Dilated Tortuous Right Coronary Artery to Coronary Sinus Fistula

Abstract

Coronary artery fistulas are rare congenital anomalies. The most common draining sites are the pulmonary artery and the right atrium, whereas drainage into the coronary sinus (CS) is reported in only few cases [1, 2]. We describe a rare combination of an enlarged and cirsoid right coronary artery (RCA) with a fistula draining into the CS. A 32-year-old man was referred for evaluation of heart murmur. He was asymptomatic and did not use any medication. The patient had a normal exercise capacity. Physical examination revealed a continuous heart murmur. The ECG showed sinus rhythm, a normal frontal axis. Chest X-ray demonstrated mild cardiomegaly. A transthoracic echocardiogram demonstrated an enlarged right atrium, right ventricle, CS, and RCA with a fistulous connection to the CS (Fig. 1a). The distal part of CS was dilated as high as 28 mm (Fig. 1b), and turbulent flow from it into the right atrium was shown (Fig. 1c). A contrast-enhanced multidetector computed tomography (MDCT) and a cardiac catheterization were performed to show vascular relationships. The MDCT demonstrated enormously dilated, tortuous RCA with a fistulous connection to the CS (Fig. 2a, b). Right heart catheterization showed normal right-sided filling pressures and a Qp:Qs of 1.7:1.0. Right coronary angiography confirmed a large and tortuous RCA and contrast medium passed from the RCA to the right atrium from the region of the CS (Fig. 3a). The left coronary artery was normal; however, the distal RCA filled retrograde via left-to-right collaterals (Fig. 3b). The patient underwent surgical closure of the fistula and did well postoperatively.