Korean J Thorac Cardiovasc Surg.  2015 Feb;48(1):7-12. 10.5090/kjtcs.2015.48.1.7.

Surgical Management of Mitral Regurgitation in Patients with Marfan Syndrome during Infancy and Early Childhood

• Kim ER
• Kim WH
• Choi ES
• Cho S
• Jang WS
• Kim YJ

• KMID: 2382220
• DOI: http://doi.org/10.5090/kjtcs.2015.48.1.7

Abstract

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.

Keyword

Marfan syndrome; Infant; Mitral valve insufficiency

MeSH Terms

Emphysema
Follow-Up Studies
Humans
Infant
Marfan Syndrome*
Mitral Valve
Mitral Valve Insufficiency*
Prostheses and Implants
Reoperation
Tricuspid Valve Insufficiency