Pediheart: Pediatric Cardiology Today

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

This week we feature a collaboration episode between the Pediheart Podcast and the PedsCrit Podcast in which pediatric critical care experts Drs. Alice Shanklin of Northwell Medical Center and Dr. Zac Hodges of UT Southwestern discuss the evaluation and management of heart block in children. What should the clinician be thinking when encountering a previously healthy child with heart block? When is pacing indicated and how should it be performed? Who is a candidate for transvenous or transcutaneous pacing, who is not and why? Drs. Pass, Shanklin and Hodges review many aspects of this in an episode from the PedsCrit Podcast. 

https://www.pedscrit.com/

This week we review a recent work from the CT group at NY Presbyterian assessing the impact of phenylephrine during pediatric congenital CT scans to lower heart rate. Why is a lower heart rate better for CCT's? Should this agent be routinely employed for this indication during CT scans in children? Dr. Yosef Cohen, pediatrics resident at the Children's Hospital at Montefiore shares his insights from a recent work he co-authored. 

doi: 10.1093/ehjimp/qyag023. eCollection 2025 Aug.

In this replay episode from 2 years ago, we delve into the world of adult congenital heart disease to review the topic of liver disease in the Fontan patient and specifically, hepatocellular carcinoma (HCC). What is the prevalence of this disease in the Fontan single ventricle adult patient? How effective are scores like the MELD-XI or Fibrosis-4 Index at identification of HCC in the Fontan patient? How should the Fontan adult patient be surveilled for this form of cancer? What evidence is there that earlier identification of HCC is associated with better outcomes? What may prove to be the most important factor in protection of the liver in the Fontan patient? Dr. Yuli Kim, Director of the ACHD program at The University of Pennsylvania shares her deep insights this week into this important topic.
DOI: 10.1093/eurheartj/ehad788

This week we review a recent Australian prospective assessment of aspirin resistance in children undergoing heart surgery. How common is this seen in this patient group and what are the reasons? What is the best test to perform to assess this and what tests may not be worthwile? Who deserves 'routine' testing for this possible problem? Cardiovascular surgeon Dr. Supreet Marathe of Queensland Children's Hospital in Brisbane, Australia shares the results of this recent publication. 

DOI: 10.1016/j.jtcvs.2025.09.013