Ebstein’s Anomaly Associated With an Anomalous Coronary Artery, 2 Myocardial Bridges, and a Mahaim Fiber
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.
Electrophysiological study (Figure 5⇓) identified antidromic atrioventricular reentrant tachycardia (AVRT) with anterograde conduction over a decrementally conducting accessory pathway (Mahaim fiber). Endocardial mapping localized this pathway to the anterolateral tricuspid annulus. Application of radiofrequency current to this site instantly terminated the tachycardia, after which no residual accessory pathway conduction remained and no reentrant tachycardia could be induced.
The patient subsequently underwent single-artery coronary artery bypass graft surgery, with a right internal mammary artery graft to the right coronary artery. In addition, the myocardial bridge in the left anterior descending artery was “unroofed.” The patient recovered uneventfully and remains free of chest pain and palpitations.
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 Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
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