Objective: Anomalies of the aortic arch (vascular rings) are uncommon anomalies in which preferred strategies for diagnosis and treatment may vary among institutions. In this study, we report a description of our approach and review of our 38-year experience in patients surgically treated for vascular rings.
Methods: A retrospective review was conducted of all patients with/without symptomatic tracheoesophageal compression secondary to anomalies of the aortic arch and great vessels diagnosed from 1970 to 2008. A total of 183 patients underwent surgical repair. Median age at the time of the operation was 5 months (range, 3 days to 30 years). Patients were classified into 5 major subtypes based on their surgical anatomy as follows: right aortic arch-left ligamentum (n = 77), double aortic arch (n = 67), aberrant (retroesophageal) right subclavian artery (n = 30), pulmonary sling (n = 8), and innominate artery compression (n = 1). Six patients (3%) had an associated Kommerell diverticulum. In patients with a double aortic arch, 82% had a dominant right arch and 18% had a dominant left arch. Preoperatively, 80 patients (44%) had stridor, and 86 patients (47%) had recurrent upper respiratory tract infection.
Results: Associated cardiac diagnosis were present in 54 (30%) of 183 of all patients with vascular rings. Left thoracotomy was a common operative approach in all patients except pulmonary artery sling patients where a median sternotomy was the preferred approach. There were 3 early and 5 late deaths (all patients had complex cardiac anomalies) with a median follow-up of 6 years. Overall survival was 96% at 35 years. Postoperative complication occurred in 3 patients (2%) as follows: tracheostomy because of severe distal tracheal compression (n = 2) and left true vocal cord paralysis (n = 1). None of the patients showed any evidence of recurrent vascular ring anomalies at last follow-up. Of the children, 75% (135/180) were free from compressive symptoms within 1 year of the operation.
Conclusions: Vascular anomalies with/without tracheoesophageal compression present symptomatically in a variety of ways, and noninvasive methods are used to identify the specific lesion and associated cardiac defects. Surgical repair is associated with low or no mortality in patients with uncomplicated complex of vascular anomalies.