Midterm outcomes of pulmonary artery sling repair with and without tracheoplasty

Objective: Review a single-centre experience with pulmonary artery sling repair and evaluate risk factors for re-intervention. Methods: Patients with surgically repaired pulmonary artery sling at a single institution between 1996 and 2018 were retrospectively reviewed. A univariate Cox regression analysis was used to evaluate variables for association with freedom from re-intervention. Results: Eighteen patients had pulmonary artery sling repair. At operation, median age and weight were 6.9 months (interquartile range 4.1-18.1) and 9.5 kg (interquartile range 6.5-14.5), respectively. A median hospital length of stay was 12 days (interquartile range 5.8-55.3). Twelve patients (67%) had complete tracheal rings, of whom six (50%) underwent tracheoplasty (five concurrently with pulmonary artery sling repair). Airway re-intervention was required in five (83%) of the six patients who underwent tracheoplasty. One patient had intraoperative diagnosis and repair of pulmonary artery sling during unrelated lesion repair and required tracheoplasty 24 days post-operatively. One patient died 55 days after pulmonary artery sling repair and tracheoplasty following multiple arrests and re-interventions. Median post-operative follow-up for surviving patients was 6.3 years (interquartile range 11 months-13 years), at which time freedom from re-intervention was 61%. When controlling for patient and tracheal size, initial tracheoplasty was associated with decreased freedom from re-intervention (hazard ratio 21.9, 95% confidence interval 1.7-284.3, p = 0.018). Conclusions: In patients with pulmonary artery sling, tracheoplasty is associated with decreased freedom from re-intervention. In select patients with pulmonary artery sling and complete tracheal rings, conservative management without tracheoplasty is feasible. Further study is necessary to delineate objective indications for tracheoplasty.