Why the study?
Although most Marfan syndrome patients do not require cardiac surgery until adulthood, cardiovascular disease in the pediatric Marfan syndrome population remains notable.
What are the short- and long-term outcomes of aortic and/or mitral valve surgery in pediatric patients with Marfan syndrome?
What are the short- and long-term outcomes of aortic and/or mitral valve surgery in pediatric patients with Marfan syndrome?
Surgical intervention for cardiovascular complications in pediatric Marfan syndrome patients is associated with low in-hospital mortality and excellent 15-year survival, though reintervention rates remain substantial.
Supports surgical safety in pediatric Marfan syndrome; leaves open need for prospective validation before practice change.
Background Individuals 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. Methods This 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. Results A 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. Conclusions Operations 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.
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O’Connor et al. (2025) studied this question.
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