We present a case of a 36 year old adult with anomalous origin of the right pulmonary artery from the aorta (hemitruncus), pulmonary hypertension, and moderate mitral regurgitation. Moreover, selective contrast echocardiogram (agitated saline injection into the right pulmonary artery) performed during preoperative cardiac catheterization demonstrated a moderate amount and prompt appearance of echo contrast in the left atrium (right pulmonary arteriovenous fistula). We successfully performed a reconstruction of the right pulmonary artery using a woven Dacron graft (12 mm in diameter). Six months postsurgery, the pulmonary arteriovenous fistula had substantially regressed, and the pulmonary arterial pressure and mitral regurgitation had both dramatically improved.