Key result
In six infants and young children with Marfan syndrome, mitral valve replacement with a mechanical prosthesis was a feasible and durable treatment option, whereas mitral valve repair may not be durable.
Why the study?
Does mitral valve replacement or repair provide better outcomes in infantile-onset Marfan syndrome patients with mitral regurgitation?
Observational (n=6)
Yes
Does mitral valve replacement or repair provide better outcomes in infantile-onset Marfan syndrome patients with mitral regurgitation?
Mitral valve replacement with a mechanical prosthesis may be a more durable and feasible option than valve repair for infantile-onset Marfan syndrome patients requiring surgery for mitral regurgitation.
May support mechanical mitral replacement over repair in young Marfan patients; leaves open need for prospective confirmation before practice change.
BACKGROUND: Mitral regurgitation is one of the leading causes of cardiovascular morbidity in pediatric patients with Marfan syndrome. The purpose of this study was to contribute to determining the appropriate surgical strategy for these patients. METHODS: From January 1992 to May 2013, six patients with Marfan syndrome underwent surgery for mitral regurgitation in infancy or early childhood. RESULTS: The median age at the time of surgery was 47 months (range, 3 to 140 months) and the median follow-up period was 3.6 years (range, 1.3 to 15.5 years). Mitral valve repair was performed in two patients and four patients underwent mitral valve replacement with a mechanical prosthesis. There was one reoperation requiring valve replacement for aggravated mitral regurgitation two months after repair. The four patients who underwent mitral valve replacement did not experience any complications related to the prosthetic valve. One late death occurred due to progressive emphysema and tricuspid regurgitation. CONCLUSION: Although repair can be an option for some patients, it may not be durable in infantile-onset Marfan syndrome patients who require surgical management during infancy or childhood. Mitral valve replacement is a feasible treatment option for these patients.
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Kim et al. (2015) conducted an observational in Mitral regurgitation in Marfan syndrome (n=6). Mitral valve surgery (repair or replacement) was evaluated on Surgical outcomes (mortality, reoperation, complications). In six infants and young children with Marfan syndrome, mitral valve replacement with a mechanical prosthesis was a feasible and durable treatment option, whereas mitral valve repair may not be durable.
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