Call a Spade a Spade: Missed Diagnosis of Apical Hypertrophic Cardiomyopathy

Abstract

Apical hypertrophic cardiomyopathy is a variant of hypertrophic cardiomyopathy characterized by apical hypertrophy, deep T-wave inversions in precordial electrocardiogram (EKG) leads, and a ventriculogram shaped like the "Ace of Spades." Patients are often asymptomatic but sometimes present with atypical chest pain, angina, or atrial fibrillation. The deep T-wave inversions on EKG often mimic acute coronary syndrome. Coronary angiogram in these patients is unrevealing, but the characteristic left ventriculogram establishes this diagnosis. The deep T-wave inversions can appear suddenly or deepen over years, making the diagnosis difficult to establish early in the disease. Transthoracic echocardiogram may miss the hypertrophied apex, but echo contrast imaging or cardiac magnetic resonance imaging can reliably confirm the diagnosis and detect apical aneurysms. We present a case of apical hypertrophic cardiomyopathy which was not evident despite many admissions, EKGs, cardiac catheterizations and echocardiograms until the diagnosis was confirmed with left ventriculogram and cardiac magnetic resonance imaging 20 years after initial presentation.