Closure of coronary artery fistula in childhood: treatment techniques and long-term follow-up.

INTRODUCTION: Coronary artery fistula (CAF) is a rare congenital anomalous connection between the coronary arteries (CA) and a cardiac chamber or great vessel. Treatment options of symptomatic CAF consist of transcatheter or surgical closure.

METHODS: Retrospective analysis of all patients with CAF diagnosed between 1993 and 2014 concerning treatment approaches and follow-up after closure.

RESULTS: In a cohort of more than 25000 patients, 194 (<0.01 %) were diagnosed to have CAF. Median age at diagnosis was 6 months (0 day-18 years). Treatment was indicated in ten patients (5.2 %). Six patients (60 %) were treated by catheter interventional approach using Coils (three patients), Amplatzer Vascular Plugs (two patients) and Amplatzer Duct Occluder (one patient). One of these patients showed a significant residual shunt through the fistula 5 days after interventional closure, necessitating surgical removal of the device and closure of CAF. At a median follow-up of 7 (range 2-12) years, the remaining five patients showed successfully closed CAF without causing thrombosis of the CA. Control angiography in three patients showed persistent dilated CA. Surgical closure of CAF was performed in four (40 %) patients; in two as an isolated procedure and in the remaining two as a part of another congenital cardiac corrective procedure.

CONCLUSIONS: CAF in paediatric cardiology patients is a very rare finding. Intervention in childhood is rarely needed; nevertheless, it is known that small fistulas may become relevant in adulthood. Transcatheter closure techniques are effective and are considered the treatment of choice, especially in isolated CAF.