Pediheart: Pediatric Cardiology Today

This week we turn back the clocks to re-review a recent research letter from the team at the Harvard Congenital Heart Program about venous insufficiency in the Fontan patient. Why do patients with Fontan circulation develop chronic venous insufficiency and what are the implications of this problem in the Fontan patient? What sort of evaluation should be performed on the Fontan patient presenting with lower extremity venous changes? How worried should the cardiologist be when presented with a Fontan patient with these changes? These are amongst the questions reviewed on this topic with this week's guest, Associate Professor at Baylor University, Dr. Tony Pastor.JACC Adv 2022 Mar, 1 (1) 100002

This week we delve into the world of heart failure and transplantation when we review a recent PHTS study assessing the impact of ischemic time on outcomes of pediatric heart transplantation. What is considered to be a long ischemic time and what is the impact on outcomes in the pediatric patient undergoing transplant? Why does it appear as the impact of a longer ischemic time is less associated with graft failure in the present era in comparison to the past? What might be the impact of newer heart perfusion devices used to transport organs for transplant? These are amongst the questions reviewed with Professor of Pediatrics at U. Colorado, Dr. Scott R. Auerbach. DOI: 10.1016/j.healun.2024.03.002

This week we review a recent report assessing LV function and properties in the Ebstein's anomaly patient using CMR. How often is LV dysfunction observed in the Ebstein's patient who is about to undergo surgical Cone repair? How often did the echo underestimate LV dysfunction in comparison to the 'gold standard' CMR? How often is LV non-compaction observed when using strict criteria in this disease? Can CMR assessment of LV function preoperatively predict postoperative ICU course? These are amongst the questions posed to the first author of this week's work, advanced imaging fellow at U. Pittsburgh, Dr. Aditi Gupta. https://doi.org/10.1007/s00246-024-03585-8

This week we delve into the world of electrophysiology and review a recent report from the team at Primary Children’s hospital on the use of intravascular ultrasound for EP ablation procedures in children. How is this technology useful for ‘routine’ ablations in kids? Are there some forms of tachycardia where its use is more important than others? How does it lower procedural time or improve efficacy? We speak with Dr. Thomas Pilcher, chief of pediatric electrophysiology at Primary Children’s about his center's recent report in the use of this technology for ablation in children and those with congenital heart disease. https://doi.org/10.1093/europace/euae047

This past week marked the passing of the great pediatric cardiology pioneer, Dr. Sylvia Griffiths. Dr. Griffiths was a trainee of Dr. Ruth Wittemore of Yale University and was one of the founders of the pediatric cardiology program at NY Presbyterian Hospital in the mid 1950's with Dr. Sidney Blumenthal. Her career spanned 7 decades, all at NY Presbyterian, Columbia University. She is remembered as a pioneering woman in medicine as well as a pioneer as a pediatric cardiologist. She is also remembered as a kind and warm colleague and friend. In today's 304th episode of Pediheart we review a 55 year old paper by Dr. Griffiths on outcomes of partial AV canal repair and speak with the present chief of pediatric cardiology at NY Presbyterian, Dr. Christopher Petit about the important role she played at Columbia. We then speak with Professor of Pediatrics at the Albert Einstein College of Medicine, Dr. Christine Walsh about the role she played in Dr. Walsh's life as well as the lives of so many patients, colleagues

This week we review a recent paper from last 2023 about outcomes of cardiac transplantation in the adult congenital heart patient (ACHD). Little has been written on this topic until this very robust and large scale report. How do single ventricle ACHD patients fair at transplant and how do they compare to non-ACHD heart transplant recipients? Why might 1 and 3 year outcomes not be a 'fair' method of assessing outcomes in this very high risk patient group? How do HLHS patient outcomes compare with other single ventricles? Are there better ways to measure risk in this patient group and how might the data in this work inform risk stratification and management of failing Fontan patients in whom transplantation is being considered? These are amongst the questions posed this week to the week's expert, Dr Daphne Hsu who is Professor of Pediatrics at the Albert Einstein College of Medicine at The Children's Hospital at Montefiore. https://doi.org/10.1016/j.jacc.2023.06.037For those interested in learning more about D

This week we go back in time to 20222 to re-review an episode focusing on gender disparities in salary in the field of congenital electrophysiology. To what degree are female electrophysiologists paid less than their male counterparts despite similar levels of education, work effort and seniority? What interventions could be enacted to help 'mind the gap' and level the playing field in this regard? We speak with the chief of pediatric electrophysiology at The Children's Hospital of Philadelphia, Professor Maully Shah, who is the first author of this week's research letter. doi: 10.1016/j.jacep.2022.02.011

