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Airway Compression by Major Aortopulmonary Collaterals with 22q11 Deletion

Department of Cardiovascular Surgery
¹ Department of Neonatology
² Department of Pediatrics, Japanese Red Cross Medical Center, Tokyo, Japan

For reprint information contact: Yukihiro Kaneko, MD Tel: 81 3 3400 1311 Fax: 81 3 3409 1604 Email: yukihirokaneko{at}hotmail.com, Deptartment of Cardiovascular Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935, Japan.

Hypoxic choking episodes due to airway obstruction occurred frequently from 4 months of age in a boy with 22q11 deletion, pulmonary atresia, ventricular septal defect, absent central pulmonary artery, tracheobronchomalacia, and an aberrant right tracheal bronchus. The tracheobronchial tree was compressed by a posteriorly displaced ascending aorta and right aortic arch with aberrant left subclavian artery and major aortopulmonary collateral arteries. Single-stage unifocalization and intracardiac repair plus aortopexy at 8 months resulted in resolution of the respiratory distress and heart failure.