A 12-year-old girl was referred to our hospital with shortness of breath and exertional chest pain lasting for 2 years. She had a history of heart surgery about 5 years ago for a coronary artery fistula. The right coronary artery communicates with the right atrium, and it seems that the surgeon closed one side of the fistula during surgery.

Physical examination revealed the following: normal heart sounds without any murmur; lungs, clear; pulse rate, 80 min regular; blood pressure, 110/70 mmHg; routine lab tests, normal; electrocardiography, normal pattern; chest-X ray, cardiothoracic ratio normal; echocardiography, normal. Cardiac catheterization and coronary angiography were performed. There were no atrial septal defects, ventricular septal defect or patent ductus arteriosus. Coronary angiography results are shown in the Figure above.

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The coronary arteriovenous fistula (CAVfs) is an unusual anomaly that consists of a communication between one of the coronary arteries and a cardiac chamber.¹ The right coronary artery, is the site of fistula in about 55% of cases, and the left coronary artery is involved in about 35%,,¹ and both coronary arteries in 5% of connections between the coronary system and a cardiac chamber, to representrepresenting the persistence of embryonic intertra becullar space and sinusoids.¹

In the vast majority of cases (about 90%), the coronary artriovenus fistula (CAVfs) terminates in the right side of the heart, ; the right ventricle is the most common site of termination, but the right atrium and pulmonary artery are often seen as sites of termination.² the The site of termination of the fistula may be single entry or multiple entry sites.²

The shunt through the fistula most often is of small magnitude and myocardial blood flow is not

compromised.¹, really s Spontaneous closure may occur,¹ with potential complications include:including exertional chest pain, congestive heart failure and pulmonary hypertension. If if a large left to right shunt existsexists, , bacterial endocarditis, rupture or thrombosis of the fistula may be occurred.¹

1. Braunwald E, Zipes DP, Libby P, eds. A Textbook Book of Cardiovascular Medicine. 6th ed. Philadelphia: Saunders; 2001.: 1537.

2. Garson A, ed. The Science and Practice of Pediatric Cardiology. 2 nd ed. Baltimore: William and Wilkins; 1998.: 1660 – 2.