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(Circulation. 1999;100:1757-1759.)
© 1999 American Heart Association, Inc.

Images in Cardiovascular Medicine

Ebstein's Anomaly Associated With an Anomalous Coronary Artery, 2 Myocardial Bridges, and a Mahaim Fiber

James A. de Lemos, MD; Etienne Delacretaz, MD; Robert N. Piana, MD; Toussaint Smith, MD; Jose Rivero, MD; Daniel I. Simon, MD; Peter L. Friedman, MD, PhD

From the Cardiovascular Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Mass.

Correspondence to James A. de Lemos, MD, Cardiovascular Division, Brigham and Women's Hospital, 75 Francis St, Boston, MA 02115. E-mail jdelemos@rics.bwh.harvard.edu

	Introduction

 
A61-year-old man with Ebstein's anomaly was referred for evaluation of palpitations and recurrent chest pain. A ^99mTc-sestamibi exercise test showed no evidence of ischemia, and a Holter monitor and ECG event recorder were unrevealing. The patient's hospital course is summarized in the accompanying images.

An echocardiogram confirmed the diagnosis of Ebstein's anomaly (Figure 1), without associated tricuspid regurgitation or right heart failure. Cardiac catheterization revealed an anomalous right coronary artery arising from the left sinus of Valsalva and passing between the aorta and pulmonary artery (Figure 2). This congenital anomaly is associated with an increased risk for sudden cardiac death, particularly during exercise. Angiography also demonstrated myocardial bridging in the mid left anterior descending coronary artery (Figure 3) and in several obtuse marginal branches of the circumflex artery (Figure 4). Myocardial bridges have been associated with ischemic chest pain, myocardial infarction, ventricular arrhythmias, and sudden cardiac death.

View larger version (96K): [in this window] [in a new window]   Figure 1. A 4-chamber echocardiographic view showing displacement of tricuspid leaflets (TV) into right ventricle, a finding characteristic of Ebstein's anomaly.

View larger version (75K): [in this window] [in a new window]   Figure 2. Right anterior oblique (left) and left anterior oblique (right) views of right coronary artery (arrows), illustrating an anomalous origin from left sinus of Valsalva. Left, artery can be seen coursing in anterior direction, between aorta and pulmonary artery.

View larger version (91K): [in this window] [in a new window]   Figure 3. Images of mid left anterior descending coronary artery in diastole (left) and systole (right), demonstrating systolic constriction consistent with a myocardial bridge (arrow).

View larger version (89K): [in this window] [in a new window]   Figure 4. Diastolic (left) and systolic (right) images of circumflex artery and its . . . [Full Text of this Article]

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