Airway Complications after Single-Stage Unifocalization for Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries

Abstract

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 2012, 106 patients with diagnosis of PA, VSD, MAPCAs underwent surgical treatment. Four of them (3.8%) developed in the postoperative course severe airflow complication. Chromosome 22q11 deletion was present in three of them. Median age at the time of unifocalization was 6.2 months (range 21 days – 11 months). Results: The first developed malacia and compression of the left bronchus from the distal RV-PA conduit 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 reconstruction. The third child presented bilateral bronchial malacia treated with bilateral stenting and after with surgical elongation of the neo-left pulmonary artery to avoid external compression. The last developed bilateral bronchomalacia treated with bilateral bronchial stenting and after with RV-PA conduit replacement and endobronchial stenting calibration Conclusion: Particular categories of patients (22q11 chromosome deletion, neonates/infants, patients with dominant/exclusive collaterals,) may be more predisposed to develop airway limitation. The treatment of the lesion should be individualized according to the supposed pathogenic mechanism. We suggest endoluminal treatment in absence of compression by vascular structures while surgery was used in proven case of extrinsic compression.