was done on 93 patients that met inclusion criteria.  Forty-four of these had normal anatomic surveys, and all were subsequently reported to have normal fetal echocardiograms.  Of the 49 who had suboptimal or abnormal anatomic surveys, thirteen had a normal fetal echo, while 36 had an abnormal fetal echo. The sensitivity and specificity of the anatomic ultrasound were found to be 100% and 77% (p < 0.01), respectively, while the positive predictive value was 73% (p < 0.01). When an anatomical ultrasound evaluation of the fetal heart was compared with the fetal echocardiogram in this population, the ultrasound had a negative predictive value (NPV) of 100% (p < 0.01). The diagnostic meta-analysis reported a pooled sensitivity of 97% (95% CI 81, 100%) and a pooled specificity of 95% (95% CI 72, 99%). The summary ROC curve showed that the anatomic survey was highly predictive of cardiac defect on fetal echo. The risk of selection bias was assessed, and the authors show significant heterogeneity between studies for both sensitivity and specificity. The study is well-conducted and addresses an essential question of fetal echocardiogram utility in those with isolated SUA and the anatomical survey showing normal cardiac anatomy. The authors highlight that fetal echocardiography is a specialized imaging modality and can impose a high cost on the mother and the health care system. Besides, the personal cost of travel to some patients living remotely and the anxiety provoked by the investigation would make it hard to recommend when a standard anatomic survey is normal. However, the conclusions have to be read in the face of the limitations of this single-center study.  The study is performed at a single prenatal diagnosis center with experience conducting anatomic surveys and standard cardiac views. Any extrapolation to other centers with heterogeneous expertise should be made with caution. Besides, no information exists on postnatal confirmation of congenital heart disease, and the authors acknowledge the limitation of using prenatal echocardiography as the “gold” standard. The current updated guidelines of the American Institute of Ultrasound in Medicine recommend obtaining the 4-chamber heart, left ventricular outflow tract, right ventricular outflow tract views, and the addition of 3-vessel view and 3-vessel trachea view. They recommend a fetal echocardiogram with SUA if the anatomical ultrasound is suspicious for CHD or if cardiac views are suboptimal.  The study reaffirms this statement. It also offers credible evidence to the debate of the lack of utility of prenatal echocardiogram when the cardiac screening, done in conformity with the standard guidelines, is normal. However, the center's experience and technical expertise to meet the guidelines will continue to dictate individual decision-making.    

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Prenatal features, associated co-morbidities and clinical course of agenesis of the ductus venosus in the current era

Prenatal features, associated co-morbidities and clinical course of agenesis of the ductus venosus in the current era View Article McBrien A, Caluseriu O, Niederhoffer KY, Hornberger LK. Prenat Diagn. 2020 Sep 13. doi: 10.1002/pd.5827. Online ahead of print. PMID: 32920862 Take Home Points: Agenesis of the ductus venosus (ADV) is rare and nearly always associated with genetic or other associated anomalies. While increased cardiac output, especially in late gestation, is common, the risk of hydrops and mortality is lower than previously suspected. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Evaluation of the ductus venosus (DV) is a routine part of the pediatric echocardiogram and risk scoring. The DV is necessary for placental blood regulation, allowing the higher oxygen concentrated blood flow to stream towards the PFO to allow for more oxygen delivery to the coronary arteries and cerebral circulation. Abnormal flow patterns of the DV have been associated with chromosomal abnormalities and absence has been associated with a wide variety of genetic, cardiac, and non-cardiac associated abnormalities, with about ⅓ having a genetic diagnosis and up to about 40% with cardiac or non-cardiac anomalies. Due to the common association with an extrahepatic connection of the umbilical vein, there is an increased risk of high output cardiac failure, with resulting hydrops or fetal demise. With a significant improvement and availability of genetic testing and imaging, the authors hypothesized that there may be a higher incidence of genetic and structural abnormalities than previously reported, and possibly better outcomes during pregnancy and postnatally. A retrospective review of patients with ADV was done between 2007-2018. Clinical records, including fetal echocardiograms, prenatal ultrasounds, and postnatal echocardiograms were reviewed. Fetal echo included any structural or functional cardiac abnormalities, combined cardiac output (CCO) measurement, and presence of hydrops. Results of genetic testing (pre and postnatal) and pregnancy outcomes were recorded. 14 cases of ADV were found, with a mean GA of 23.9 weeks. None of the cases were isolated (ADV only). 9/14 (64%) had cardiac abnormalities with 2 patients having bilateral SVC as the only cardiac diagnosis (considered to be a non-pathologic variant). 13/14 (93%) had a non-cardiac abnormality with cystic hygroma being the most frequent diagnosis (29%). 7/14 (50%) had prenatal genetic testing and an additional patient had postnatal testing. Only 14% had aneuploidy, suggesting that more robust genetic testing is necessary to obtain a genetic diagnosis. There were two postnatal deaths. See table 1. There were no terminations of pregnancy and only 1 fetal demise (hydrops at presentation).  Mean GA at delivery was 36.4 weeks. Cardiac output was measured in 10 patients and 6 had CCO above a z-score of +2 with only 2 patients developing hydrops (Figure 1). 12/14 had extrahepatic connection of the umbilical vein; the 2 with intrahepatic connection did not have hydrops. The authors discuss the specific genetic diagnoses (previously reported in Turner and Noonan Syndrome) and speculate the association with PHACE and RASA1 mutations. Additionally, they discuss the CCO elevations that were more common in the 3rd trimester/later gestation as compared to other high output conditions such as TTTS or sacrococcygeal teratoma that are more common mid-gestation, which could possibly explain why hydrops was less likely. They also speculate that longer term neurodevelopmental data may be useful given the pathophysiology of decreased oxygenated blood to the fetal cerebral circulation in those with ADV.    

