A 35-year-old woman was seen because of a two-year history of exertional dyspnea. She had a high-pitched systolic and diastolic murmur and evidence of left ventricular hypertrophy. Increased hemoglobin oxygen saturation was noted at the level of the pulmonary trunk, and indocyanine green dye curves were characteristic of a left-to-right shunt. Aortic root angiography revealed that the right coronary artery arose from the pulmonary trunk and that blood flow was reversed. The incidence, clinical manifestations, and therapy of this rare syndrome are reviewed.
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