Coronary Arteriovenous Fistula- Clinical Features, Morphologic Aspects, Diagnostic Criteria, Management Options, and Outcome

Objective: Coronary artery fistula is an unusual coronary anomaly. To thespectrum of coronary cameral communications, we want to add this separate entityof coronary cameral fistula by presenting the clinical features, morphologic aspects, diagnostic criteria, management options, and outcome in various clinical settings forbetter understanding of this developmentally intriguing, clinically complex, and therapeutically challenging disorder. Methods: From June 1992 through June 2016, 16 patients were treated for coronary cameral fistula at our institution. Ages ranged from 1 to 53 years. There were 8 male subjects and 8 female subjects. Morphologically, isolated fistulas arise from both the right and left coronary arteries, probably with similar frequency, but terminate much more commonly in the right heart or pulmonary artery than in the left heart. But in our series 76.4%(n=13) of the fistula originated from the left coronary artery and 23.6% (n=4) from the right coronary artery. The termination or drainage to the right ventricle in 47% (n=8), to the right atrium in 29.4% (n=5), to the coronary sinus and left atrium in 5.9% each (n=1 each) and to the pulmonary artery in 11.6% (n=2); In all patients the diagnosis was established by means of 2-dimensional echocardiography and transesophageal echocardiography and confirmed by means of angiography and contrast enhanced tomography scan. Fifteen patients were treated surgically, and1 patient was treated with coil embolization. Postoperative echocardiograms obtained for all patients before discharge confirmed complete obliteration of the tunnel. Results: There was no early or late mortality. All patients were discharged in stable condition. During follow-up at 3 months, 1year, 5years, 10 years and more than 10 years, all patients were in New York Heart Association class I, and echocardiography showed no residual shunts. Conclusions: Coronary cameral fistula is a rare congenital anomaly. It can be seen isolated or in association with other cardiac defects. It may arise from one coronary system or involve both coronary systems. Fistulas draining into the coronary sinus are prone to develop congestive cardiac failure. Fistulas draining in Right atrium or coronary sinus are prone to develop atrial fibrillation& its sequel. Cardiac cath and coronary angiography gives definitive diagnosis and planning management and to rule out other cardiac lesions. Computed tomography angio may replace angiography in the future for noninvasive diagnosis. Surgery is indicated for lesions not amenable to percutaneous closure. Surgical closure can be done with or without cardio pulmonary bypass by ligation, tangential arteriorraphy or closure via cardiac chamber. Most of the fistula can be dealt with in the interventional catheterization laboratory. However, occasionally large fistulas have a difficult origin and may present particular challenges to the interventional catheterization team so that they require surgical management.The location and the size of the fistula dictates technical details. Follow-up reveals excellent functional recovery.