Document Detail

Congenital mitral valve regurgitation in adult patients. A rare, often misdiagnosed but repairable, valve disease.
MedLine Citation:
PMID:  18760929     Owner:  NLM     Status:  MEDLINE    
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. CONCLUSION: Congenital MVR is rare in adults, often misdiagnosed and accessible to valve repair with excellent mid-term results.
Rachid Zegdi; Brahim Amahzoune; Mustapha Ladjali; Ghassan Sleilaty; Jérome Jouan; Christian Latrémouille; Alain Deloche; Jean-Noël Fabiani
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Publication Detail:
Type:  Journal Article     Date:  2008-08-30
Journal Detail:
Title:  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery     Volume:  34     ISSN:  1873-734X     ISO Abbreviation:  Eur J Cardiothorac Surg     Publication Date:  2008 Oct 
Date Detail:
Created Date:  2008-10-17     Completed Date:  2009-03-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8804069     Medline TA:  Eur J Cardiothorac Surg     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  751-4     Citation Subset:  IM    
AP-HP, Assistance Publique - Hôpitaux de Paris, Service de Chirurgie Cardiovasculaire, Hôpital Européen Georges Pompidou, Paris, France.
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MeSH Terms
Echocardiography, Transesophageal
Follow-Up Studies
Middle Aged
Mitral Valve Insufficiency / congenital*,  surgery*,  ultrasonography
Treatment Outcome
Young Adult

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