Apical Hypertrophic Cardiomyopathy
Ahealthy 38-year-old white man with no significant past medical history was referred for cardiac evaluation because of an abnormal ECG. The negative T waves (arrows, Figure 1) seen most strikingly along the midlateral precordial leads were present 5 years before presentation. Physical examination revealed a displaced apical impulse and a prominent fourth heart sound (S4). 201Tl scintigraphic analysis (Figure 2) demonstrated increased apical count density at rest. Gated tomographic imaging revealed normal overall left ventricular systolic performance, but regional wall motion analysis revealed moderate apical hypokinesis. On 2-dimensional echocardiography, an apical 4-chamber view of the left ventricle revealed hypertrophy of the apex in an “ace-of-spades” configuration (Figure 3). Follow-up 24-hour Holter monitoring revealed no atrial or ventricular ectopy. The patient was diagnosed with the benign form of hypertrophic cardiomyopathy (HCM) originally described in Japan, apical HCM, and received calcium channel blocker treatment. This apical variant constitutes ≈25% of cases of HCM in Japan but only 1% to 2% of the cases of HCM in the non-Japanese population.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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