Coronary Arteriovenous Fistula Ali Sepahdari, MD 4/6/07

Cardiomegaly, increased vascularity, atrial enlargement

Fundamental CXR approach Vessels -> ---------------- Heart size INCREASED NORMAL OR DECREASED ENLARGED VSD, ASD, PDA, PAPVC, other, TGA, truncus, TAPVC, TA, single vent, DORV, DOLV, HLH, multiple pulm AVM’s Ebstein’s, Pulm sten, TA w/ restr. ASD, PA w/ intact IVS, transient TR NORMAL OR SMALL TAPR TOF; Pulm stenosis and VSD with: TGA, DOR/LV; hypoplastic RV, TR w/ ASD and pulm sten

Further subdivision… VSD, ASD, PDA, PAPVC, other TGA, truncus, TAPVC, TA, single vent, DORV, DOLV, HLH, multiple pulm AVM’s

Noncyanotic disease Is there left atrial enlargement? If yes, then consider VSD and PDA, and exclude ASD and PAPVC; then ask: Is there aortic arch enlargement? If yes, then PDA is more likely than VSD Exception is in prematurity, where PDA is more common and may not show arch enlargement

Questionable LA enlargement; marked RA enlargement

Coronary arteriovenous fistula Approximate incidence is 2 in 100,000 RCA and LCA probably about equal Drainage to RV more common than RA; can drain to pulmonary artery All combinations have been seen Can be congenital or acquired -- sometimes associated with other anomalies Sometimes asymptomatic -- presentation with CHF and MI RCA to RA in this case Mavroudis published a series in which nearly all isolated fistulas were asymptomatic -- others have shown that congenital lesions are frequently symptomatic; best guess is that 50% are symptomatic

Treatment Early ligation is preferred -- expect persistent dilation of the ligated artery Sometimes do coronary bypass with mammary artery graft Endovascular embolization also extensively documented Unclear which method is more effective -- surgical ligation probably better

References Higgins CB. Clinical-radiographic Classification of Congenital Heart Disease. In “Thoracic Imaging: Pulmonary and Cardiovascular Radiology”. LWW; 2006: 679-706 Wang NK et al. Coronary arteriovenous fistula in pediatric patients: a 17-year institutional experience. Journal of the Formosan Medical Association. 101(3):177-82, 2002 Mar. Chopra V, et al. Isolated congenital coronary arteriovenous fistula. Indian Journal of Pediatrics. 67(9):661-4, 2000 Sep Trejo Gutierrez, J F, et al. Coronary arteriovenous fistula. Study of 14 cases. Archivos del Instituto de Cardiologia de Mexico. 55(2):153-64, 1985 Mar-Apr Mavroudis C, et al. Coronary Artery Fistulas in Infants and Children: A Surgical Review and Discussion of Coil Embolization. Ann Thorac Surg. 1997 May;63(5):1235-42. Kamiya H, et al. Surgical treatment of congenital coronary artery fistulas: 27 years' experience and a review of the literature. Journal of Cardiac Surgery. 17(2):173-7, 2002 Mar-Apr