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Genomic analyses implicate noncoding de novo variants in congenital heart disease

Genomic analyses implicate noncoding de novo variants in congenital heart disease. View Article Richter F, Morton SU, Kim SW, Kitaygorodsky A, Wasson LK, Chen KM, Zhou J, Qi H, Patel N, DePalma SR, Parfenov M, Homsy J, Gorham JM, Manheimer KB, Velinder M, Farrell A, Marth G, Schadt EE, Kaltman JR, Newburger JW, Giardini A, Goldmuntz E, Brueckner M, Kim R, Porter GA Jr, Bernstein D, Chung WK, Srivastava D, Tristani-Firouzi M, Troyanskaya OG, Dickel DE, Shen Y, Seidman JG, Seidman CE, Gelb BD. Nat Genet. 2020 Aug;52(8):769-777. doi: 10.1038/s41588-020-0652-z. Epub 2020 Jun 29. PMID: 32601476 Take Home Points: A genetic etiology is identified for one-third of patients with congenital heart disease (CHD), with 8% of cases attributable to coding de novo variants (DNVs). In this National Heart, Lung, and Blood Institute (NHLBI)-funded Pediatric Cardiac Genomics Consortium (PCGC) study of >13,000 patients with CHD utilizing whole-exome sequencing (WES) and chromosome microarrays rare transmitted variants and DNVs in 8% of patients with sporadic CHD. CHD probands and their parents when compared to unaffected trios demonstrated a higher burden of DNVs in individuals with CHD. DNV burden was also observed in RNA-binding-protein regulatory sites. Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric and Fetal Cardiology Journal Watch:   In this landmark study National Heart, Lung, and Blood Institute (NHLBI)-funded Pediatric Cardiac Genomics Consortium (PCGC) recruited >13,000 patients and utilized whole-exome sequencing (WES) and chromosome microarrays to study CHD genetic architecture. The analyses identified damaging rare transmitted variants and DNVs in 8% of patients with sporadic CHD (including 28% of syndromic and 3% of isolated CHD). Independent analyses of enhancers showed an excess of DNVs in associated genes (27 genes versus 3.7 expected, P = 1 × 10−5). CHD DNVs altered transcription levels in 5 of 31 enhancers assayed. The study also demonstrated DNV burden in RNA-binding-protein regulatory sites (OR = 1.13, 95% CI 1.1–1.2, P = 8.8 × 10−5).  

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Reduced right ventricular function on cardiovascular magnetic resonance imaging is associated with uteroplacental impairment in tetralogy of Fallot.

