Hypertrophic cardiomyopathy (HCM), a common cause of sudden death in the young, is characterized by unexplained left ventricular wall thickening in the presence of a nondilated cavity. Although the morphological spectrum is broad, patients with HCM may show the greatest magnitude of wallthickening of any known cardiac disease. The young patient illustrated here shows the most extreme example of hypertrophy yet observed, defining the probable outer limits of ventricular septal thickness in cardiac disease (up to 6.0 cm).
This young boy (147 lb), a talented athlete, was first identified by echocardiography as having nonobstructive HCM at age 14 after a syncopal spell associated with blood drawing, which was regarded as a vasovagal event. He remained asymptomatic for 2 years until he experienced a cardiac arrest (with documented ventricular fibrillation) while sleeping, from which he was promptly and successfully resuscitated. An automatic cardioverter/defibrillator was implanted, and the patient has survived to date without the device discharging.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s 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 Dr Hugh A. McAllister, Jr, St Luke’s Hospital and Texas Heart Institute, 6720 Bertner, MC 4-265, Houston, TX 77030.
- Copyright © 1995 by American Heart Association