Abnormal septal convexity into the left ventricle occurs in subclinical hypertrophic cardiomyopathy

Journal of Cardiovascular Magnetic Resonance

Research

Silvia Castelletti¹  Gabriella Captur¹  Perry M. Elliott¹  Maite Tome-Esteban¹  Daniel M. Sado¹  William J. McKenna¹  James C. Moon¹  Charlotte Manisty¹  Petros Syrris¹  Viviana Maestrini¹  Anna S. Herrey¹  Arthur Nasis¹  Sharon Jenkins¹  Patricia Reant²  Mariana Mirabel³ 

[1] Division of Cardiovascular Imaging and Inherited Cardiac Disease Unit, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK;Division of Cardiovascular Imaging and Inherited Cardiac Disease Unit, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK;Hôpital Cardiologique du Haut-Levêque (Pessac), CHU de Bordeaux, Université de Bordeaux, Bordeaux, France;Division of Cardiovascular Imaging and Inherited Cardiac Disease Unit, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK;INSERM U970, Paris Cardiovascular Research Center PARCC, Paris, France;

关键词: Hypertrophic cardiomyopathy;    Cardiovascular magnetic resonance;    Septal curvature;    Genetics;   

DOI  :  10.1186/s12968-015-0160-y

 received in 2015-01-21, accepted in 2015-06-23,  发布年份 2015

来源: Springer

【 摘 要 】

BackgroundSarcomeric gene mutations cause hypertrophic cardiomyopathy (HCM). In gene mutation carriers without left ventricular (LV) hypertrophy (G + LVH-), subclinical imaging biomarkers are recognized as predictors of overt HCM, consisting of anterior mitral valve leaflet elongation, myocardial crypts, hyperdynamic LV ejection fraction, and abnormal apical trabeculation. Reverse curvature of the interventricular septum (into the LV) is characteristic of overt HCM. We aimed to assess LV septal convexity in subclinical HCM.MethodsCardiovascular magnetic resonance was performed on 36 G + LVH- individuals (31 ± 14 years, 33 % males) with a pathogenic sarcomere mutation, and 36 sex and age-matched healthy controls (33 ± 12 years, 33 % males). Septal convexity (SCx) was measured in the apical four chamber view perpendicular to a reference line connecting the mid-septal wall at tricuspid valve insertion level and the apical right ventricular insertion point.ResultsSeptal convexity was increased in G + LVH- compared to controls (maximal distance of endocardium to reference line: 5.0 ± 2.5 mm vs. 1.6 ± 2.4 mm, p ≤ 0.0001). Expected findings occurred in G + LVH- individuals: longer anterior mitral valve leaflet (23.5 ± 3.0 mm vs. 19.9 ± 3.1 mm, p ≤ 0.0001), higher relative wall thickness (0.31 ± 0.05 vs. 0.29 ± 0.04, p ≤ 0.05), higher LV ejection fraction (70.8 ± 4.3 % vs. 68.3 ± 4.4 %, p ≤ 0.05), and smaller LV end-systolic volume index (21.4 ± 4.4 ml/m2vs. 23.7 ± 5.8 ml/m2, p ≤ 0.05). Other morphologic measurements (LV angles, sphericity index, and eccentricity index) were not different between G + LVH- and controls.ConclusionsSeptal convexity is an additional previously undescribed feature of subclinical HCM.

【 授权许可】

CC BY   
© Reant et al. 2015