Three-Decade Experience With Management of Coronary Artery Fistulas in Children

Abstract

Background: The treatment approach for coronary artery fistulas (CAFs) is debatable, and long-term outcomes are unknown.

Methods: This was a retrospective institutional data review of children in whom echocardiographically suspected CAFs were confirmed during cardiac catheterisation from 1997 to 2023. Treatment approach and outcomes were assessed.

Results: We identified 94 CAFs in 78 patients (42.3% male), median age 3.4 years (interquartile range [IQR] 0.9-6.6 y). Twenty-five patients (32%) had other congenital anomalies; 41 (78.8%) of the 52 patients with isolated CAFs were asymptomatic. The most common site of CAF origin and drainage was the left system (62.8%) and right cardiac cavities (80.8%). Overall median follow-up was 101 months (IQR 41-185 mo); 23 patients (29.5%) with 35 (37.2%) small or nonshunting CAFs had conservative management, and 20 (87%) of those 23 patients had an uneventful follow-up; 8 patients (10.2%) with 9 (9.6%) complex CAFs were directly sent for surgery; 1 patient had early surgical patch failure needing surgical reintervention; 47 patients (60.3%) had catheter closure of 50 (53.2%) medium- or large-sized CAFs with the use of coils (30%), vascular plugs (20%), nitinol duct occluders (40%), or material combination (10%). Six serious complications occurred. Two of the 3 patients with unsuccessful catheter procedures had subsequent surgeries. Two of the 3 patients with mild shunts had successful redo closures. One asymptomatic patient had recanalisation after 12 years and is under watchful observation.

Conclusions: CAFs have various anatomies and clinical presentations. Transcatheter closure is effective in carefully selected patients but is not complication free. Surgery is a valuable up-front option in complex CAFs or bailout of unsuccessful transcatheter closures, although it is not frequently used.