title: Congenital mitral valve regurgitation in adult patients. A rare, often misdiagnosed but repairable, valve disease.
abstract: OBJECTIVE
  Congenital mitral valve regurgitation (MVR) is a rare disease occurring in infancy or childhood. Although congenital MVR has been described in adults, no surgical series has been reported so far. We describe here a 6-year surgical experience of congenital MVR in adults at a single institution.
  
  
  METHODS
  We reviewed the data of 15 consecutive patients (8 men), aged more than 16 years (median: 38 years; range: 16-70 years) operated on for severe congenital MVR from June 2000 to March 2006. Congenital MVR represented 2.1% of mitral valve surgery performed in adults during the same period. Patients with atrio-ventricular septal defect or atrio-ventricular discordance were excluded.
  
  
  RESULTS
  The congenital MVR was preoperatively diagnosed in six (40%) cases. Two (13%) patients had a Williams-Beuren syndrome. The lesions consisted in annular dilation (100%), prolapsed leaflet (87%), chordal abnormalities (80%), papillary muscle abnormalities (40%) or valvular cleft (33%). Mitral valve repair was performed in all cases using Carpentier's techniques. There was no hospital death or late mortality. At last follow-up (median: 60 months; range: 6-83 months), all patients were in NYHA functional class I or II and in a sinus rhythm. On transthoracic echocardiography, 11 (73%) patients had no or trivial MVR. Mild MVR was present in four (27%) patients. No patient was reoperated and endocarditis did not occur.
  
  
  CONCLUSIONS
  Congenital MVR is rare in adults, often misdiagnosed and accessible to valve repair with excellent mid-term results.
authors.affiliation: AP-HP, Assistance Publique - Hôpitaux de Paris, Service de Chirurgie Cardiovasculaire, Hôpital Européen Georges Pompidou, Paris, France. rzegdi@hotmail.com
authors.forename: Rachid
authors.initials: R
authors.lastname: Zegdi
authors.forename: Brahim
authors.initials: B
authors.lastname: Amahzoune
authors.forename: Mustapha
authors.initials: M
authors.lastname: Ladjali
authors.forename: Ghassan
authors.initials: G
authors.lastname: Sleilaty
authors.forename: Jérome
authors.initials: J
authors.lastname: Jouan
authors.forename: Christian
authors.initials: C
authors.lastname: Latrémouille
authors.forename: Alain
authors.initials: A
authors.lastname: Deloche
authors.forename: Jean-Noël
authors.initials: JN
authors.lastname: Fabiani