OBJECTIVE: A direct communication between the pulmonary artery and the left atrium is a rare anomaly. On the basis of two cases of our own and a literature review of 49 cases, we focus on clinical presentation, anatomy, diagnosis, and the role of surgery. METHODS: Two cases of a fistula between the right pulmonary artery and the left atrium are described in a girl of 4 years and a boy of 15 years. Both presented with unexplained cyanosis. Diagnosis was made on echocardiography and angiography. The fistula was ligated using extracorporeal circulation in the first case and not in the second case. RESULTS: The surgical results were successful with resolution of the cyanosis. CONCLUSIONS: In newborns, urgent surgery may be necessary. In other patients, early elective surgical correction should be performed to prevent complications, especially systemic and cerebral emboli, cerebral abscesses, and rupture of aneurysmal fistulas. Complete cure can be achieved by ligation and possible division or by intracardiac repair.
OBJECTIVE: A direct communication between the pulmonary artery and the left atrium is a rare anomaly. On the basis of two cases of our own and a literature review of 49 cases, we focus on clinical presentation, anatomy, diagnosis, and the role of surgery. METHODS: Two cases of a fistula between the right pulmonary artery and the left atrium are described in a girl of 4 years and a boy of 15 years. Both presented with unexplained cyanosis. Diagnosis was made on echocardiography and angiography. The fistula was ligated using extracorporeal circulation in the first case and not in the second case. RESULTS: The surgical results were successful with resolution of the cyanosis. CONCLUSIONS: In newborns, urgent surgery may be necessary. In other patients, early elective surgical correction should be performed to prevent complications, especially systemic and cerebral emboli, cerebral abscesses, and rupture of aneurysmal fistulas. Complete cure can be achieved by ligation and possible division or by intracardiac repair.