Pediheart: Pediatric Cardiology Today

This week we review the topic of heart block in the newborn or fetus and review a novel prospective trial assessment of hydroxychloroquine to reduce recurrences of heart block in the offspring of Anti-SSA/Ro-positive mothers. We speak with Professor Jill Buyon and Professor Peter Izmirly, both of NYU Medical Center about a recent multi center trial assessing the impact of this agent for this purpose. How effective was this drug and what if any complications were encountered? What is next in the study of this topic? Drs. Buyon and Izmirly share many insights this week. doi: 10.1016/j.jacc.2020.05.045

This week we review a recent work in the area of medical ethics specifically focused on the notion of comfort care for congenital heart disease. Which diseases or patients should be offered this option and who should not? What sort of agreement is there amongst congenital heart specialists? Who should be involved in such decisions? We speak with Professor Angira Patel of Lurie Children's Hospital about this very important and uncommonly discussed topic. doi: 10.1007/s00246-020-02367-2

Surgery is on tap this week as we discuss a recent paper from the team at Children's Hospital LA about diaphragm plication in the single ventricle patient. What impact did this intervention have on outcomes? How were hemodynamics impacted and did this change outcomes of the bidirectional Glenn or Fontan? What percentage of patients recovered diaphragm function? Professor S. Ram Kumar of USC shares his insights into his recent work with his team. DOI: 10.1016/j.jtcvs.2020.06.007

This week we review a recent report on the prevalence and types of psychiatric problems that pediatric and adolescent heart failure/transplant patients may have. How common are these illnesses, which are most common and what to do about this? We speak with Assistant Professor of Pediatrics at Vanderbilt University, Dr. Isaura Diaz about her recent work this week. DOI: 10.1016/j.jpeds.2019.10.022

This week we move into the arena of pediatric preventive cardiology and have a somewhat different format to the podcast in that we will be listening to a lecture delivered by Associate Professor of Pediatrics at The Icahn School of Medicine at Mount Sinai - Dr. Joseph Mahgerefteh - speak with us about the topic of familial hypercholesterolemia. He reviews the incidence and consequences of not identifying and managing this properly and many clinical pearls regarding this complex topic. Though a lecture aimed at generalists, this talk is a good introduction to a complex topic. Dr. Mahgerefteh's email is Joseph.Mahgerefteh@mssm.edu for anyone interested in reaching out to ask him any questions they may have regarding this important review this week.

This week we delve into the world of interventional cardiology and speak with Professor Damien Kenny of Children's Hospital, Ireland in Dublin, Ireland about a recent work he co-authored on pre-clinical animal experiments on the use of a novel bioresorbable scaffold/stent. How easy were these to implant? How did these compare with bare metal stents for patency, thrombus or inflammation in the vascular wall? Dr. Kenny shares his insights all the way from Dublin this week!doi: 10.1002/ccd.28893

This week we delve into the liver once more to review an intriguing report from the group at Johns Hopkins regarding hepatic stiffness in the 2 ventricle congenital heart patient. We know from prior works that single ventricle patients have enhanced liver stiffness but this work suggests that some 2 ventricle patients are also similarly affected. We speak with Professor Ari Cedars of Johns Hopkins University for his insights into these findings and who he thinks might warrant routine liver follow-up in the ACHD patient following congenital heart surgery. doi: 10.1016/j.athoracsur.2020

In this week's episode we replay episode 98 with Professor David Goldberg of Children's Hospital of Philadelphia and the so-called "FONTAN FUEL" trial which assessed the impact of udenafil on measures of exercise in the Fontan patient. This is one of the most important episodes of the 137 series thus far - definitely worth a re-listen. New intro and ending added this week! doi: 10.1161/CIRCULATIONAHA.119.044352

This week's episode is co-branded with SADS.ORG and is on the topic of pre-participation screening. Can the ECG offer important benefits vs. the standard 14 point AHA H&P approach? Which approach was more effective in 'real life'? We speak with Professor Robert Campbell of Emory University this week to delve into this contentious and important topic. DOI: 10.1016/j.hrthm.2020.04.032

This week we enter the world of interventional catheterization to review an important and practical topic which is the notion of blood transfusions during or immediately after catheterization in children with congenital heart disease. We speak with Dr. R. Allen Ligon of Joe DiMaggio Children's Hospital in Hollywood, Florida about a recent work he authored while completing fellowship at Emory University. Are there factors that may help predict the need for blood at cath? Dr. Ligon shares his insights this week! doi: 10.1016/j.jpeds.2019.10.033

