Mitral Valve Abnormalities Identified by Cardiovascular Magnetic Resonance Represent a Primary Phenotypic Expression of Hypertrophic Cardiomyopathy

DOI Web Site 6 Citations
  • Martin S. Maron
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • Iacopo Olivotto
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • Caitlin Harrigan
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • Evan Appelbaum
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • C. Michael Gibson
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • John R. Lesser
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • Tammy S. Haas
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • James E. Udelson
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • Warren J. Manning
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...
  • Barry J. Maron
    From the Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA (M.S.M., C.H., J.E.U.); Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy (I.O.); PERFUSE Core Laboratory and Data Coordinating Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); Department of Medicine, Cardiovascular Division, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA (E.A., C.M.G., W.J.M.); and...

Description

<jats:sec> <jats:title>Background—</jats:title> <jats:p>Whether morphological abnormalities of the mitral valve represent part of the hypertrophic cardiomyopathy (HCM) disease process is unresolved. Therefore, we applied cardiovascular magnetic resonance to characterize mitral valve morphology in a large HCM cohort.</jats:p> </jats:sec> <jats:sec> <jats:title>Methods and Results—</jats:title> <jats:p> Cine cardiac magnetic resonance images were obtained in 172 HCM patients (age, 42±18 years; 62% men) and 172 control subjects. In addition, 15 HCM gene-positive/phenotype-negative relatives were studied. Anterior mitral leaflet (AML) and posterior mitral leaflet lengths were greater in HCM patients than in control subjects (26±5 versus 19±5 mm, <jats:italic>P</jats:italic> <0.001; and 14±4 versus 10±3 mm, <jats:italic>P</jats:italic> <0.001, respectively), including 59 patients (34%) in whom AML length alone, posterior mitral leaflet length alone, or both were particularly substantial (>2 SDs above controls). Leaflet length was increased compared with controls in virtually all HCM age groups, including young patients 15 to 20 years of age (AML, 26±5 versus 21±4 mm; <jats:italic>P</jats:italic> =0.0002) and those ≥60 years of age (AML, 26±4 versus 19±2 mm; <jats:italic>P</jats:italic> <0.001). No relation was evident between mitral leaflet length and LV thickness or mass index ( <jats:italic>P</jats:italic> =0.09 and <jats:italic>P</jats:italic> =0.16, respectively). A ratio of AML length to LV outflow tract diameter of >2.0 was associated with subaortic obstruction ( <jats:italic>P</jats:italic> =0.001). In addition, AML length in 15 genotype-positive relatives without LV hypertrophy exceeded that of matched control subjects (21±3 versus 18±3 mm; <jats:italic>P</jats:italic> <0.01). </jats:p> </jats:sec> <jats:sec> <jats:title>Conclusions—</jats:title> <jats:p>In HCM, mitral valve leaflets are elongated independently of other disease variables, likely constituting a primary phenotypic expression of this heterogeneous disease, and are an important morphological abnormality responsible for LV outflow obstruction in combination with small outflow tract dimension. These findings suggest a novel role for cardiac magnetic resonance in the assessment of HCM.</jats:p> </jats:sec>

Journal

  • Circulation

    Circulation 124 (1), 40-47, 2011-07-05

    Ovid Technologies (Wolters Kluwer Health)

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