We reviewed our surgical experience with anomalous origin of one pulmonary artery from the ascending aorta (AOPA). From 1989 to 2012, 12 children (five neonates) aged 3-734 days (mean 152 ± 222) with AOPA underwent operations. Eight patients had right AOPA, and four patients had left AOPA. The majority of the patients had elevated right ventricular pressure, with 58 % (7 of 12) demonstrating suprasystemic right ventricular pressure. Surgery was performed by direct anastomosis (group 1) in seven patients and by employing an autologous patch (group 2) in five patients. There were two postoperative mortalities caused by heart failure and pulmonary hypertensive crisis. The mean follow-up duration was 12.6 ± 8 years. Catheterization showed that the right ventricle-to-systemic pressure ratio decreased following operation (preoperative vs. postoperative; 1.13 ± 0.19 vs. 0.48 ± 0.03, p = 0.043). There was no difference in the perfusion of the affected lung as measured by the final lung perfusion scan, between the two groups (group 1 vs. group 2; 50.0 ± 10.3 vs. 42.7 ± 28.7 %, p = 0.158). Two patients required reoperations for pulmonary regurgitation and pulmonary artery stenosis. There were two catheter-based interventions. At 20 years, survival by the Kaplan-Meier was 91.7 ± 8.0 %, freedom from reoperation was 80.0 ± 17.9 %, and freedom from catheter intervention was 80.8 ± 12.2 %. Early repair of AOPA improves right ventricular pressure and overall hemodynamics with excellent survival and low risk of reintervention. The type of surgical repair did not significantly affect the long-term outcomes (measured via lung perfusion scan).
Keywords: Aorta; Great vessel anomalies; Hemitruncus; Pulmonary arteries.