Sudden cardiac death in congenital arrhythmogenic syndromes: Risk stratification and management

In young individuals, sudden cardiac death (SCD) is often caused by genetic heart diseases, which are usually classified into inherited cardiomyopathies and primary electrical heart diseases. The aim of the review is to describe primary electrical diseases and define predictors of SCD. Primary electrical diseases (more commonly) encountered in clinical practice including Brugada syndrome (BrS) and long QT syndrome (LQTS). Several risk prediction tables have been developed to estimate the likelihood of SCD after identifying multiple risk factors. Syndromes that need ICD, cardiac arrest survivors, arrhythmogenic right ventricular dysplasia, LQTS or BrS with syncope or + need for electrophysiological study, early repolarization syndrome, Wolff-Parkinson-White Syndrome with ventricular tachycardia, catecholaminergic polymorphic ventricular tachycardia. Congenital arrhythmogenic syndromes are relatively common and represent an important cause of SCD in athletes and young individuals. Early recognition requires a high index of suspicion, particularly in cases of unexplained syncope or tachyarrhythmia, to enable timely risk stratification and preventive intervention.