Long-term outcomes in children undergoing vascular ring division: a multi-institution experience

Abstract

Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division. A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed. The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up. The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.