Anomalous origin of the pulmonary arteries from the pulmonary trunk is frequently associated with genetic syndromes, such as Alagille syndrome (ALGS). Despite being uncommon, these anomalies may be underestimated according to a new study published in the World Journal for Pediatric and Congenital Heart Surgery.

“These positional anomalies may not be easily identified on routine echocardiographic studies and a high index of suspicion is needed to detect its presence which may be confirmed on cross-sectional imaging to facilitate successful operative/transcatheter planning,” the study’s authors said.

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Three patients had isolated atrial and/or ventricular septal defects and 11 patients had septal defects associated with other anomalies. Seventeen patients had aortic arch anomalies, including arch hypoplasia with or without coarctation (n = 9) and complete vascular rings with aberrant retroesophageal left subclavian artery (n = 4). Moreover, 8 patients had right sided aortic arch and 4 patients had mirror-image branching mirror-image branching. Ten patients showed a variety of extracardiac anomalies.

All patients underwent surgical interventions to manage their cardiac defects. In addition, 4 patients required pulmonary artery branch and/or right ventricular outflow tract interventions prior to surgery.

Patients with ALGS underwent concomitant pulmonary arterioplasties and pulmonary arterial branch rehabilitation and had their pulmonary arteries uncrossed during the reconstruction process. No other patient had underwent interventions on the crossed pulmonary arteries.

There were no early or late mortalities. One patient with ALGS underwent liver transplant prior to discharge and 4 patients needed late transcatheter interventions, and 4 patients required repeat aortic arch augmentation for recurrent arch obstruction.

The study enrolled 24 patients (median age, 3 months, age range, 5 days to 29 years). Half of patients had genetic syndromes, including 4 patients with ALGS.

Reference

Said SM, Hoggard E, Narasimhan S. Surgical experience with crossed and anomalous origin of the pulmonary arteries from the pulmonary trunk: a single center report of 24 cases. World J Pediatr Congenit Hear Surg. Published online December 13, 2022. doi:10.1177/21501351221139833