Surgical Intervention in Pediatric Marfan Syndrome: A Multiinstitutional Study

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, p = 0.023). The overall survival rate at 15 years was 94.2% (95% CI: 90.5-98.0), and no factors were associated with increased mortality.ConclusionsOperations for cardiovascular complications of MFS can be performed safely in appropriately selected children with low morbidity and mortality and with good short-term and long-term outcomes. These findings support the safety and efficacy of surgical intervention in this population.