Apical Hypertrophic Cardiomyopathy: A Fatal yet Underappreciated Variant of Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy (HCM) is a group of diseases affecting the left ventricle heart muscle that share a common feature of left ventricular hypertrophy without associated cardiac or systemic disorder. It was found to have a genetic basis with autosomal dominant mutations in the sarcomeric protein genes. Apical HCM is a rare subtype and underappreciated variant of HCM that primarily affects the apex of the heart. Apical HCM is dissimilar to classic HCM, with more challenges in diagnosis and inconsistent clinical course than other types.We report a case of a 91-year-old female who presented with a syncopal episode. Workup revealed atypical nonclassic features. Her transthoracic echocardiogram revealed a "spade-like" configuration of the left ventricular cavity at end-diastole consistent with apical hypertrophic cardiomyopathy. The remaining of her workup was consistent with the apical hypertrophic cardiomyopathy as a reason for the syncopal episode on presentation.Apical HCM is a distinct form of HCM that requires more attention among clinicians. In our case, the patient ended up having an implantable cardioverter defibrillator (ICD) for secondary prevention and a prescription of a beta blocker with a good outcome in her case.