Reduced right ventricular function on cardiovascular magnetic resonance imaging is associated with uteroplacental impairment in tetralogy of Fallot. View Article Siegmund AS, Willems TP, Pieper PG, Bilardo CM, Gorter TM, Bouma BJ, Jongbloed MRM, Sieswerda GT, Roos-Hesselink JW, van Dijk APJ, van Veldhuisen DJ, Dickinson MG. J Cardiovasc Magn Reson. 2020 Jul 16;22(1):52. doi: 10.1186/s12968-020-00645-9. PMID: 32669114 Take Home Point: This study suggests that pre-existing reduced right ventricular ejection fraction in repaired tetralogy of Fallot patients is associated with impaired uteroplacental circulation Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  In this interesting study the authors investigated the association of pre-pregnancy right and left ventricular functional and uteroplacental Doppler flow parameters in pregnant women with repaired Tetralogy of Fallot (ToF). 31 pregnant women with repaired ToF presenting at ≤20 weeks of gestation and who underwent cardiovascular magnetic resonance (CMR) imaging ≤2 years before pregnancy were included (Table 1). Uteroplacental Doppler flow measurements including the pulsatility index (PI) of the uterine and umbilical arteries were performed at 20 and 32 weeks of gestation. CMR studies were used to analyse end-diastolic and end-systolic biventricular volumes, stroke volume, ejection fraction, ventricular mass and RV/LV volume ratio.In addition, CMR flow measurements in the pulmonary artery and aorta were used to analyse cardiac output and regurgitation fraction. The most important finding was that a reduced RVEF before pregnancy was significantly associated with higher umbilical artery PI at 32 weeks of gestation (Figure 1). RVEF was also significantly lower in patients with high PI. Further studies evaluating the clinical impact are needed.  

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Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing? View Article Ronai C, Freud LR, Brown DW, Tworetzky W. Prenat Diagn. 2020 Apr 20. doi: 10.1002/pd.5715. [Epub ahead of print] PMID: 32314369 Take Home Points Despite routine prenatal cardiac screening ultrasounds, congenital heart disease lesions with normal four chamber view has low sensitivity in prenatal detection. Prenatal diagnosis of pulmonary valve stenosis maybe improved with the use of cine clips to define the valve mobility and color flow Doppler to detect valvar flow acceleration. Unlike pulmonary atresia and intact ventricular septum, fetuses with pulmonary stenosis has near normal appearing tricuspid valve and pulmonary valve size making the diagnosis of pulmonary stenosis without cine clips and Doppler imaging challenging. Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch:  This is a single center retrospective chart review of all neonates who underwent cardiac catherization for critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum.  The cohort was evaluated for prenatal diagnosis of congenital heart disease and those patient’s fetal echocardiograms were reviewed. Methods:  Data was collected on all neonates who underwent cardiac catheterization from 2000-2014 at Boston Children’s Hospital with the diagnosis of critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum. Results:  A total of 178 patients met inclusion criteria with 91 patients having critical pulmonary valve stenosis and 87 patients have pulmonary atresia intact ventricular septum.   Prenatal diagnosis was significant lower in those neonates with critical pulmonary valve stenosis at 37% compared to those with pulmonary atresia intact ventricular septum 60% (p=0.003). Tricuspid and pulmonary valve measurements were evaluated for those patients with fetal echocardiograms and all patients with postnatal transthoracic echocardiograms.  The tricuspid and pulmonary valve z score was significantly higher in those patients with critical pulmonary stenosis compared to those with pulmonary atresia intact ventricular septum (p = <0.001 and p =0.004).  Comparison of the transthoracic tricuspid and pulmonary valve z score of those patients prenatally and postnatally diagnosed showed no statically significance. Discussion: In this cohort, as the authors proposed the prenatal detection of critical pulmonary valve stenosis would be significantly lower than those patients with pulmonary atresia intact ventricular septum.   The decreased detection for those patients with critical pulmonary valve stenosis was thought to be linked to a normal appearing tricuspid valve and right ventricular on the obstetric screening four chamber view, obstetric imaging of the right ventricular outflow tract was not routinely recommended until 2013 and the decreased obstetric use of cine/color flow mapping imaging.  Lastly, critical pulmonary valve stenosis may progress in severity over pregnancy therefore being undetected in routine obstetric ultrasound. Limitations:  Single center, retrospective study. Next Steps:  It would be of interest to see how many of the critical pulmonary valve stenosis patients had progressive pulmonary valve disease with a normal screening obstetric ultrasound at 18-20 weeks of gestation.  

