Pediheart: Pediatric Cardiology Today

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

This week we delve into cardiac surgery and genetics as we review a recent large dataset study from Drs. Anita Moon-Grady and Laxmi Ghimire who used the KID database to determine the impact of 22q11.2 deletion syndrome on outcomes of repair of TOF and Truncus. Does this syndrome affect mortality or morbidity? If so, why? Professor Moon-Grady of UCSF and Dr. Ghimire of LRGHealthcare provide many insights in this episode. doi: 10.1007/s00246-020-02333-y.

This week we review a fascinating epidemiological study from the University of Ottawa on the impact of assisted reproductive technologies (ART) and twins on the rates of congenital heart disease. What is the more important factor in the development of congenital heart disease - twin pregnancy or ART? Do all ART pregnancies require a fetal echo? We speak with 3 world authorities - epidemiologist Dr. Shi Wu Wen, pediatric cardiologist Dr. Jane Lougheed and fertility expert, Dr. Arthur Leader - all professors at the University of Ottawa. A fascinating topic and discussion! DOI: 10.1001/jamapediatrics.2019.6096

This week we re-review an impressive review of the large Australian/New Zealand registry of Fontan patients assessing the impact of AV valve regurgitation and failure on Fontan outcomes. We discuss this work with the senior author Yves D'udekem, Associate Professor of Surgery at The Murdoch Children's Research Institute and also speak with Professor Frank Cetta of the Mayo Clinic who authored a wonderful editorial on this work. Who is at most risk for AV valve failure at Fontan? How well do patients do with AV valve replacement or repair? We discuss all these issues this week on this exciting episode. doi: 10.1016/j.jacc.2018.12.025

This week we have a very topical episode on the recently identified multi system inflammatory syndrome in children associated with SARS-CoV2 infection (MIS-C). What defines this syndrome and how does it differ from other inflammatory disorders such as toxic shock syndrome or Kawasaki disease? We speak with 2 world authorities - Associate Professor of Pediatrics - Albert Einstein - Dr. Nadine Choueiter as well as Commissioner of Health for NY State - Dr. Howard Zucker. A rare opportunity to speak with a clinician 'on the ground' as well as get a public health perspective from Dr. Zucker who is also a pediatric cardiologist and anesthesiologist. One of the most interesting episodes of the series. doi: 10.1056/NEJMoa2021756 & doi: 10.1056/NEJMoa2021680

This week we review a recent large database study of WPW with particular interest in the patient with intermittent preexcitation. Traditionally, this finding has been considered a sign of low risk. Is this true? Who deserves formal invasive EP testing in this setting? Should all WPW pathways be ablated? These are amongst the many interesting questions discussed with Assistant Professor of Pediatrics - University of Alberta - Dr. Carolina Escudero. doi: 10.1016/j.hrthm.2020.05.035

This week we speak with Professor Robert Hamilton of University of Toronto about a recent potentially groundbreaking work on a novel approach to the diagnosis of Brugada syndrome using an immunological test. How accurate is this test for diagnosis of this often elusive syndrome? Is autoimmunity the cause of disease or are the antibodies just a marker of disease. What's the latest on Professor Hamilton's work on testing for ARVC (reviewed previously in episode 43)? Dr. Hamilton provides us with lots of answers this week. For those interested in collaborating in his studies, his email and that of his research coordinator are in the podcast this week. doi: 10.1093/eurheartj/ehaa383