Sudden cardiac death risk stratification in heritable cardiomyopathies: phenotype-based to genotype-based risk scores.

This article discusses the risk stratification for sudden cardiac death (SCD) in patients with heritable cardiomyopathies. Risk scores have been developed and validated to predict major ventricular arrhythmia events and guide the decision to implant an implantable cardiac defibrillator (ICD) for both secondary and primary prevention. Different risk scores have been developed for different types of cardiomyopathies, such as hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC). The article also introduces a new risk score specifically for carriers of pathogenic variants in the desmoplakin (DSP) gene. The authors emphasize the importance of clinical judgment and shared decision-making when using these risk scores to determine the need for ICD implantation. [Extracted from the article]