Three-Decade Experience with Management of Coronary Artery Fistulas in Children.
Haddad RN, Bonnet D, Malekzadeh-Milani S. Can J Cardiol. 2024 Jun 6:S0828-282X(24)00430-6. doi: 10.1016/j.cjca.2024.05.028. Online ahead of print. PMID: 38851390
Take home points:
- Coronary artery fistulas are rare and are heterogeneous in their anatomy and presentation.
- Clinically asymptomatic CAFs can be managed conservatively without long-term complications.
- Transcatheter closure is effective in appropriately selected patients but carries a relatively high risk of complications making it imperative that providers are prepared to deal them should they arise.

Commentary from Dr. Konstantin Averin (Cohen Children’s Heart Center), catheterization section editor of Pediatric Cardiology Journal Watch:
Coronary artery fistulas (CAFs) are rare anomalous connections from the normal coronary artery to another cardiac structure. These lesions range in severity from completely asymptomatic to those that can cause myocardial ischemia, primarily driven by the diameter of the orifice and length of the fistulous connection. Data on management and outcomes are limited so the authors sought to describe a 3-decade (1997-2023) single center experience with the management of CAFs in children.
During the study period 94 CAFs in 78 patients (33 male) were identified, the median age at diagnosis was 3.4 years (IQR 0.9-6.6 years) and weight 15 kg (IQR 8.9-24.9 kg). Main closure indications were hemodynamically significant shunt, prevention of endocarditis or rupture in an aneurysmal feeder artery, or evidence of myocardial ischemia. Twenty-three patients did not have indications for closure and were managed conservatively, 8 had primary surgical ligation, and the rest (47) underwent trans-catheter closure.
The catheterization procedural details are summarized in the table below. A wide variety of approaches (antegrade v retrograde) and devices (coils, vascular plugs, and nitinol occluders) were utilized. Antegrade approaches were utilized more commonly in the later part of the study period likely due to availability of smaller profile closure devices. Trans-catheter closure was successful in 44/47 patients. The rate of procedural complications was high with 31.9% experiencing complications, and almost half of these (6/15) being serious – CA pseudoaneurysm, myocardial infarction, air embolism, RCA dissection, and aortic valve regurgitation requiring surgical repair. There were no significant long-term complications in any of the treatment groups.
The authors should be congratulated on a robust demonstration of the heterogeneous nature of CAFs and the various management strategies that can be applied. In the modern era transcatheter closure is effective in appropriately selected patients however the rate of serious complication is relatively high (12%) and requires upfront preparation and the availability of surgical back up. Smaller profile devices that can be delivered via microcatheters and 4-French diagnostic catheter may allow for treatment of more complex fistulas in smaller patients.