Rapid Progression of Extreme Septal Hypertrophic Cardiomyopathy
A 21-year-old man presented with syncope and a heart murmur. Screening echocardiography revealed severe asymmetric septal hypertrophic cardiomyopathy (thickness, 3.7 cm). No resting or provocable left ventricular outflow obstruction was present. Because the patient was not an athlete and performed no regular strenuous physical activity, medical therapy with β-blockers was prescribed. Subsequently, the patient remained asymptomatic but became extremely dependent on the medical therapy. Even one missed dose would lead to presyncopal episodes. On follow-up at 23 years of age, echocardiography revealed extreme septal hypertrophy (thickness, 4.2 cm) (Movies I and II; see Data Supplement). A cardioverter-defibrillator was implanted.
In this patient, the relatively rapid progression of severe hypertrophic cardiomyopathy to extreme septal hypertrophy and the consequent potential increase in risk of sudden death was believed to necessitate placement of an implantable cardioverter-defibrillator.
Movies I and II are available in an online-only Data Supplement at http://www.circulationaha.org.
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.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.