What ongoing care do children need after surgery for a pulmonary artery sling with severe airway narrowing?

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A left pulmonary artery (LPA) sling (PAS) is a vascular ring frequently associated with long-segment tracheal stenosis (TS). Mortality among operated children remains high, especially with severe tracheal hypoplasia and/or associated congenital heart defects (CHDs). This descriptive pediatric cohort reported treatment and follow-up experience in children with PAS and severe tracheobronchial involvement.

From 2005 to 2017, the study enrolled 11 children with PAS and congenital TS requiring surgery. All underwent echocardiography, computed tomography, and bronchoscopy. Associated CHDs were present in 5 children (45%).

Tracheal reconstruction used slide tracheoplasty in 7/11 children (63%), slide tracheoplasty combined with a costal cartilage graft in 2/11 (18%), and the Hazekamp technique in 2/11 (18%). Nine children underwent direct LPA reimplantation together with tracheoplasty. Three children also underwent concomitant surgical repair of CHDs.

During a mean follow-up of 30 months (range: 3–75 months), late mortality was 18%; there were no early deaths. Good blood flow through the LPA was documented in all patients. Ten children required interventional bronchoscopies, averaging 16 per patient, for stent placement or removal, treatment of granulomas, and tracheobronchial dilation.

The authors identified severe tracheobronchial stenosis and associated CHDs as the main determinants of hospitalization duration, intensive support, and repeated endoscopic procedures. They concluded that children with the PAS/TS complex require careful management at highly specialized centers with multidisciplinary teams. Respiratory endoscopy may play a central role both in preoperative assessment and in postoperative management when tracheobronchial involvement is severe.