Congenital coronary-pulmonary artery fistulas complicated by continuity to a systemic artery and aneurysm are extremely rare. A 78-year-old woman was admitted to our hospital with a complaint of chest discomfort. Coronary angiography showed a coronary aneurysm and coronary-pulmonary artery fistula arising from the left circumflex artery and entering into the right lower pulmonary artery. Computed tomography angiography revealed a left subclavian artery-pulmonary artery fistula communicating with the coronary-pulmonary artery fistula. Myocardial scintigraphy showed ischemia in the inferolateral wall, indicating a coronary blood steal phenomenon because of the fistula. Endovascular therapy involves a potential risk of life-threatening complications, such as myocardial infarction, and is not recommended for aneurysms with anatomical features, such as the extreme tortuosity of the fistulous tract. Therefore, fistula ligation and aneurysmal resection were performed under cardiopulmonary bypass with cardiac arrest. The postoperative course was uneventful. The coronary steal phenomenon disappeared on postoperative scintigraphy.

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