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

2. Cardiomegaly, increased vascularity, atrial enlargement

3. 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

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

5. 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

6. Questionable LA enlargement; marked RA enlargement

9. 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

10. 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

11. References
Higgins CB. Clinical-radiographic Classification of Congenital Heart Disease. In “Thoracic Imaging: Pulmonary and Cardiovascular Radiology”. LWW; 2006:
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 May;63(5):
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