Apical hypertrophic cardiomyopathy (AHC) is a variant of hypertrophic cardiomyopathy (HCM) in which the hypertrophy predominantly involves the left ventricular apex. The typical features of AHC include giant negative T waves in the precordial ECG leads, a spade-like configuration of the left ventricle at end-systole, the absence of an outflow tract pressure gradient, and mild symptoms. We present a patient with AHC, evidence of prior myocardial infarction with aneurysm of the apical region, and myocardial ischemia on radionuclide scanning despite angiographically normal coronary arteries. The characteristic electrocardiographic, echocardiographic, hemodynamic and angiographic findings as well as prognosis and treatment options are discussed.

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