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Surgical Intervention in Pediatric Marfan Syndrome: A Multiinstitutional Study
Mario O'Connor1, Andrew Well2, Maria E Hoyos1
1Texas Center for Pediatric and Congenital Heart Disease, The University of Texas at Austin Dell Medical School, Austin, TX, USA.
Insights
Pediatric Marfan syndrome (MFS) patients undergoing aortic or mitral valve surgery show low morbidity and mortality. Surgical intervention in children with MFS is safe and effective, with good long-term outcomes and reduced reintervention risk in older patients.
Area of Science:
- Cardiology
- Pediatric Surgery
- Genetics
Background:
- Marfan syndrome (MFS) patients face significant cardiovascular risks.
- While many MFS patients need surgery in adulthood, pediatric cardiovascular disease is a concern.
- Early surgical intervention may be necessary for managing MFS-related cardiac complications in children.
Purpose of the Study:
- To evaluate the safety and outcomes of aortic (Ao) and/or mitral valve (MV) surgery in pediatric patients with Marfan syndrome.
- To identify factors influencing reintervention and mortality in this cohort.
- To assess the long-term efficacy of surgical interventions for cardiovascular complications in pediatric MFS.
Main Methods:
- Retrospective analysis of the Pediatric Health Information System (2004-2023).
- Inclusion of patients under 18 with MFS undergoing Ao and/or MV surgery.
- Statistical analysis including multivariable Cox regression to determine risk factors.
Main Results:
- 343 pediatric MFS patients underwent surgery; 70% had aortic procedures.
- In-hospital mortality was low (1%), with 3% requiring extracorporeal membrane oxygenation.
- 15-year freedom from reintervention was 48.8%; older age at surgery reduced reintervention risk.
- Overall 15-year survival rate was 94.2% with no factors associated with increased mortality.
Conclusions:
- Surgical repair of cardiovascular complications in pediatric Marfan syndrome is safe and effective.
- Low morbidity and mortality rates were observed, with favorable short- and long-term outcomes.
- These findings support timely surgical intervention for children with MFS-related cardiac issues.
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, 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.