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Telediagnosis system for congenital heart disease in a Japanese prefecture

Telediagnosis system for congenital heart disease in a Japanese prefecture View Article Mabuchi A, Waratani M, Tanaka Y, Mori T, Kitawaki J.J Med Ultrason (2001). 2020 Apr 23. doi: 10.1007/s10396-020-01020-y. Online ahead of print. PMID: 32328807 Take Home Points Fetal diagnosis via telehealth using 3D images transferred via an optical fiber network system is feasible and reliable. In 161 cases screened, cardiac defects were noted in 24 fetal scans. Though there were some discrepancies in prenatal cardiac diagnosis versus post-natal diagnosis, no significant cardiac abnormalities were missed. Appropriate post-natal planning was performed using the telehealth fetal images with significant cardiac defects birthed at a tertiary pediatric hospital whereas minor cardiac defects were birthed at the home hospital. Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   In this retrospective study from a single institution, Kyoto Prefectural University of Medicine, a spatio-temporal image correlation (STIC) based tele-diagnosis system was created for remote fetal diagnostic purposes.  STIC is a 3D technique of acquiring structural and temporal data using a single scan.  Data are digitized and cross-sectional images can be reconstructed to be reviewed at a later date.  This system was created to help screen fetal cardiac exams from distant locations from the main pediatric cardiac hospital to maximize care and minimize unnecessary travel for the families. A total of 182 fetal scans over a nine-year period (2009 – 2018) were referred for evaluation from six hospitals within the Kyoto Prefecture.  21 cases were excluded (10 – error in transmission, 11 – no postnatal diagnosis available), therefore 161 cases were studied.  Images were obtained via a Voluson E7, E8, or E10 (GE Medical Systems) and transferred images were reviewed by a pediatric cardiologist within 24 to 72 hours from transmission.  Images were evaluated using a standard protocol to assess the cardiac anatomy. Cardiac disease was noted 14.9% of cases (24/161) and accuracy of diagnosis was 95.0% (153/161).  In four cases, the cardiac diagnosis changed from prenatal to post-natal: (1) double outlet right ventricle (DORV) to ventricular septal defect (VSD); (2) DORV and pulmonary artery stenosis (PS) to single ventricular and tricuspid atresia; (3) transposition of the great arteries (TGA) to DORV and PS; and (4) total anomalous pulmonary venous connection to normal heart after the visit.  In another four cases, the prenatal diagnosis was thought to be normal, but postnatally, two patients had a VSD, one had an ASD, and one patient an aneurysm of the PDA.  Seven cases suspected of having severe cardiac issues were delivered at the tertiary pediatric hospital whereas the other patients birthed at their respective home hospitals experienced no adverse outcomes. This study is limited by its retrospective nature, single center evaluation, and relatively small cases evaluated.  That being said, this paper adds more evidence that tele-health for fetal cardiac screening purposes is feasible and allows for efficiency of medical care for both the family and health professional.    

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Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing? Ronai C, Freud LR, Brown DW, Tworetzky W. Prenat Diagn. 2020 Apr 20. doi: 10.1002/pd.5715. [Epub ahead of print] PMID: 32314369 Similar articles Select item 32354651] Take Home Points Despite routine prenatal cardiac screening ultrasounds, congenital heart disease lesions with normal four chamber view has low sensitivity in prenatal detection. Prenatal diagnosis of pulmonary valve stenosis maybe improved with the use of cine clips to define the valve mobility and color flow Doppler to detect valvar flow acceleration. Unlike pulmonary atresia and intact ventricular septum, fetuses with pulmonary stenosis has near normal appearing tricuspid valve and pulmonary valve size making the diagnosis of pulmonary stenosis without cine clips and Doppler imaging challenging.  Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch:  This is a single center retrospective chart review of all neonates who underwent cardiac catherization for critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum.  The cohort was evaluated for prenatal diagnosis of congenital heart disease and those patient’s fetal echocardiograms were reviewed. Methods:  Data was collected on all neonates who underwent cardiac catheterization from 2000-2014 at Boston Children’s Hospital with the diagnosis of critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum. Results:  A total of 178 patients met inclusion criteria with 91 patients having critical pulmonary valve stenosis and 87 patients have pulmonary atresia intact ventricular septum.   Prenatal diagnosis was significant lower in those neonates with critical pulmonary valve stenosis at 37% compared to those with pulmonary atresia intact ventricular septum 60% (p=0.003). Tricuspid and pulmonary valve measurements were evaluated for those patients with fetal echocardiograms and all patients with postnatal transthoracic echocardiograms.  The tricuspid and pulmonary valve z score was significantly higher in those patients with critical pulmonary stenosis compared to those with pulmonary atresia intact ventricular septum (p = <0.001 and p =0.004).  Comparison of the transthoracic tricuspid and pulmonary valve z score of those patients prenatally and postnatally diagnosed showed no statically significance.   Discussion: In this cohort, as the authors proposed the prenatal detection of critical pulmonary valve stenosis would be significantly lower than those patients with pulmonary atresia intact ventricular septum.   The decreased detection for those patients with critical pulmonary valve stenosis was thought to be linked to a normal appearing tricuspid valve and right ventricular on the obstetric screening four chamber view, obstetric imaging of the right ventricular outflow tract was not routinely recommended until 2013 and the decreased obstetric use of cine/color flow mapping imaging.  Lastly, critical pulmonary valve stenosis may progress in severity over pregnancy therefore being undetected in routine obstetric ultrasound. Limitations:  Single center, retrospective study. Next Steps:  It would be of interest to see how many of the critical pulmonary valve stenosis patients had progressive pulmonary valve disease with a normal screening obstetric ultrasound at 18-20 weeks of gestation.  

