Fistula between the right pulmonary artery and the left atrium—an unusual cause of cyanotic heart disease

A fistula from the pulmonary artery to the left atrium is a very rare congenital anomaly. The communication, almost exclusively arising from the right pulmonary artery, may give rise to a variety of symptoms with cyanosis as the predominant sign. To our knowledge only seven neonates have been reported with this anomaly, and five of them died postoperatively. We present another two neonates with such a fistula which was closed by simple ligation using a transsternal approach and cardiopulmonary bypass. Both patients had an uneventful postoperative course and are alive and well at the age of two and three years, respectively.