Purpose or Case Report: To describe CTA findings of children with left pulmonary artery sling (LPA) using the classification proposed by Wells, et al and report associated anomalies and surgical outcomes.
Methods & Materials: In a multicentric study from 2013 to 2019, we retrospectively identified 11 patients with a left pulmonary artery sling referred for CT angiography. Demographics, clinical data and outcomes were collected from their medical records. CTA findings were collected by two pediatric radiologists. In all cases, 3D volume-rendered reconstructions plotting the airway and central vessels were obtained.
Results: of the 11 patients (median age: 6 months, range: 1-44 months; 6 girls:5 boys), 9 (81.8%) were symptomatic, with shortness of breath and/or stridor at presentation.
There were seven patients with type 1 (type 1A: n=5, type 1B: n=2) and 4 patients with type 2 (type 2A: n=3, type 2B: n=1). Seven patients had associated cardiovascular anomalies, including ventricular and atrial septal defects (n=3, each), persistent ductus arteriosus and persistent left superior vena cava (n =2, each), and also one case each of partial anomalous pulmonary venous return, pulmonary stenosis, total anomalous pulmonary venous return and foramen oval permeable respectively. The proximal branches of the right pulmonary artery (RPA) had its origin 10 of the 11 cases on the RPA, only one patient showed an anomalous origin of the right middle pulmonary artery from a common orifice between the RPA and LPA. The LPA reanastomosis was performed in 8 patients, including two patients with accompanying tracheoplasties. 2 patients of this series died before surgical correction.
Conclusions: Left pulmonary artery sling is an uncommon vascular ring and CT angiography is an accurate method for demonstrating it and its associated cardiovascular anomalies for an optimal presurgical evaluation of LPAS.