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Surgical management of mitral regurgitation associated with Marfan's syndrome
J F Fuzellier1, S M Chauvaud, P Fornes
1Department of Cardiovascular Surgery, Broussais Hospital, Paris, France.
Background:
The surgical treatment of mitral regurgitation associated with Marfan's syndrome remains controversial because of the underlying degenerative process.
Methods:
From October 1986 to June 1996, 33 patients with Marfan's syndrome underwent a mitral valve procedure. The mean age was 30 years (range, 2 to 55 years). Mitral regurgitation was caused by annulus dilatation in 2 patients, leaflet prolapse in 30 patients, and restricted leaflet motion in 1 patient. Mitral valve repair was performed in all patients except 1 who had mitral valve replacement.
Results:
Two patients died in the perioperative period. All survivors were available for follow-up, which ranged from 1 month to 122 months (mean follow-up, 39 months). There were three late deaths, two of which were related to aortic complications. The actuarial survival rate was 78.9% at 10 years. Freedom from mitral valve reoperation was 87.1% at 10 years. Echocardiographic studies were obtained in all survivors and showed absent or mild (1+) mitral regurgitation in 21 patients and moderate (2+) mitral regurgitation in 3.
Conclusions:
Mitral valve repair for mitral regurgitation in patients with Marfan's syndrome can be performed safely in almost all instances. This technique provided stable midterm results comparable with those obtained for other degenerative mitral valve diseases.
Insights
Mitral valve repair is a safe and effective treatment for Marfan syndrome patients with mitral regurgitation, offering stable midterm outcomes. This approach yields results comparable to other degenerative mitral valve diseases.
Area of Science:
- Cardiovascular Surgery
- Cardiac Valve Disease
- Genetics
Background:
- Surgical treatment for mitral regurgitation in Marfan syndrome is debated due to the condition's degenerative nature.
- Marfan syndrome often involves connective tissue abnormalities affecting heart valves.
Purpose of the Study:
- To evaluate the safety and efficacy of mitral valve repair in patients with Marfan syndrome.
- To assess midterm outcomes of mitral valve repair for degenerative mitral regurgitation in this population.
Main Methods:
- A cohort of 33 Marfan syndrome patients underwent mitral valve procedures between 1986 and 1996.
- Mitral valve repair was the primary surgical approach, with one replacement.
- Follow-up ranged from 1 to 122 months.
Main Results:
- Perioperative mortality was 6% (2 patients).
- Actuarial survival at 10 years was 78.9%.
- Freedom from mitral valve reoperation at 10 years was 87.1%, with most survivors showing absent or mild mitral regurgitation.
Conclusions:
- Mitral valve repair is a safe and effective procedure for mitral regurgitation in Marfan syndrome.
- The technique provides durable midterm results comparable to other degenerative mitral valve conditions.