Airway complications after single-stage unifocalization for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries

Objective We analyze the incidence of postoperative severe airflow limitation after single-stage unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) and comment on the treatment performed. Methods From 1994 until 2014, 118 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four patients (3.4%) developed severe airflow complications postoperatively. Chromosome 22q11 deletion was present in three of them. Median age at the time of unifocalization was 6.2 months (range 21 days to 11 months). Results The first patient developed malacia and compression of the left bronchus from the distal RV-PA conduit and was treated with external bronchial stenting with two incomplete costal cartilage rings. The second patient developed recurrent esophagus-left bronchus fistula treated with multiple surgical esophageal and bronchus reconstructions. The third child presented with bilateral bronchial malacia treated with bilateral stenting followed by surgical elongation of the neo-left pulmonary artery to avoid external compression. The last patient developed bilateral bronchomalacia treated with bilateral bronchial stenting followed by RV-PA conduit replacement and endobronchial stenting calibration. Conclusion Particular categories of patients with PA, VSD, MAPCAs (22q11 chromosome deletion, neonates/infants, patients with dominant/exclusive collaterals) may be more predisposed to develop airway compromise. The treatment of the lesion should be individualized according to the pathogenic mechanism. We suggest endoluminal treatment in absence of compression by vascular structures while surgery was used in case of extrinsic compression.