Role of Subpulmonary Right Ventricle in Sudden Cardiac Death in Adults with Congenital Heart Disease

Despite improved childhood survival in congenital heart disease (CHD) due to advances in management, late-onset sudden cardiac death (SCD) from malignant ventricular arrhythmias remains a leading cause of mortality in adults with CHD (ACHD). Preventing SCD in these patients requires an understanding of underlying pathophysiological mechanisms. Many CHD patients experience significant hemodynamic stress on the subpulmonary right ventricle (RV), leading to pathological remodeling. Unlike in acquired heart disease where left ventricle (LV) pathology is prevalent, RV pathologies are crucial in the SCD pathogenesis in CHD patients. This review examines the mechanisms and management of SCD related to subpulmonary RV pathologies in CHD patients.