Anomalous origin of the right pulmonary artery from the aorta and CATCH 22 syndrome.

We report repair of anomalous origin of the right pulmonary artery from the ascending aorta in a premature neonate with a deletion in the CATCH 22 region of chromosome 22. This case suggests that the pathogenesis of anomalous origin of the right pulmonary artery involves genetically determined abnormalities of the neural crest. Repair of this defect in a premature infant can prevent the development of severe pulmonary vascular disease.