title: Surgical Management of Mitral Regurgitation in Patients with Marfan Syndrome during Infancy and Early Childhood.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine.
authors.forename: Eung Re
authors.initials: ER
authors.lastname: Kim
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine.
authors.forename: Woong-Han
authors.initials: WH
authors.lastname: Kim
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine.
authors.forename: Eun Seok
authors.initials: ES
authors.lastname: Choi
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul National University College of Medicine.
authors.forename: Sungkyu
authors.initials: S
authors.lastname: Cho
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital.
authors.forename: Woo Sung
authors.initials: WS
authors.lastname: Jang
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine.
authors.forename: Yong Jin
authors.initials: YJ
authors.lastname: Kim
keywords: Infant; Marfan syndrome; Mitral valve insufficiency