RESUMO
INTRODUCTION AND IMPORTANCE: Coronary artery abnormalities are uncommon and mostly asymptomatic. The combination of double right coronary artery (RCA) with a fistula and valvar deformity is very rarely reported in the literature. However, it is important to identify these deformities as they have relatively high complication rates. PRESENTATIONS OF CASE: A 47-year-old male came with chest pain that radiated to the lower jaw. ECG showed equivalent changes. Blood tests including troponin were within normal range. However, echocardiogram showed a severe mitral valve regurgitation with anterior leaflet prolapse and hypokinesia of the ventricular wall. Coronary angiogram showed a double RCA with a complete block in the main RCA and a fistula to the right atrium (RA). The left coronary system showed atherosclerosis in left anterior descending artery (LAD) and circumflex artery (CX). Surgical treatment, including the repair of the RCA-RA fistula, replacement of mitral valve and coronary artery bypass grafting (CABG) were performed. The patient had no complications in the follow-ups. DISCUSSION: Coronary fistulas may be congenital or acquired malformations. Their treatment depends on the symptoms, origin, size and the receiving chamber. Furthermore, double RCA is debatable whether the rate of atherosclerosis and other cardiac abnormalities are increased with this anomaly. The surgeon must keep in mind these rare anomalies before cardiac operations. CONCLUSION: Double RCA might accompany other deformities which are important to detect before intervention. More studies are required to decrease complications and have better outcomes.