Histopathological Changes and Clinical Implications in Patients with Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy (HCM) is a familial disease with a wide clinical spectrum. Many patients have normal life expectancy, some die suddenly – often as adolescents or young adults – whereas only a few develop end-stage heart failure. In the total HCM population, only 25% develop significant left ventricular outflow tract obstruction. The histopathological hallmarks of HCM are general myocyte hypertrophy, regions of myocyte disarray, different types of fibrosis and small-vessel disease. To better understand the disease development and the aetiology of the electrical instability leading to serious arrhythmias and sudden cardiac death (SCD) in these patients, it is important to explore the relationship between histopathological features and clinical findings and outcome in patients with HCM. This article provides a brief overview of this theme.