ArticleWorld journal for pediatric & congenital heart surgery2025
Surgical Intervention in Pediatric Marfan Syndrome: A Multiinstitutional Study.
Article in World journal for pediatric & congenital heart surgery, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
BackgroundIndividuals with Marfan syndrome (MFS) are at risk for the development of cardiovascular complications. Although the majority of MFS patients do not require cardiac surgery until adulthood, cardiovascular disease in the pediatric MFS population is still notable.MethodsThis is a retrospective of the Pediatric Health Information System from January 2004 to December 2023. All patients <18 years of age with a diagnosis of MFS who underwent aortic (Ao) and/or mitral valve (MV) surgery were included.ResultsA total of 343 patients were identified, with 115 (34%) females, 190 (55%) White non-Hispanic, and a median age of 13.0 [interquartile range (IQR): 9.0-16.0] years at surgery. Among the cohort, 241/343 (70%) underwent an aortic procedure, 40/343 (12%) aortic + MV procedure, and 62/343 (18%) MV procedure. Median hospital length of stay was 6.0 [IQR: 5.0-9.5] days, 9/343 (3%) patients required extracorporeal membrane oxygenation, and 4/343 (1%) died in-hospital. Freedom from any reintervention at 15 years was 48.8% (95% CI: 34.0-70.0). Multivariable Cox regression analysis revealed that older age at the time of surgery was associated with a reduced risk of reintervention (hazard ratio: 0.94; 95% CI: 0.89-0.99,
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