This week's topic is ductal stenting in a low resource environment. We review a recent report from the team at Amrita Institute in Kerala, India about their large, over 10 year experience in ductal stenting. Why has this approach superceded BTTS in this environment? How does the team determine if a patient is a candidate and what morphologies of duct would be inappropriate for intervention? How does the team manage PGE prior to ductal stenting to allow that perfect balance between pulmonary blood flow and ductal constriction to improve procedural success? What can operators in the west learn from the experience of these operators? We review these questions and more with the first author of this work, Dr. Navaneetha Sasikumar who is Associate Professor of Pediatrics at the Amrita Institute.DOI: 10.1007/s00246-024-03496-8

This week we review a novel multivariable model used to predict mortality in the repaired tetralogy of Fallot patient. Can this model identify who amongst the repaired TOF patients is at risk for death in the coming years? What are the most important inputs to this model? Why does the use of a validation cohort provide greater strength to this model and is there still room for improvement in the model? Can the data in this work help inform decisions regarding pulmonary valve replacement timing? These are amongst the questions posed to the senior author of this work, Cardiologist-in-Chief at Boston Children's Hospital and Professor of Pediatrics at Harvard University, Dr. Tal Geva. Novel risk calculator: https://github.com/rTOF-INDICATOR/MORTALITY-RISK-Scoredoi: 10.1161/JAHA.123.034871. Epub 2024 Jun 11.

This week we listen in to a wonderful roundtable discussion with 3 of the pioneers in the world of pediatric electrophysiology, namely Dr. John Triedman, Dr. J. Philip Saul and Dr. Edward P. Walsh. All three were 'there at the beginning' and share their memories of how they helped to shape the field of pediatric EP. How did Drs. Walsh and Saul choose to proceed with EP training in a pre-ablation and pre-pediatric EP training era? How did all three decide to start a fellowship program to train the next generation of EP doctors? What sort of 'technology' existed to perform ablations in Boston in the early 1990's and how did these experts come to be the first to perform an RF ablation in any human in Boston? How does the field differ today in comparison to its origins? Amongst their many professional accomplishments, what does each 'guru' view as their greatest professional achievement? These are amongst the many questions posed during a wonderful symposium that was held in Boston on 5/16/24 to celebrate both th

This week we review a recent editorial commentary from multiple congenital heart surgical leaders in the US today. The topic of their expert opinion piece is the concept of public reporting of results and their 'amplification' through organizations such as US News and World Report, to name one. How has the use of the database from STS been sometimes misinterpreted through public reporting or ranking systems? What was the initial intent of the STS database and how does the present usage of these data differ from the initial intent? Do STS risk adjustment models capture all aspects of risk for patients undergoing surgery and how do deficiencies in this result in unfair 'rankings' of programs? Can we 'take control' of our data and interpret it more clearly and accurately for the public and reduce misusage or misinterpretation of the data? How can public reporting result in improved outcomes? How can 'gaming' of the system be reduced? Dr. Emile Bacha, Professor of Surgery at Columbia University shares his deep in

In this week's podcast, we discuss a work that tries to challenge convention when we review a recent work from the team at Emory University about anticoagulation following left sided ablation of SVT in children. A short course of aspirin is commonly prescribed in this setting but what is the evidence for this? How do patients who receive aspirin fare vs. those who do not? Are there some patients in which aspirin might be more 'useful' than others? Is there adequate equipoise to consider a multicenter trial? Is that even necessary? Joining us to discuss this and more about this topic is Dr. Vincent Palmieri, senior electrophysiology fellow at Children's Healthcare of Atlanta. doi: 10.1016/j.hrthm.2024.01.004.

This week we review a recent large scale, multicenter study of the Pediatric Cardiac Intensive Care Society Investigators on outcomes of pulmonary atresia with intact ventricular septum (PA/IVS) in the present era. How are patients faring in the present era in comparison to the prior 1-2 decades? What are the most important factors to account for improvements observed in survival? Are there subtypes of PA/IVS that warrant early referral to heart transplantation? What is the best predictor of a better outcome and what percentage of PA/IVS patients ultimately have a 2 ventricle repair? Where are the areas for improvement in the coming decade to improve upon these already, improved outcomes? These are amongst the many questions reviewed in this week's episode with the first author, Dr. Ilias Iliopoulos, who is the director of pediatric cardiac critical care at Inova LJ Murphy Children's Hospital.https://doi.org/10.1007/s00246-022-02954-5

