Objective: Anomalous origin of one pulmonary artery from the aorta (hemitruncus) is a rare congenital lesion with a high mortality and morbidity if early diagnosis is not made and correction is not undertaken. In this study, we report a description of our approach and review of our 25-year experience in patients surgically treated for hemitruncus.

Methods: Between 1983 and 2008, fourteen patients (neonates – 50%;7/14) were operated on for hemitruncus. Median age at presentation was 26 days (range, 5 days to 8 months). Five patients (36%) had associated cardiac lesions. All patients presented with congestive cardiac failure. Diagnosis was established by echocardiography in all patients. Eleven of the 14 had anomalous right pulmonary artery (RPA) from aorta, and 3 had left pulmonary artery from aorta.

Results: There was no operative or late mortality. Follow-up period ranged from 6 months to 20 years (median 15 years). Two patients required reoperations: for thrombosis of anastomosis when RPA was very small (n=1) and for RPA stenosis (n=1) 1 and 2 years after surgery, respectively. At 20 years, survival by Kaplan-Meier was 100%, and freedom from reoperation, 86%. All patients except 1 has normal hemodinamic function and were in NYHA class I.

Conclusions: Early repair of hemitruncus results in excellent hemodinamic and anatomic results. Survival is excellent, with a low incidence of reoperation or reintervention.