Biventricular repair for pulmonary atresia with intact ventricular septum associated with sinusoidal communications.

Pulmonary atresia with intact ventricular septum (PA-IVS) is very difficult to treat due to the variety of right ventricular hypoplasia and coronary artery anomalies. Biventricular repair is viewed as possible in patients with mild right ventricular hypoplasia but not in those with severe right ventricular hypoplasia or with sinusoidal communication. We report a case of a 17-day-old boy with PA-IVS and severe right ventricular hypoplasia and large sinusoidal communications. We successfully conducted biventricular repair, ligated coronary artery and right ventricular fistulas, and implemented right ventricular outflow tract reconstruction. He now enjoys good health and attends elementary school. Sinusoidal communications are not always a limiting factor in biventricular repair for PA-IVS if the right ventricle can be decompressed without inviting ventricular dysfunction by ligating fistulas.