Pediheart: Pediatric Cardiology Today

This week we delve again into the world of adult congenital heart disease and specifically the realm of biomarkers when we review a recent report of the proteomics of the adult Fontan patient. What is proteomics and how can this inform our understanding of the pathophysiology and adaptive pathways of the Fontan circulation? Are there meaningful differences in the patterns of protein expression between the adult Fontan patient and control 2 ventricle subjects? What might account for differences and how can we understand these differences to explain outcomes in the Fontan adult patient? We speak with the first and senior authors of this week's work, Professor Alexander Opotowsky of the University of Cincinnati and Mr. Ismael Assi who is a Sarnoff Fellow and soon to be 4th year medical student at the University of Cincinnati. 
DOI: 10.1016/j.jacadv.2026.102810

This week we speak with a true pioneer in pediatric cardiology, Professor Rica Arnon of Mount Sinai in NYC. Dr. Arnon's career has spanned from the mid 1970's right up to the present time where she is still working as a cardiology consultant and educator at Mount Sinai in Pediatrics after more than 50 years. In this episode we learn the travails of a pioneering women in medicine/cardiology as Dr. Arnon shares with us the challenges and triumphs of a career that balanced outstanding clinical excellence with a rich family life. Why did she travel from Israel to the US to train? How did she choose to become a pediatric cardiologist and who were her mentors? What makes a good teacher of cardiology for young learners? What advice can this master of cardiology provide regarding 'balance' in a clinically active life when one is also devoted to their family? What is better in our field today and what does Dr. Arnon believe may have been lost along the way? Dr. Arnon tells it to us 'straight' in a delightful conversation with a master clinician and teacher. 

A conversation with Dr. Michael Freed:
https://podcasts.apple.com/us/podcast/pediheart-podcast-replay-of-203-a-conversation/id1341472214?i=1000681074493

A conversation with Dr. Welton Gersony:
https://podcasts.apple.com/us/podcast/pediheart-podcast-247-a-conversation-with-living/id1341472214?i=1000607813461

This week we review a recent meta-analysis of studies assessing efficacy of adenosine in children to terminate SVT. Does the PALS recommendation of 100 mcg/kg as a starting dose for SVT management make scientific sense given what we know about the efficacy rates of this dose? Why might inadequate doses be potentially dangerous for children with acute SVT? Is there adequate data to consider changes to the recommended starting dose in this situation? Dose adenosine work well for all forms of tachycardia involving the AV node? Pediatric emergency physician and family medicine physician, Dr. Lais dos Santos of Mossoro', Brazil shares the results of a large scale meta-analysis that she performed and offers some answers to these and other questions. 

DOI: 10.1007/s00246-026-04281-5

This week we review a recent report of a novel form of CPVT (catecholaminergic polymorphic ventricular tachycardia) with associated neurodevelopmental delays. What is the genetic basis for these patients? What is different about the arrhyhthmias seen and how they are triggered in this variant? Should all patients with CPVT be screened for neurodevelopmental delays? Should those with neurodevelopmental delays and RYR2 variants be screened for CPVT? Associate Professor of Peditrics at Baylor College of Medicine/Texas Children's Hospital, Dr. Christina Miyake, shares her deep insights this week. 

doi: 10.1161/CIRCEP.124.013437

This week we go back 2.5 years and delve into the world of cardiovascular surgery when we review a review of STS data on the pulmonary artery band (PAB). The STS assigns a STAT category of 4 to this operation, denoting higher risk for mortality. Is this warranted? Are all PAB candidates equal? What features are associated with higher or lower mortality rates in patients undergoing banding? Should the data in this work drive innovation to avoid the PAB in some settings? These are amongst the questions posed to the senior author of this week's work, cardiovascular surgeon Dr. Tara Karamlou who is Professor of Surgery at the Cleveland Clinic in Cleveland, Ohio. 
DOI: 10.1016/j.athoracsur.2023.09.020