Pathological findings in apical hypertrophic cardiomyopathy: an indication for orthotopic heart transplantation – Case Report

Apical Hypertrophic cardiomyopathy (HCM) is a relatively rare morphological expression of the disease (<5% of patients), in which left ventricular wall thickening is confined to the most distal portion of the ventricles, below the papillary muscle level. Crypts may be an early sign of maladaptive myocardial alterations that ultimately lead to the development of the characteristic hypertrophy in HCM patients. Distinguishing this entity with noncompaction cardiomyopathy clinically is difficult proposition. The very recent discovery of the crypts in HCM carriers probably means that HCM carriers have been misdiagnosed in the past as having some form of noncompaction cardiomyopathy. However, distinguishing between these 2 cariomyopathies has important clinical therapeutic implications because HCM carriers may remain asymptomatic for decades and patients with noncompaction cardiomyopathy are at increased risk of thromboembolic events and supraventricular arrhythmia when heart failure is present.