Concomitant management of airway and its adjacent vascular pathology in addition to repair of congenital cardiac defects

Respiratory symptoms are often present in infants with congenital cardiac anomalies, but the intrinsic pathology of the airway itself or external compression by abnormal vessels is frequently undetected before cardiac repair. We collected 12 patients with airway pathology from July 1996 to October 1998; all had definite diagnosis of lesions of the airway and its adjacent vessels by preoperative ultrafast computed tomography. Four had intrinsic pathology (one retrotracheal diverticulum, three tracheal stenosis) as well as external compression. Among them, six had complete vascular ring, four partial rings and three had bronchial compression by aneurysmal dilatation of branch pulmonary arteries. Simultaneous airway repair (one diverticulectomy, three patch tracheoplasty) and external decompression were performed in ten cases under cardiopulmonary bypass, nine of ten had simultaneous cardiac repair; the other two were done before cardiac repair. All survived except three. We concluded that ultrafast computed tomography is indispensable for definite diagnosis of airway and adjacent cardiovascular pathology. Mere repair of congenital cardiac defects without rectification of the airway and its adjacent structures is incompatible with survival.