This week we discuss the topic of mortality attributable to CHD in the US and persistent racial/ethnic disparities over the past 20 years. We speak with Professor Keila Lopez of Texas Children's Hospital about a recent large scale, 'big data' study she co-authored about this topic and review the reasons for these disparities and think about possible remedies. An essential episode on a critically important topic for CHD care. doi: 10.1161/CIRCULATIONAHA.120.046822

This week we review a recent PCCC study assessing outcomes of single ventricle patients who have undergone replacement of the AV or semilunar valve. We speak with Professor Geetha Raghuveer of University of Missouri-Kansas City about this work she co-authored. Outcomes are generally poor in this patient group but are there lessons that can be gleaned from the data regarding optimal timing for repair or replacement of valves in this patient group? This is a great follow-up of episode # 58 from a year ago. doi: 10.1007/s00246-019-02234-9.

This week we delve into the world of electrophysiology and ACHD when we review a very recent work on TOF and factors associated with possible risk for sudden death. Can a novel algorithm identify high or low risk patients in this cohort? We speak with Professor Joseph Atallah of The University of Alberta and Dr. Edward Walsh of Harvard University. Both share their deep knowledge in a roundtable discussion this week. DOI: 10.1016/j.amjcard.2020.07.012

This week we enter the dual worlds of ACHD and echocardiography to review a recent work from Amsterdam on the use of speckle tracking echocardiography to predict clinical outcomes in the patient with a systemic RV. Can this modality predict a problem prior to more conventional factors or even CMR data? Can this substitute for a CMR? Why is this type of echo more challenging in the systemic RV patient? Dr. Odilia Woudstra shares her deep knowledge of this topic from her research this week! doi: 10.1016/j.cjca.2019.12.014

This week we explore an important paper from CHOP on the optimal approach to repair for Tetralogy of Fallot (TOF). Is there a benefit to a staged approach versus primary complete repair? Are there factors that might influence this decision of approach? We speak with Assistant Professor of Pediatrics Dr. Laura Mercer Rosa of The University of Pennsylvania about her recent large PHIS database study. This is a rare replay episode from last year but one of the very best of the entire series thus far with redone intro and musical endings. doi: 10.1016/j.jacc.2019.05.057.

This week we review a recent work from the C3P0 registry on trans-catheter pulmonary valves. How common were adverse events encountered in this 'real world' assessment? How does this value compare with surgical pulmonary valve replacement? What factors are associated with higher doses of radiation in this procedure? We speak with Dr. Barry Love, Director of the Congenital Catheterization Laboratory at Mount Sinai Kravis Children's Hospital who wrote a very insightful accompanying editorial comment on this paper. doi: 10.1016/j.jacc.2019.11.042

This week we delve into the topic of fellow wellness and review a recent work from the Boston Children's group on fellow fears and stressors when beginning their training. Are there patterns amongst the fellow concerns that can be identified and used to inform interventions to help allay these fears and reduce the stress? We speak with Dr. David Brown, Associate Professor of Pediatrics, Harvard Medical School and also Dr. Tony Pastor who is the ACHD Fellow at Boston Children's Hospital/Brigham and Women's Hospital about their thoughts on fellow fears and stressors as well as fellow (and doctor) wellness. An important topic for our milestone 130th episode. doi: 10.1007/s00246-019-02276-z.

This week we speak with Assistant Professor of Pediatrics at the Icahn School of Medicine at Mount Sinai as well as Chief, Pediatric Cardiology at BronxCare Health System, Dr. Kanika Mathur, about a recent work she co-authored on the topic of enalapril in the single ventricle patient. How safe are these agents in the single ventricle patient? How common were side effects encountered at standard dosing? Dr. Mathur shares her insights from a recent large database analysis study. DOI: 10.1016/j.jpeds.2020.07.058

This week we review a recent report of the novel IV anti arrhythmic agent sotalol. We speak with EP fellow Dr. Alejandro Borquez of Rady Children's Hospital about this agent and where the team at Rady believes it fits in the 'armamentarium' of agents used to treat difficult arrhythmias in children. How effective is the drug for 'regular' SVT or infant atrial flutter? How commonly were adverse events seen with its administration? Dr. Borquez provides the answers this week. doi: 10.1016/j.jacep.2019.11.019

This week we enter the worlds of fellow education and echocardiography to review a recent report of a QI project performed at Children's Hospital of Philadelphia regarding the performance of echocardiograms by first year fellows in cardiology. Can the implementation of a checklist protocol improve the consistency of echocardiograms performed by fellows? What was the impact of this protocol? What areas were particularly well served by this approach? Dr. Brian White, senior cardiology fellow at CHOP provides important insights into this project that he co-authored. doi: 10.1111/echo.14441