Rare Occurrence of Apical Hypertrophic Cardiomyopathy Among Hispanics

Apical hypertrophic cardiomyopathy (ApHCM), also known as Yamaguchi syndrome represents an uncommon morphologic variant of hypertrophic cardiomyopathy (HCM) in which the myocardial hypertrophy predominantly involves the apex of the left ventricle (LV). It is exemplified by “giant” negative precordial T-waves on electrocardiography and a peculiar “spade-like” configuration of LV cavity on ventriculography historically, and more recently, on echocardiography with use of image enhancing agents. The disease entity was first described in 1976. Available literature reveals that it is prevalent largely among the East-Asian population but is rare among non-Asians. Here, we report a case of a 66-year-old Hispanic male with multiple cardiac histories including persistent atrial fibrillation, non-ST-elevation myocardial infarction (NSTEMI), and ventricular fibrillation cardiac arrest with multiple inconclusive evaluations, who later in life was found to have ApHCM. This case highlights the rare incidence of the disease among the Hispanic population and underlines the challenging diagnosis that requires a high index of suspicion in patients with cardiac symptoms, as ApHCM can masquerade as ischemic coronary heart disease. Our case also describes an unusual clinical course for ApHCM presenting with extreme clinical features, including ventricular arrhythmias and cardiac arrest, unlike the usual benign natural history of this disease.