Abstract
Background Left pulmonary artery (LPA) sling (PAS) is a vascular ring, which is frequently associated
with long-segment tracheal stenosis (TS). Mortality rate in operated children is still
high, especially in cases of severe tracheal hypoplasia and/or associated congenital
heart defects (CHDs). We report our experience of treatment and follow-up in a pediatric
cohort of patients affected by PAS with severe tracheobronchial involvement.
Methods From 2005 to 2017, we enrolled 11 children diagnosed with PAS and congenital TS requiring
surgical intervention. Echocardiography, computed tomography, and bronchoscopy were
performed in all patients. Associated CHD were present in 5 (45%) patients. Tracheal
reconstruction techniques included slide tracheoplasty (7/11; 63%), slide tracheoplasty
and costal cartilage graft (2/11; 18%), and Hazekamp technique (2/11; 18%).
Nine patients underwent LPA direct reimplantation and concomitant tracheoplasty; concomitant
surgical repair for CHD was performed in three children.
Results Over a mean follow-up of 30 months (range: 3–75 months), a late mortality of 18%
was registered; no early death occurred. Good flow through LPA could be documented
in all patients. Ten children required operative bronchoscopies (mean: 16/patients)
aimed at stent positioning/removal, treatment of granulomas, and tracheobronchial
dilatation.
Conclusions Severe tracheobronchial stenosis and associated CHD were the main determinants for
hospitalization time, intensive assistance, and repeated endoscopic procedures.
Patients affected by PAS/TS complex require a careful management at high-specialized
centers providing multidisciplinary team.
Respiratory endoscopy may play a central role both in preoperatory assessment and
in postoperative management of patients showing severe tracheobronchial involvement.
Keywords
congenital heart disease - CHD - pulmonary arteries - trachea
