Pediatric EP Genetics Oral Abstract Session (non-ACE)-Pediatric EP Genetics Oral Abstract Session (non-ACE)

Video Transcription

Video Summary

In this conference session, several speakers presented on various cardiac-related topics, emphasizing arrhythmia syndromes, patient management, and outcomes of cardiac events. Lucilia Giammarino introduced her work on identifying Calcium-Released Channel Deficiency Syndrome (CRCDS) in patients with Adiopathic Ventricular Fibrillation. She explored how dysfunctional ryanodine receptors affect calcium handling, potentially causing cardiac arrest in otherwise asymptomatic individuals.<br /><br />A pediatric cardiology study discussed survival rates in out-of-hospital cardiac arrest cases, revealing that 63% of children admitted to Texas Children's Hospital after such events did not survive to discharge. The evaluation process for these patients varied significantly, highlighting a need for standardized protocols to ensure comprehensive assessment and diagnosis.<br /><br />LQT1213, a new drug inhibiting SGK1, showed promise in reducing the QTc interval in long QT syndrome patients, with significant reductions observed in clinical trials, paving the way for future development and potential approval for clinical use.<br /><br />An international registry study on Long QT Syndrome Type 2 was presented, focusing on young patients. The study reported low mortality with effective beta-blocker therapy but noted breakthrough events mostly occurred at rest, suggesting continuous risk assessment.<br /><br />Lastly, the limited utilization and documentation of cardiac evaluation, such as ECGs and echocardiograms in children surviving cardiac arrests, were underscored. The session highlighted the significant need for better management protocols and interdisciplinary communication to improve cardiac care outcomes, especially in pediatric cases.

Keywords

arrhythmia syndromes

Calcium-Released Channel Deficiency Syndrome

ryanodine receptors

pediatric cardiology

out-of-hospital cardiac arrest

standardized protocols

LQT1213

SGK1 inhibitor

Long QT Syndrome Type 2

cardiac evaluation