Anomalous origin of the right pulmonary artery from the ascending aorta: results of direct implantation surgical repair in 6 infants

BACKGROUND: Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is a rare and potential fatal kind of congenital heart disease. This study summarizes the techniques and outcomes of 6 infants with AORPA who underwent the surgical repair. METHODS: Between November 2012 and November 2014, 6 infants with AORPA received surgical repair in the Second Xiangya Hospital and were included in the present study. RESULTS: Six infants (4 male, 66.7 %) with a median age of 101.5 ± 70.0 days, and a median body weight of 4.13 ± 0.62 kg underwent the surgical repair at our institute. There were no operative, in-hospital or follow-up deaths. Clinical symptoms of all 6 patients relieved at time of discharge, and mean pulmonary artery pressure (MPAP) decreased significantly after surgery. During follow-up, there were no further operations or interventions, mild stenosis at the anastomotic site presented in one patient, and all patients were asymptomatic and in stable clinical condition. CONCLUSIONS: The short and mid-term surgical outcomes of AORPA are excellent in this group of operations. Moreover, we believe the direct implantation to be the optimal surgical strategy for the patients with the proximal form of AORPA.