Airway compression by major aortopulmonary collaterals with 22q11 deletion.
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.
Kaneko
Yukihiro
Y
Deptartment of Cardiovascular Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo 150-8935, Japan. yukihirokaneko@hotmail.com
Yoda
Hitoshi
H
Tsuchiya
Keiji
K
D000015:Abnormalities, Multiple; D000402:Airway Obstruction / Q000209:etiology* / Q000235:genetics*; D001011:Aorta / Q000002:abnormalities; D004062:DiGeorge Syndrome / Q000150:complications* / Q000235:genetics; D004200:Diseases in Twins; D006330:Heart Defects, Congenital / Q000235:genetics / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009389:Neovascularization, Pathologic / Q000150:complications* / Q000235:genetics; D011651:Pulmonary Artery / Q000002:abnormalities; D013348:Subclavian Artery / Q000002:abnormalities