A 67-year-old man with a 3-year history of dyspnea on exertion arrived to our institution with discontinuous palpitations and short breath. He was diagnosed with congenital coronary artery fistula (CAF). Angiography revealed 3 giant aneurysmal formations and coronary artery calcification. We report a case of successful repair of CAF with a giant coronary aneurysm by closing the orifice and resecting the aneurysm and reconstructing the left coronary artery. The surgical procedure included closure from within a vessel dilated by aneurysm and excision of the aneurysm. We were able to completely obliterate the fistula and preserve the normal blood flow through the coronary arteries post operation. The postoperative course was eventful, but the patient was discharged home. The patient was doing well at his 28-month follow-up visit.
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