Surgical Approach to the Management of a Large Right Coronary Artery Aneurysm and Coronary Sinus Fistula

BACKGROUND: Coronary artery fistulas (CAF) are rare anomalous communications between a coronary artery and a second cardiovascular structure that can have marked physiological impact. CASE SUMMARY: A 66-year-old woman presented with progressive dyspnea and was found to have an ectatic arteriovenous fistula of the right coronary artery to the coronary sinus with resultant diffuse aneurysmal right coronary artery dilatation. She underwent fistula ligation and a triple aorto-greater saphenous vein bypass to the sinus node artery, right ventricular branch, and right posterior descending artery. Her postoperative course was complicated by ventricular fibrillation arrest and persistent moderate right ventricular systolic dysfunction. DISCUSSION: Hemodynamically significant CAF require surgical intervention. Principles of repair involve fistula ligation, exclusion of any aneurysmal segments, and myocardial revascularization. TAKE-HOME MESSAGES: CAF can present as worsening left-to-right shunts with symptoms of progressive cardiomyopathy or myocardial steal necessitating complex surgical repair. Perioperative myocardial infarction remains a serious complication.