BACKGROUND AND AIM OF THE STUDY: Right pulmonary artery to left atrium fistula is a very rare cardiac malformation. We sought to describe our experience with surgical and transcatheter treatment of this defect in three cases. MATERIAL AND METHODS: Between October 2000 and October 2009, three patients with right pulmonary artery to left atrial fistula were treated at our institute. They were all males and aged 23, 7, and 14, respectively. They presented with cyanosis with clubbing of fingers and toes in three cases, and exertional dyspnea in two. Laboratory tests showed elevated hemoglobin and decreased systemic arterial oxygen saturation. Preoperative diagnosis was made by echocardiography and cardioangiography. The fistula was closed surgically in two patients either by simple ligation without cardiopulmonary bypass or by suture of the fistula from inside the right pulmonary artery lumen under cardiopulmonary bypass. In the third patient, the fistula was occluded with an 18-mm Amplatzer septal occluder (AGA Medical, Plymouth, MN, USA). RESULTS: The systemic oxygen saturation rose up to above 96% immediately after the procedures. No operative or procedural complication and late deaths occurred. All three patients were asymptomatic on follow-up. CONCLUSIONS: Both surgical and transcatheter closure are safe and reliable treatment for patients with right pulmonary artery to left atrial fistula. The treatment should be individualized and performed as soon as the diagnosis is made.
BACKGROUND AND AIM OF THE STUDY: Right pulmonary artery to left atrium fistula is a very rare cardiac malformation. We sought to describe our experience with surgical and transcatheter treatment of this defect in three cases. MATERIAL AND METHODS: Between October 2000 and October 2009, three patients with right pulmonary artery to left atrial fistula were treated at our institute. They were all males and aged 23, 7, and 14, respectively. They presented with cyanosis with clubbing of fingers and toes in three cases, and exertional dyspnea in two. Laboratory tests showed elevated hemoglobin and decreased systemic arterial oxygen saturation. Preoperative diagnosis was made by echocardiography and cardioangiography. The fistula was closed surgically in two patients either by simple ligation without cardiopulmonary bypass or by suture of the fistula from inside the right pulmonary artery lumen under cardiopulmonary bypass. In the third patient, the fistula was occluded with an 18-mm Amplatzer septal occluder (AGA Medical, Plymouth, MN, USA). RESULTS: The systemic oxygen saturation rose up to above 96% immediately after the procedures. No operative or procedural complication and late deaths occurred. All three patients were asymptomatic on follow-up. CONCLUSIONS: Both surgical and transcatheter closure are safe and reliable treatment for patients with right pulmonary artery to left atrial fistula. The treatment should be individualized and performed as soon as the diagnosis is made.
Authors: Dominik C Benz; Barbara Burkhardt; Daniel Quandt; Dominik Stambach; Walter Knirsch; Oliver Kretschmar Journal: Eur J Pediatr Date: 2014-05-21 Impact factor: 3.183