Annual Meeting of Japanese Society of Pediatric Cardiology and Cardiac Surgery

Background: Treatment approach of coronary artery fistulas (CAFs) and long-term outcomes are debatable. Objectives: To evaluate our experience with CAF management. Methods: Retrospective data review of children in whom echocardiographically suspected CAFs were confirmed during cardiac catheterization from 1997-to-2023. Treatment approach and outcomes were assessed. Results: We identified 94 CAFs in 78 patients (42.3% males), median age of 3.4years (IQR, 0.9-6.6). 25 (32%) patients had other congenital anomalies and 9 (11.5%) had associated coronary artery anomalies. 41/52 (78.8%) patients with isolated CAFs were asymptomatic. Most common site of CAF origin and drainage was the left system (61.7%) and right cardiac cavities (80.8%). Overall median follow-up was 101months (IQR, 41-185). 23 (29.5%) patients with 35 (37.2%) small or non-shunting CAFs had conservative management and 20/23 (87%) patients had uneventful follow-up. 8 (10.2%) patients with 9 (9.6%) complex CAFs were directly sent for surgery. One had early closure patch failure and redo surgical ligation 2-month postoperative. 47 (60.3%) patients had catheter closure of 50 (53.2%) medium or large-sized CAFs using coils (30%), vascular plugs (20%), Nitinol duct occluders (40%), or material combination (10%). Six serious complications occurred. 2/3 patients with unsuccessful catheter procedures had subsequent surgeries. 2/3 patients with mild shunts had successful redo closures. One asymptomatic patient had trivial recanalization after 12 years. Conclusions: CAFs can present with various anatomic configurations and clinical syndromes. Transcatheter closure is effective in carefully selected patients but is not complication-free. Surgery is a valuable upfront option in complex CAFs or bailout of unsuccessful transcatheter closures.