Abstract
Background
Anomalies of the pulmonary origin of the pulmonary arteries are uncommon; however, the true incidence is unknown and may be underestimated. We sought to review our experience with this unusual pathology and examine its surgical implications.
Methods
We reviewed medical records between 2011 and 2022.
Results
A total of 24 patients were identified. Genetic syndromes were present in 12 patients (50%). The patients were divided into two main groups. Those with septal defects (14 patients,58.3%), and those without (10 patients, 41.7%). Aortic arch hypoplasia with/without coarctation was present in nine patients (37.5%). Pulmonary arterial branch hypoplasia and/or obstruction was present in six patients (25%) with resultant preoperative systemic or suprasystemic right ventricular pressure. Four patients (16.7%) underwent pulmonary artery branch and/or right ventricular outflow tract interventions prior to surgery. All patients underwent surgical interventions for their associated cardiac defects. The crossed pulmonary arteries were uncrossed for those four patients (16.7%) who required concomitant pulmonary arterioplasties and pulmonary arterial branch rehabilitation and who had elevated right ventricular pressures preoperatively. No early or late mortalities. One Alagille's syndrome underwent liver transplant prior to discharge. Late transcatheter interventions were needed in four (16.7%) during the follow-up period, while four patients required repeat aortic arch augmentation for recurrent arch obstruction.
Conclusions
Anomalous origin of the pulmonary arteries from the pulmonary trunk including crossed pulmonary arteries is an uncommon anomaly that may be underrecognized. It can occur in isolation or in association with other heart defects. Recognition of this malpositional anomaly is important as it has specific surgical and/or transcatheter implications.
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