Transcatheter coil occlusion of coronary artery to right ventricular fistulae in pulmonary atresia with intact ventricular septum.

We describe a young adult with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology who presented with extreme dyspnoea on exertion. Coronary artery to right ventricular fistulae and reversible myocardial ischaemia secondary to a right ventricular steal phenomenon were present. The right coronary artery to right ventricular fistula was completely occluded with coils, by a transcoronary approach, with amelioration of symptoms. However, one coil embolised and lodged uneventfully in the left atrium during the procedure. This case demonstrates that a transcatheter approach may be successfully employed not only in patients with isolated coronary artery fistulae, but also in patients with coexistent complex congenital heart disease.