title: Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death.
abstract: BACKGROUND
  Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular electric instability remains elusive.
  
  
  RESULTS
  The cardiac pathology registry of 650 young adults (≤40 years of age) with SCD was reviewed, and cases with MVP as the only cause of SCD were re-examined. Forty-three patients with MVP (26 females; age range, 19-40 years; median, 32 years) were identified (7% of all SCD, 13% of women). Among 12 cases with available ECG, 10 (83%) had inverted T waves on inferior leads, and all had right bundle-branch block ventricular arrhythmias. A bileaflet involvement was found in 70%. Left ventricular fibrosis was detected at histology at the level of papillary muscles in all patients, and inferobasal wall in 88%. Living patients with MVP with (n=30) and without (control subjects; n=14) complex ventricular arrhythmias underwent a study protocol including contrast-enhanced cardiac magnetic resonance. Patients with either right bundle-branch block type or polymorphic complex ventricular arrhythmias (22 females; age range, 28-43 years; median, 41 years), showed a bileaflet involvement in 70% of cases. Left ventricular late enhancement was identified by contrast-enhanced cardiac magnetic resonance in 93% of patients versus 14% of control subjects (P<0.001), with a regional distribution overlapping the histopathology findings in SCD cases.
  
  
  CONCLUSIONS
  MVP is an underestimated cause of arrhythmic SCD, mostly in young adult women. Fibrosis of the papillary muscles and inferobasal left ventricular wall, suggesting a myocardial stretch by the prolapsing leaflet, is the structural hallmark and correlates with ventricular arrhythmias origin. Contrast-enhanced cardiac magnetic resonance may help to identify in vivo this concealed substrate for risk stratification.
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy. cristina.basso@unipd.it.
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Martina
authors.initials: M
authors.lastname: Perazzolo Marra
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Rizzo
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Manuel
authors.initials: M
authors.lastname: De Lazzari
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Benedetta
authors.initials: B
authors.lastname: Giorgi
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Cipriani
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Anna Chiara
authors.initials: AC
authors.lastname: Frigo
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Rigato
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Federico
authors.initials: F
authors.lastname: Migliore
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Pilichou
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Emanuele
authors.initials: E
authors.lastname: Bertaglia
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Cacciavillani
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Bauce
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
authors.affiliation: From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy.
authors.forename: Sabino
authors.initials: S
authors.lastname: Iliceto
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology* / Q000473:pathology; D002037:Bundle-Branch Block / Q000209:etiology / Q000473:pathology; D002815:Chordae Tendineae / Q000473:pathology; D017023:Coronary Angiography; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology*; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D005260:Female; D005355:Fibrosis; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008943:Mitral Valve / Q000473:pathology; D008945:Mitral Valve Prolapse / Q000150:complications* / Q000473:pathology; D010210:Papillary Muscles / Q000473:pathology; D012737:Sex Factors; D055815:Young Adult
keywords: arrhythmias, cardiac; death, sudden, cardiac; implantable cardioverter defibrillator; magnetic resonance imaging; mitral valve; pathology