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Ann Thorac Surg 1995;60:681-683
© 1995 The Society of Thoracic Surgeons

Articles

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

MD Mark C. Johnson*, PhD Michael S. Watson, MD Arnold W. Strauss, MD Thomas L. Spray

Division of Cardiology, Edward Mallinckrodt Department of Pediatrics, Division of Cardiothoracic Surgery, Department of Surgery, and James S. McDonnell Department of Genetics, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, Missouri, USA

Accepted for publication February 14, 1995.

* Address reprint requests to Dr Johnson, Division of Pediatric Cardiology, St. Louis Children's Hospital, One Children's Place, St. Louis, MO 63110.

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.




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