Predictors of sudden death in congenital arrhythmogenic syndromes

Background: Genetic heart diseases are common causes of sudden cardiac death (SCD) in young individuals, and are typically divided into inherited cardiomyopathies and primary electrical heart diseases. Hypertrophic cardiomyopathy (HCM) and arrhythmogenic cardiomyopathy (ACM) are both cardiomyopathies that increase the risk of SCD.

Objective: To define risk predictors of SCD. Primary electrical diseases that are more commonly seen in clinical practice include Brugada syndrome (BrS) and long QT syndrome (LQTS). Risk stratification for SCD is a must in managing patients with these genetic heart diseases. Numerous risk factors have been identified, and risk prediction models have been developed to estimate the risk of sudden cardiac death (SCD). These tools assist clinicians and patients in making decisions about the use of prophylactic implantable cardioverter-defibrillators (ICDs).

Conclusions: This paper examines the current literature on risk stratification in ARVC and other ACMs, such as BrS and LQTS, and summarizes current recommendations for using ICD.

To be updated shortly..