Objective

Congenital coronary artery fistulae (CAF) are rare. Two-thirds of CAF arise from the right coronary artery (RCA). About 90% of CAF drain into the right side. We report the variation in clinical characteristics and management of CAF.


Methods

Retrospective review of database identified 18 patients with CAF between January 2001 and December 2008 (8yrs).


Results

Median age was 15.7years (11days-50yrs). Mean follow-up was 42 +/- 30.4 months.
Origin sites were RCA (n=9;50%), left main (n=8;45%) and LAD (n=1;5%). Common drainage sites were the right ventricle (n=7) & right atrium (n=7).
Therapeutic strategies were based on symptoms and shunt size. Three patients (16%) with small shunts had conservative management. 6 patients (33%) had surgical ligation. 9 patients (50%) were considered for transcatheter occlusion. One patient has been referred for surgery because of the extreme tortuosity of CAF. One patient had a diagnostic angiogram and was then lost to follow-up. 4 patients had coil occlusion with various different coils, 1 had Amplatzer PDA device, 1 had Amplatzer mVSD device and 1 had Amplatzer Vascular Plug. All had complete occlusion. The only complication was AV dissociation in one patient. There was no death. All patients are doing well at follow-up.


Conclusion

CAF have variable clinical presentation and management depending upon the size, type and manifestations. Our results are comparable with those in the literature (similar efficacy and morbidity but less mortality). Most of the series in literature are small; an international registry will provide further thoughts for optimal management.