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Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium and is most often caused by mutations in sarcomere genes. The structural and functional abnormalities are not explained by flow-limiting coronary artery disease or loading conditions. The disease affects at least 0.2% of the population worldwide and is the most common cause of sudden cardiac death in young people and competitive athletes because of fatal ventricular arrhythmia. In some patients, however, HCM has a benign course. Therefore, it is of utmost importance to properly evaluate patients and single out those who would benefit from an implanted cardioverter defibrillator. In this article, we review and summarize the sudden cardiac death risk stratification algorithms, methods of preventing death due to HCM, and novel factors that may improve the existing prediction models.