In this case report we describe an unusual appearance seen on a ventilation-perfusion (V/Q) scan in a woman with pulmonary hypertension. Although the pulmonary hypertension was not caused by pulmonary emboli, the V/Q scan suggested several cardiac anomalies which may lead to pulmonary hypertension. Most of the cardiac anomalies, including right-sided aortic arch and right-to-left shunt, can be deduced from careful examination of the V/Q scan. A subsequent cardiac MRI scan confirmed the anomalies.

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