Imaging in cardiology M.J.M. Cramer and W. Jaarsma, editors

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vr1 Fiqure 1. Fiqure demonstrates A) standard 12-lead electrocardiogram demonstrating left ventricular bypertrophy, B) twodimensional echocardiogram with 'ace ofspades' configuration at end-diastole, C) ventriculogram showing apical cavity in diastole. Apical hypertrophic cardiomyopathy

A 47-year-old white male presented for evaluation of dyspnoea and fatigue. Physical examination revealed a prominent S4, and a grade 2/6 mid-systolic ejection murmur. The electrocardiogram (ECG) revealed deep, symmetrical T-wave inversion in the mid and lateral precordial leads. Initial echocardiographic examination demonstrated hypertrophy with apical cavity obliteration in diastole. This finding was confirmed with the use of an intravenous echo contrast agent which revealed a typical 'ace of spades' configuration. Coronary angiography revealed a 40% stenosis ofthe mid left anterior descending branch (LAD) in the systolic frames, which suggested myocardial bridging. Left ventriculography demonstrated a spade-like deformity, with systolic apical cavity obliteration. The patient was started on long-acting beta-blockers. Follow-up with continu-ous 24-hour ambulatory ECG recording revealed no complex atrial or ventricular ectopy. The patient was diagnosed with a variant form of hypertrophic cardiomyopathy (HCM), also called apical hypertrophic cardiomyopathy (Yama-guchi's syndrome). This entity was first described by Yamaguchi et al. in a Japanese population where it accounted for

Netherlands Heart Joumal, Volume 9, Number 1, April 2001

up to 25% of all cases of HCM. However, this variant of HCM comprises only I to 2% ofcases in the United States. A TA. Mixon. D. Scott Gantt. T. Tak. Department of Cardiology, Scott White Clinic and Memorial Hospital, United States. Reference -

Yamaguchi H, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S, Takatsu F, et al. Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients. AmJ Cardiol 1979;44: 401-12.

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Apical hypertrophic cardiomyopathy.

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