Abstract
Despite improved childhood survival of congenital heart disease (CHD) as a result of advances in management, late-onset sudden cardiac death (SCD) from malignant ventricular arrhythmias remains a leading cause of mortality in adults with CHD. Preventing SCD in these patients requires an understanding of the underlying pathophysiological mechanisms. Many CHD patients experience significant hemodynamic stress on the subpulmonary right ventricle (RV), leading to pathologic remodeling. Unlike acquired heart disease in which left ventricular 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.
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