A Rare case of Coronary A-V Fistula Draining into SVC

A Congenital coronary arteriovenous fistula is a direct communication between a coronary artery and the lumen of any of the cardiac chambers, the coronary sinus, the pulmonary artery, or the superior vena cava. The number, origin, and course of the coronary arteries are otherwise normal. Coronary arteriovenous fistula is a rare congenital anomaly that is seen in 0.1 to 0.2 of coronary angiograms.They are classified into 3 categories abnormalities of origin, distribution, and termination. Coronary artery fistulae are considered to be termination abnormalities. They are uncommon and are seen in only 0.1 to 0.2 of coronary angiograms. In the majority of reported cases, coronary fistulae were found to originate from the right coronary artery (52 of cases) and to drain into the right ventricle (40 of cases). Fistulae originated from the circumflex artery in 18 of cases and drained into the pulmonary artery in 17 of cases. In the majority of cases (over 90 of patients), the coronary fistula results from an abnormal coronary artery system with aberrant termination (coronary artery fistula or coronary-cameral fistula) rather than arteriovenous fistula. In more than 90 of reported cases, the fistula terminates in the right side of the heart (approximately 40 in the RV, 30 in the RA, and 20 in the PA). It rarely terminates in the left side of the heart, but when it does, the majority enter the left atrium (LA). We present a rare case of coronary A-V fistula drains into SVC.Our patient is 42 year old not a known heart disease presented with class II dyspnea and palpitation.Clinical examination revealed continuous murmur over right sternal border.Echocardiogram showed turbulence present in LCx origin and its course.Coronary angiogram and CT angiogram showed hugely dilated tortuous left circumflex artery draining into posterior aspect of superior vena cava.