Title : Predictors of sudden death in congenital arrhythmogenic syndromes
Abstract:
Genetic heart diseases are common causes of sudden cardiac death (SCD) in the young and are typically divided into inherited cardiomyopathies and primary electrical heart diseases. Cardiomyopathies associated with the risk of SCD include hypertrophic cardiomyopathy (HCM) and arrhythmogenic cardiomyopathy (ACM). The latter includes arrhythmogenic right ventricular cardiomyopathy (ARVC). Primary electrical diseases more commonly seen in clinical practice include Brugada syndrome (BrS) and long QT syndrome (LQTS). Risk stratification of SCD is a central component of the management of patients with these genetic heart diseases. Numerous risk factors have been identified, and risk prediction models have been developed to estimate the absolute risk of sustained arrhythmias and SCD to support clinicians and patients in decision-making regarding prophylactic implantable cardioverter-defibrillators (ICDs). This lecture provides a practical review of the current literature on risk stratification in ARVC and other ACMs, HCM, BrS, and LQTS, and summarizes current recommendations for ICD use.
