SynthesisEuropean journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery2024
Long-term surgical outcomes of congenital supravalvular aortic stenosis: a systematic review, meta-analysis and microsimulation study.
Synthesis in European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 16 citations in OpenAlex.
- A Scoping Review of the Literature on Child and/or Adolescent Health Microsimulation Models.Applied health economics and health policy · 2026Review
- Article
- Development and validation of a prediction model for early major adverse cardiovascular events in children undergoing surgical repair of supravalvular aortic stenosis.BMC cardiovascular disorders · 2026Article
- Severe Supravalvular Aortic Stenosis Treated With Double Patch Aortoplasty: A Case Report.Clinical case reports · 2026Article
- Case Report: Diagnostic pitfalls in localized aortic root dissection: two cases and a literature review.Frontiers in cardiovascular medicine · 2026Article
- Article
- Hypertrophic cardiomyopathy occurred after successful surgical correction of supravalvular aortic stenosis: a case report of Williams-Beuren syndrome.Frontiers in pediatrics · 2025Article
- Association between cardiopulmonary bypass duration and early major adverse cardiovascular events after surgical repair of supravalvular aortic stenosis.Frontiers in cardiovascular medicine · 2025Article
- Management of supravalvular aortic stenosis with the Doty technique, in a South American pediatric center.Archivos de cardiologia de Mexico · 2024Article
- Review
- Aortic Valve Embryology, Mechanobiology, and Second Messenger Pathways: Implications for Clinical Practice.Journal of cardiovascular development and disease · 2024Review
- Elastin Regulates Vascular Smooth Muscle Cell Behavior During Development and in Diseases.Pediatrics international : official journal of the Japan Pediatric SocietyReview
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
Abstract
objectivesCongenital supravalvular aortic stenosis (SVAS) is a rare form of congenital outflow tract obstruction and long-term outcomes are scarcely reported. This study aims to provide an overview of outcomes after surgical repair for congenital SVAS.
methodsA systematic review of published literature was conducted, including observational studies reporting long-term clinical outcome (>2 years) after SVAS repair in children or adults considering >20 patients. Early risks, late event rates and time-to-event data were pooled and entered into a microsimulation model to estimate 30-year outcomes. Life expectancy was compared to the age-, sex- and origin-matched general population.
resultsTwenty-three publications were included, encompassing a total of 1472 patients (13 125 patient-years; pooled mean follow-up: 9.0 (6.2) years; median follow-up: 6.3 years). Pooled mean age at surgical repair was 4.7 (5.8) years and the most commonly used surgical technique was the single-patch repair (43.6%). Pooled early mortality was 4.2% (95% confidence interval: 3.2-5.5%) and late mortality was 0.61% (95% CI: 0.45-0.83) per patient-year. Based on microsimulation, over a 30-year time horizon, it was estimated that an average patient with SVAS repair (mean age: 4.7 years) had an observed life expectancy that was 90.7% (95% credible interval: 90.0-91.6%) of expected life expectancy in the matched general population. The microsimulation-based 30-year risk of myocardial infarction was 8.1% (95% credible interval: 7.3-9.9%) and reintervention 31.3% (95% credible interval: 29.6-33.4%), of which 27.2% (95% credible interval: 25.8-29.1) due to repair dysfunction.
conclusionsAfter surgical repair for SVAS, 30-year survival is lower than the matched-general-population survival and the lifetime risk of reintervention is considerable. Therefore, lifelong monitoring of the cardiovascular system and in particular residual stenosis and coronary obstruction is recommended.
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