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The long-term outcome of an isolated vascular ring - A single-center experience
Anouk Depypere1, Marijke Proesmans1, Björn Cools2
1Department of Pediatrics, Pediatric Pulmonology, University Hospital Leuven, Leuven, Belgium.
Insights
Long-term outcomes for congenital vascular ring patients show surgery is safe, but many retain symptoms like stridor. Conservative management may suit less symptomatic individuals.
Area of Science:
- Pediatric Surgery
- Cardiovascular Surgery
- Medical Diagnostics
Background:
- Congenital vascular rings are rare anomalies affecting the great arteries.
- Long-term outcomes and symptom resolution following treatment are not well-documented.
Purpose of the Study:
- To document the long-term clinical outcomes of patients diagnosed with a complete vascular ring.
- To evaluate the effectiveness of surgical and conservative management strategies.
Main Methods:
- Retrospective review of 51 patients diagnosed with complete vascular rings between 1980-2013.
- Data collected included age at diagnosis, diagnostic tools, surgical procedures, complications, and long-term symptoms via parental questionnaires.
- Follow-up duration was at least 2 years, with a median of 8 years.
Main Results:
- Surgery was performed in 41 patients, primarily for double aortic arch, with few reversible complications.
- After 2 years, 21 patients were asymptomatic, while 30 continued to experience symptoms, with varying degrees of improvement or worsening.
- After 10 years, 26/36 patients were symptom-free, with stridor being the most frequent residual symptom.
Conclusions:
- Surgical treatment for congenital vascular rings, especially double aortic arches, is safe and effective.
- Conservative management may be appropriate for patients with minimal symptoms.
- A significant proportion of patients experience persistent long-term symptoms even after surgical intervention.
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.
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