Airway Compression After Unifocalization in Pulmonary Atresia and Aortopulmonary Collateral Arteries

We hypothesized that reconstructed pulmonary artery (PA) size and postrepair PA pressure are associated with airway compression (AC) after complete unifocalization for pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries.

Complete unifocalization was performed in 48 consecutive patients between 2000 and 2016. Clinical course and outcome were reviewed, predictors for AC were identified by logistic regression, and the freedom from death was analyzed using Kaplan-Meier method.

Postoperative respiratory distress occurred in 23 patients (48%), and AC occurred in 14 (29%). The median duration of follow-up was 3.7 years. AC was caused by central PA and aorta in 7, conduit in 3, and branch PA in 4. Surgical treatment was required in 5 patients (conduit downsizing, suspension of branch PA, conduit + aorta, branch PA + aorta, and aorta + trachea in 1 patient each). Three patients (21%) subsequently required airway stenting. Most (85.7%) of the AC occurred in patients with high right ventricular systolic pressure/left ventricular systolic pressure (>65%), large Nakata index (>200 mm²/m²), and large conduit index (>35 mm/m²). Patients with AC had significantly worse 3-year survival (no AC, 91.2%; AC, 64.2%; p = 0.01). Multivariate analysis identified higher right ventricular systolic pressure/left ventricular systolic pressure (p = 0.04), larger conduit index (p = 0.03), and Nakata index (p = 0.004) as predictors for AC.

AC is a common cause of postoperative respiratory distress and tends to be associated with higher postrepair PA pressure, more frequent right ventricular dysfunction, and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor major aortopulmonary collateral arteries anatomy and physiology remains a challenge.