In this replay of our milestone 200th episode of Pediheart, we review a 2005 paper from the team at Nicklaus Children's Hospital on how lactate level was used to monitor oxygen delivery and consumption in the postop congenital heart patient and the impact that a protocol involving frequent monitoring and response to this value improved outcomes in the pediatric CICU. Joining us is the first author of the work, Dr. Anthony Rossi, former chief of cardiology at NIcklaus Children's Hospital. Once again, let's come 'on rounds' with this master of postoperative care to learn how he developed his approach to assessing the pediatric postoperative patient in his quest to identify the right 'goal' of therapy that could potentially mitigate the need only for experience at the bedspace.doi: 10.1007/s00134-004-2504-1

This week we delve into the world of interventional cardiology and congenital heart surgery when we review a recent report from the team at Toronto Sick Children's Hospital about their experience with interventions in the pulmonary arteries following the arterial switch operation. How often are branch or main pulmonary artery narrowings serious enough to warrant intervention? What degree of RV pressure should warrant a cath or intervention? How commonly did simple balloon dilation result in a meaningful degree of reduction in pulmonary artery obstruction and/or RV pressure? How commonly were complications observed? These are amongst the questions reviewed this week with the work's first author, senior cardiology fellow at the University of Toronto, Dr. Michael N. Gritti.https://doi.org/10.1007/s00246-024-03408-w

This week we review a report from Italy regarding the observation of RA disk thrombus formation in some patients following GORE Cardioform ASD closure. How common is this complication seen and are there some patient or device characteristics that would be considered higher risk for this complication? How does this information square with the Assured trial data? Why might there be differences between the two data sources? We speak with Associate Professor of Pediatrics at Kravis Children's Hospital at Mount Sinai, Dr. Barry Love who is also Director of the Congenital Cardiac Catheterization Laboratory at Mount Sinai Hospital.  https://doi.org/10.1002/ccd.31002

This week we delve into the world of antibiotic prophylaxis for dental procedures. What are the data to support its use in the congenital heart patient? Why has this been such a difficult topic to study? What do we now know about this topic and what still remains unanswered? Given the low prevalence but dire consequences of this disease, how can investigators consider studying this topic in the future? Is there adequate equipoise to proceed with a randomized controlled trial? We speak with Advanced Imaging Fellow at Boston Children's Hospital, Dr. Francesca Sperotto of Harvard University about her recent multicenter meta-analysis of 30 studies including over one million cases of endocarditis. DOI: 10.1001/jamacardio.2024.0873

This week we review a recent work on the use of sodium-glucose cotransporter 2 inhibitors in the ACHD heart failure patient from Europe. Can these agents impact heart failure outcomes in this patient group? What is the mechanism by which they may improve outcomes? Are there data on their effects on the single ventricle patient? What sorts of side effects can be seen and are these more or less commonly observed in the ACHD patient? We speak with the corresponding author of this week's work, Dr. Anastasia Egorova of Leiden University in the Netherlands. https://doi.org/10.1016/j.jacc.2024.02.017

This week we delve into the world of fetal cardiology when we review a recent large, multicenter study on the impact of prental diagnosis of 22.q11 deletion syndrome. How did the prenatally diagnosed patients differ from those diagnosed postnatally in regards to degree of heart disease and postnatal morbidity and mortality? What is the role of cell-free DNA testing in the screening for patients with this deletion syndrome? How do outcomes differ for this syndrome between the US and other countries and why might this be? These are amongst the many questions reviewed with the first author of this week's work, Dr. Lindsay Freud who is the director of fetal cardiology at Toronto Sick Kid's Hospital and also Associate Professor of Pediatrics at the University of Toronto. Also joining the podcast this week are the co-directors of this year's Mount Sinai Imaging Symposium entitled: Conversations in Care - The Single Ventricle. Dr. David Ezon, Dr. Jennifer Cohen, Dr. Kenan Stern and Mount Sinai Technical Director and

This week we delve into the world of cognitive bias when we review a recent review of this topic by the team at the University of Arkansas. What are some of the more important common cognitive biases that plague humans and are particularly deleterious for good decision making in 'case management/surgical' conference? What are some approaches to reducing the impact of these biases? How does the advent of ZOOM enhance or reduce bias for surgical conference decisions? We speak with noted authority on this topic, Dr. Joshua Daily who is Associate Professor of Pediatrics at The University of Arkansas in Little Rock for his deep insights into this topic. https://doi.org/10.1007/s00246-024-03462-4Other resources mentioned by Dr. Daily include:- Thinking Fast and Slow by Daniel Kahneman- Thinking In Bets by Annie Duke - Predictably Irrational by Dan Ariely- How To Decide by Annie Duke