Pediheart: Pediatric Cardiology Today

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

This week we speak with Professor Marc Gewillig of Leuven, Belgium about a recent paper he co-authored on the topic of the relation of ductal morphology to success at endovascular stenting. Are there some morphologies that are more difficult than others? How is the length of the ductus optimally estimated when choosing a stent length? Dr. Gewillig shares many pearls this week. doi: 10.1002/ccd.28725

This week we review the outcomes of Fontan palliation in the Down Syndrome patient. We speak with Assistant Professor of Pediatrics Dr. Lauren Sarno of East Carolina University about a recent work she co-authored on a complex statistical analysis she performed from the STS database. What accounts for worse outcomes in this patient population? Prepare to be surprised by the information provided this week! DOI: 10.1016/j.athoracsur.2019.07.085

This week we have the rare opportunity to speak for the entire podcast with a pioneer in pediatric heart transplantation and VAD support - Professor James Kirklan of University of Alabama. Dr. Kirklin shares his experiences in forming the PHTS and other organizations devoted to mechanical assist devices in adults and children and how he started the process of multi-institutional studies to answer important questions in surgery and medicine. Dr. Kirklin shares his thoughts regarding the future in this field and also offers some general career and life advice. This is a 'not to be missed' chance to learn from one of the greats of our time in medicine!

This week we replay one of the most listened to episodes of the early part of this podcast history - episode 21 with Dr. Anthony Rossi. Professor Rossi is the Director of Pediatric Cardiology at Nicklaus Children's Hospital in Miami, Florida and is one of the leaders and pioneers in the field of pediatric cardiac critical care. We sit down with Dr. Rossi to review his approach to the assessment and management of the single ventricle patient following stage I palliation. The clinical 'pearls' come fast and furiously from Dr. Rossi - this is one to have pen and paper available to take notes! An inspiring figure to usher out the month of May!

This week we enter the world of cardiovascular anesthesiology to review a recent work from Duke University on the results of the use of IV methadone during cardiac surgery in children. Does this agent result in reductions in postoperative opioid usage while affording similar analgesia? What are the advantages of this agent in this setting? How much should the cardiologist worry about the impact of methadone on the QT interval? We speak with the first author of this work, Assistant Professor of Anesthesiology at UNC, Dr. Annika Barnett. doi: 10.1111/pan.13903

This week we enter into the worlds of fetal cardiology and preventative cardiology to review a recent work from Professor Paul Leeson of Oxford University on the topic of pregnancy induced hypertension and the impact this plays on the cardiovascular health of the offspring. How does this form of HTN affect the structure of the RV and LV in the offspring and why might this explain the increased rates of adult HTN in the offspring of women who were hypertensive during pregnancy? Dr. Leeson provides many interesting insights and context this week. doi: 10.1161/JAHA.119.014586

This week we review a recent work from the team at Mercy Children's on the variability of sheath outer diameter (OD) amongst various sheaths. Can novel 4Fr adult radial sheaths have similar OD to more standard 3.3Fr pediatric sheaths? What are the trade-offs in using a radial sheath for this indication in a newborn? Senior author Dr. Abhay Divekar of Rocky Mountain Pediatric Cardiology shares his insights this week. doi: 10.1002/ccd.28825

On this episode we speak with Professor Edward Walsh of Harvard University about a recent work he co-authored on Ebstein's anomaly. Can review of pathological specimens offer some insights into why ablations in this patient group are so challenging? Is there an optimal age or size to perform an ablation in this patient group? Why are these ablations so difficult? Dr. Walsh, Director of the Arrhythmia Service at Boston Children's Hospital, shares his deep insights this week. doi: 10.1016/j.hrthm.2020.01.013

This week we delve into the world of cardiomyopathy and also stress echo to review a recent work from the team at Boston Children's on stress echo and its role in the assessment of the pediatric HCM patient. We speak with Assistant Professor of Pediatrics Ming Hui Chen of Harvard University about a recent work she co-authored on this modality in children with HCM. Are changes in the LVOT gradient with exercise associated with differences in medium term outcome? Dr. Chen provides the answers this week. doi: 10.1016/j.echo.2020.01.020

This week's episode, co-branded with SADS.ORG, reviews a recent guideline paper by the Mayo Clinic on how to improve the safety of potential COVID-19 therapies such as azithromycin or hydroxychloroquine. These agents can both prolong the QT interval and enhance the risk for torsades making the development of a simple protocol to prevent possible life threatening cardiac side effects critical, particularly in an era before there is strong scientific evidence of efficacy of these agents when used for this indication. Dr. Ackerman shares with us his thoughts on a brief protocol he devised with his colleagues to identify 'at risk' individuals and shares his thoughts on how to thoughtfully consider the risks of these agents when considering their use for COVID-19 therapy. A timely episode with one of the world's greatest authorities on the QT interval in Professor Ackerman. Dr. Ackerman's algorithm is located at https://cdn.prod-carehubs.net/n1/802899ec472ea3d8/uploads/2020/03/2020-COVID-QTc-Algorithm-Internal-and

This week we speak with Professor Candice Silversides of U. Toronto about a recent work she co-authored on the topic of serious cardiac events for women with heart disease. How common are serious cardiac events encountered and what percentage of these are preventable? What is the optimal team for the pregnant patient with heart disease? What are the components of a pre-pregnancy cardiac evaluation? Dr. Silversides provides many insights this week. doi: 10.1016/j.jacc.2020.01.039

This week we delve into the world of preventative cardiology and speak with Associate Professor of Pediatrics at The Albert Einstein College of Medicine, Dr. Joseph Mahgerefteh about a recent work he authored on the impact of obesity and obstructive sleep apnea on hypertension. Which of these factors, which often occur together, is more important in the youth with elevated BP? Dr. Mahgerfeteh shares his insights regarding this week's work while also reviewing a general framework to think about the assessment and approach to obesity in the young. doi: 10.1002/ppul.24693

This week we speak with Dr. Anne Marie Valente, Associate Professor of Medicine and Pediatrics, Harvard University about a recent work she co-authored on the topic of outcomes following PVR and the relation of the RV volume to these. What is the evidence for using RVEDVi as a rationale to replace the pulmonary valve? Does PVR affect long term survival in the TOF patient? What factors are associated with worse long term survival and complications? Dr. Valente is one of the foremost ACHD experts in the world and provides deep insights this week. doi: 10.1016/j.amjcard.2019.12.017.

This week we review a recent paper from the team at the University of Wisconsin regarding weaning from bypass after the Norwood procedure. If there are difficulties in 'coming off', what are the most common problems encountered and what does this difficulty mean for survival and the general hospital course? We speak with the senior author of this work, congenital heart surgeon Dr. Ronald Woods about this somewhat common problem in the OR and how his team manages it. doi: 10.1053/j.semtcvs.2019.08.005