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Telediagnosis system for congenital heart disease in a Japanese prefecture.

Telediagnosis system for congenital heart disease in a Japanese prefecture. Mabuchi A, Waratani M, Tanaka Y, Mori T, Kitawaki J.J Med Ultrason (2001). 2020 Apr 23. doi: 10.1007/s10396-020-01020-y. Online ahead of print.PMID: 32328807 Take Home Points Fetal diagnosis via telehealth using 3D images transferred via an optical fiber network system is feasible and reliable. In 161 cases screened, cardiac defects were noted in 24 fetal scans. Though there were some discrepancies in prenatal cardiac diagnosis versus post-natal diagnosis, no significant cardiac abnormalities were missed. Appropriate post-natal planning was performed using the telehealth fetal images with significant cardiac defects birthed at a tertiary pediatric hospital whereas minor cardiac defects were birthed at the home hospital. Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   In this retrospective study from a single institution, Kyoto Prefectural University of Medicine, a spatio-temporal image correlation (STIC) based tele-diagnosis system was created for remote fetal diagnostic purposes.  STIC is a 3D technique of acquiring structural and temporal data using a single scan.  Data are digitized and cross-sectional images can be reconstructed to be reviewed at a later date.  This system was created to help screen fetal cardiac exams from distant locations from the main pediatric cardiac hospital to maximize care and minimize unnecessary travel for the families. A total of 182 fetal scans over a nine-year period (2009 – 2018) were referred for evaluation from six hospitals within the Kyoto Prefecture.  21 cases were excluded (10 – error in transmission, 11 – no postnatal diagnosis available), therefore 161 cases were studied.  Images were obtained via a Voluson E7, E8, or E10 (GE Medical Systems) and transferred images were reviewed by a pediatric cardiologist within 24 to 72 hours from transmission.  Images were evaluated using a standard protocol to assess the cardiac anatomy. Cardiac disease was noted 14.9% of cases (24/161) and accuracy of diagnosis was 95.0% (153/161).  In four cases, the cardiac diagnosis changed from prenatal to post-natal: (1) double outlet right ventricle (DORV) to ventricular septal defect (VSD); (2) DORV and pulmonary artery stenosis (PS) to single ventricular and tricuspid atresia; (3) transposition of the great arteries (TGA) to DORV and PS; and (4) total anomalous pulmonary venous connection to normal heart after the visit.  In another four cases, the prenatal diagnosis was thought to be normal, but postnatally, two patients had a VSD, one had an ASD, and one patient an aneurysm of the PDA.  Seven cases suspected of having severe cardiac issues were delivered at the tertiary pediatric hospital whereas the other patients birthed at their respective home hospitals experienced no adverse outcomes. This study is limited by its retrospective nature, single center evaluation, and relatively small cases evaluated.  That being said, this paper adds more evidence that tele-health for fetal cardiac screening purposes is feasible and allows for efficiency of medical care for both the family and health professional.                        

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Septal Flash-like Motion of the Earlier Activated Ventricular Wall Represents the Pathophysiology of Mechanical Dyssynchrony in Single-Ventricle Anatomy

Septal Flash-like Motion of the Earlier Activated Ventricular Wall Represents the Pathophysiology of Mechanical Dyssynchrony in Single-Ventricle Anatomy Hayama Y, Miyazaki A, Ohuchi H, Miike H, Negishi J, Sakaguchi H, Kurosaki K, Shimizu S, Kawada T, Sugimachi M. J Am...

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Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis O'Connor MJ, Lorts A, Davies RR, Fynn-Thompson F, Joong A, Maeda K, Mascio CE, McConnell...

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