Pediheart: Pediatric Cardiology Today

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

This week we speak with 2 pioneers in the field of pediatric cardiac critical care, Dr. Anthony Rossi and Dr. Gil Wernovsky. Both were present at the very start of the field of cardiac critical care for children. What was it like in an era before transesophageal echocardiography or even postoperative echo? Why was the advent of the bidirectional cavo-pulmonary anastomosis such a game changer in the care of children with heart disease? What do Drs. Rossi and Wernovsky think were the most important improvements to care for children with heart disease in their 35+ year careers? What about care today troubles these intensive care gurus? This is a rare opportunity to speak with two who have seen and done it all in cardiac critical care for children.

For those interested to hear Dr. Rossi speak about goal directed therapy, take a listen to episode 21 and episode 200 of this podcast!

This week we review a recent report from the team at Amrita Institute in Cocchin, India about their use of extended reality and virtual reality as well as heart model printing to aid in planning for complex intracardiac baffles for the treatment of complex congenital heart defects. What was the process used to provide actionable advice in the operating room during surgery? How has the team in southern India created a workflow that can accurately predict this complex anatomy and the patches needed to successfully septate complex hearts? We speak with the director of the 3D imaging group at Amrita, Professor Mahesh Kappanayil about this remarkable achievement of imaging in collaboration with surgery. 

DOI: 10.1016/j.jtcvs.2026.03.616

This week we review a landmark paper from the NEJM by the SCOUT-HCM study group assessing the impact of mavacamten on HCM in the adolescent patient. How does this agent work and what impact did it have on the LVOT gradients in obstructed HCM teens? Does this agent affect other biomarkers associated with more obstruction in this setting? Who is a good potential candidate for the use of this agent? Why must the EF be carefully monitored while using this agent? Dr. Joseph Rossano, Professor of Pediatrics at The University of Pennsylvania and the chief of pediatric cardiology at The Children's Hospital of Philadelphia joins the podcast to discuss this groundbreaking work.

doi: 10.1056/NEJMoa2601103