Tracheal development after left pulmonary artery reimplantation: an individual study

Xiaoyang Hong,Qin Yu,Gengxu Zhou,Zhe Zhao,Gang Wang,Zhichun Feng,Hui Wang,Jun Li,Jiangheng Guan,Xiaohong Liu,Ruijie Li

doi:10.1038/s41598-020-74890-4

Abstract

Pulmonary artery sling (PA sling) often presents as a life-threatening condition requiring urgent surgical correction. We reported 32 cases of PA sling in children who were followed up postoperatively in the past 6 years. All patients with PA slings who were admitted to the hospital from January 2012 to December 2017 and underwent surgery were retrospectively analyzed. The mean age of the 32 patients at repair was 16.97 months (range, 15 days to 128 months). Six patients required ventilator assistance for respiratory failure. All children underwent left pulmonary artery (LPA) reimplantation (n = 32), and 3 patients needed reimplantation slide tracheoplasty (n = 3) due to ventilation weaning failure. Four patients died, 27 patients survived until discharge, and 18 patients were followed up. Pulmonary computed tomography imaging and echocardiography were performed in 18 patients who were followed up. After LPA reimplantation, the tracheal carina area was significantly enlarged compared to that preoperation (p = 0.0002). In this follow-up cohort study, 75% of the patients who underwent LPA reimplantation survived until discharge. The survivors had subsequently well-developed pulmonary arteries and tracheas.

Highlights

Pulmonary artery sling (PA sling) often presents as a life-threatening condition requiring urgent surgical correction
Left pulmonary sling (PA sling) was first reported in 1897, and it is still a rare congenital cardiovascular abnormality[1,2]. It is characterized by a left pulmonary artery (LPA) from the right pulmonary artery (RPA) and flow between the trachea and esophagus to the left lung hilum; ~ 1/2–2/3 of PA sling patients were found to have complete tracheal rings, known as "sling complexes"[1,2]
For PA sling children with tracheal stenosis, it is still unclear whether left pulmonary artery transplantation alone can promote further growth and development of the stenosed trachea

Summary

Introduction

Pulmonary artery sling (PA sling) often presents as a life-threatening condition requiring urgent surgical correction. All children underwent left pulmonary artery (LPA) reimplantation (n = 32), and 3 patients needed reimplantation slide tracheoplasty (n = 3) due to ventilation weaning failure. After LPA reimplantation, the tracheal carina area was significantly enlarged compared to that preoperation (p = 0.0002) In this follow-up cohort study, 75% of the patients who underwent LPA reimplantation survived until discharge. Kwak et al performed left pulmonary artery transplantation alone in 14 PA sling children with tracheal stenosis[12]. For PA sling children with tracheal stenosis, it is still unclear whether left pulmonary artery transplantation alone can promote further growth and development of the stenosed trachea. We report our experience with 32 patients with PA sling who underwent left pulmonary artery reimplantation from 2012 to 2017. A comparison of various developmental indicators among children of the same age was carried out

Methods

Results

Conclusion