講演情報
[II-TSPCJS-2]Sudden Cardiac Death in Adults with a Systemic Right Ventricle
○Chun-Wei Lu (Adult Congenital Heart Center, Department of Pediatric Cardiology, National Taiwan University Children’s Hospital, Taipei, Taiwan)
キーワード：
Systemic Right Ventricle、Sudden Cardiac Death、Adult Congenital Heart Disease
A systemic right ventricle (SRV) is most commonly seen in patients with congenitally corrected transposition of the great arteries (ccTGA) or in those who have undergone atrial switch procedures for complete transposition of the great arteries (d-TGA). Advances in medical and surgical care have significantly improved long-term survival in this population. However, adults with an SRV remain at a markedly increased risk of sudden cardiac death (SCD), arising from a range of causes and triggers. This presentation explores the current understanding of SCD in this unique group, with a focus on pathophysiological mechanisms, challenges in risk stratification, exercise recommendations, and clinical management. Key issues include progressive SRV dysfunction, arrhythmogenic substrates, and the limitations of conventional SCD risk models. We will examine the emerging roles of advanced imaging, electrophysiological studies, and novel biomarkers in identifying high-risk individuals. In addition, current guideline recommendations and ongoing controversies regarding the use of implantable cardioverter-defibrillators (ICDs) will be discussed. Ultimately, the aim is to highlight strategies for improved surveillance and individualized interventions to reduce SCD risk and enhance long-term outcomes in adults with an SRV.