title: Rupture of mitral valve chordae in hypertrophic cardiomyopathy.
abstract: BACKGROUND
  While occasional reports of mitral valve chordal rupture have been described in hypertrophic cardiomyopathy, the exact prevalence and characteristics of this event in a large medical cohort have not been reported.
  
  
  OBJECTIVE
  To assess the prevalence of mitral valve chordal rupture in hypertrophic cardiomyopathy and the clinical, echocardiographic, surgical and histological profiles of those patients.
  
  
  METHODS
  We searched for patients with mitral valve chordal rupture diagnosed by echocardiography among all electronic files of patients admitted to our centre for hypertrophic cardiomyopathy between 2000 and 2010.
  
  
  RESULTS
  Among 580 patients admitted for hypertrophic cardiomyopathy, six patients (1%, 5 men, age 68-71 years) presented with mitral valve chordal rupture, symptomatic in five cases, always involving the posterior mitral leaflet. In all cases, echocardiography before rupture showed mitral valve systolic anterior motion, with anterior (and not posterior) leaflet elongation compared with a random sample of patients with non-obstructive hypertrophic cardiomyopathy (P=0.006) (and similar to that observed in obstructive hypertrophic cardiomyopathy). Significant resting left ventricular outflow tract obstruction was always present before rupture and disappeared after rupture in the five cases requiring mitral valve surgery for severe mitral regurgitation. Histological findings were consistent with extensive myxomatous degeneration in all cases.
  
  
  CONCLUSIONS
  Mitral valve chordal rupture is: infrequent in hypertrophic cardiomyopathy; occurs in aged patients with obstructive disease; involves, essentially, the posterior mitral leaflet; and causes, in general, severe mitral regurgitation requiring surgery. Myxomatous degeneration may be the substrate for rupture in these patients.
authors.affiliation: Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
authors.forename: Florence
authors.initials: F
authors.lastname: Boissier
authors.affiliation: Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
authors.forename: Guy
authors.initials: G
authors.lastname: Achkouty
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, 75475 Paris, France; Department of Pathology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Bruneval
authors.affiliation: Université Paris Descartes, Sorbonne Paris Cité, 75475 Paris, France; Department of Cardiovascular Surgery, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
authors.forename: Jean-Noël
authors.initials: JN
authors.lastname: Fabiani
authors.affiliation: Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.
authors.forename: Anh Tuan
authors.initials: AT
authors.lastname: Nguyen
authors.affiliation: Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France; Université Paris Descartes, Sorbonne Paris Cité, 75475 Paris, France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Riant
authors.affiliation: Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France; Université Paris Descartes, Sorbonne Paris Cité, 75475 Paris, France; Inserm U970, Paris Cardiovascular Research Centre (PARCC-HEGP), 75015 Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Desnos
authors.affiliation: Department of Cardiology, hôpital Européen Georges Pompidou, Assistance publique-Hôpitaux de Paris, 75015 Paris, France; Université Paris Descartes, Sorbonne Paris Cité, 75475 Paris, France; Inserm U970, Paris Cardiovascular Research Centre (PARCC-HEGP), 75015 Paris, France. Electronic address: albert.hagege@egp.aphp.fr.
authors.forename: Albert
authors.initials: A
authors.lastname: Hagège
mesh_terms: D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D002815:Chordae Tendineae / Q000000981:diagnostic imaging / Q000601:surgery; D015150:Echocardiography, Doppler; D005260:Female; D006341:Heart Rupture / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D008943:Mitral Valve / Q000000981:diagnostic imaging / Q000601:surgery; D058386:Mitral Valve Annuloplasty; D008944:Mitral Valve Insufficiency / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D010297:Paris / Q000453:epidemiology; D011237:Predictive Value of Tests; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D016896:Treatment Outcome
keywords: Cardiomyopathie hypertrophique; Chordal rupture; Hypertrophic cardiomyopathy; Insuffisance mitrale; Mitral regurgitation; Mitral valve repair; Plastie mitrale; Rupture de cordage mitral