A rare cause of wheezing in a child with pulmonary atresia

The determination of the exact cause for symptomatic airway obstruction in pediatric patients not responding to medication can be a clinical dilemma. Very rarely external vascular compressions can produce airway obstruction symptoms unresponsive to usual bronchodilator medications. The successful management of a child with pulmonary atresia and an innominate artery compression syndrome with respiratory compromise due to tracheal compression is described.