The long‐term outcome of an isolated vascular ring – A single‐center experience

Abstract

Background and Objectives The aim of this study is to document the long‐term outcome of patients with a vascular ring. Methods A single‐center retrospective review of clinical symptoms was conducted in all patients born between 1980 and 2013, diagnosed with a complete vascular ring and at least 2 years of follow‐up. Data were extracted from patient files and clinical symptoms were assessed by questionnaires sent to the parents. Age and diagnostic tools, type of surgery, postoperative complications, and the prevalence of clinical symptoms (stridor at rest, stridor with exercise, cough, exercise intolerance, dysphagia, and frequent respiratory infections) were reviewed. Results Fifty‐one patients were included. The diagnosis was made before the age of 2 in 35/51. Surgery was performed in 41/51 patients with a limited number of reversible complications. The median follow‐up was 8 (IQR 5‐12) years. After 2 years, 21/51 patients were asymptomatic: 2/7 asymptomatic patients at diagnosis became symptomatic and symptoms resolved in 16/44 initially symptomatic patients. Consequently, 30/51 patients still had symptoms after 2 years. Of these, 16 had partial improvement, 5 stable symptoms, and 9 aggravation of symptoms. After 10 years, 26/36 patients were free of complaints. The most frequent long‐term symptom was stridor. Conclusion Surgical treatment of a congenital vascular ring is safe and mostly performed in patients with a double aortic arch. Conservative treatment for patients with little symptoms seems to be justifiable. A considerable number of patients have residual long‐term symptoms after surgical relief of the vascular ring.

Functional capacity and quality of life in patients with vascular ring

“…Nine patients complained of persistent respiratory symptoms, mostly cough, dyspnea, and/or wheezing with abnormal spirometry and/or lung volumes in seven subjects. Depypere et al 7 proposed that residual symptoms could be a risk for the development of exercise‐induced wheezing. In our study, none of the patients had postexercise bronchoconstriction.…”

“…The operation is considered safe with low rates of complications 4 and clinical improvement appears in the vast majority of children. [5][6][7] Clinical follow-up (ranging from 1 year to 34 years) showed that 16%−68% of patients were symptom-free. 4,8 Double aortic arch patients were found to have an increased risk of remaining symptomatic 8,9 with lessened improvement with associated tracheomalacia.…”

Aortic arch anomalies, embryology and their relevance in neuro-interventional surgery and stroke: A review

“…Due to the sporadic nature of this anomaly, there is not enough data on the long-term prognosis or possible complications during endovascular procedures. 58…”

Pediatric pulmonology 2019 year in review: rare and diffuse lung disease

“…Depypere et al 19 reported single-center experience of 51 children with an isolated vascular ring with a median follow-up of 8 years.…”

“…As with CLM, these lesions are being detected prenatally with increasing frequency, raising the question of when and whether to intervene surgically in asymptomatic or mildly symptomatic cases. Depypere et al 19 reported single‐center experience of 51 children with an isolated vascular ring with a median follow‐up of 8 years. Most were diagnosed before 2 years of age and all patients with double aortic arch were symptomatic on presentation.…”