Transesophageal echocardiography in adult-type Bland-White-Garland syndrome.

The origin of the left main coronary artery (LMCA) from the pulmonary artery (PA), known as BlandWhite-Garland syndrome (BWGS), is a rare anomaly with mortality of 90% during infancy (1). If collateral circulation between right and left coronary systems ensues, BWGS patients may reach adulthood (1,2). However coronary flow directs preferentially into the lower pressure PA, away from the left ventricle (LV) creating “coronary steal,” and left-to-right shunt. Mitral regurgitation (MR) is further common in BWGS most likely due to multiple factors (3). Surgical correction of BWGS is strongly recommended, even in asymptomatic patients, because of the high risk of developing heart failure, myocardial infarction, ventricular arrhythmias, and sudden death (1,2). A 44-yr-old man presented with recent onset of fatigue and atrial fibrillation. Transthoracic echocardiography demonstrated severe MR with a posteriordirected jet, mild anterior mitral leaflet prolapse, a dilated mitral annulus, enlarged left atrium (5.8 cm), preserved global LV function, a moderately dilated right ventricle with moderate tricuspid regurgitation, and estimated right ventricle systolic pressure of 60 mm Hg. Because of the patient’s occasional chest pain, a persantine–thallium study was performed demonstrating a significant perfusion defect of the anterior