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

Reant, P; Captur, G; Mirabel, M; Nasis, A; M Sado, D; Maestrini, V; Castelletti, S; Manisty, C; Herrey, AS; Syrris, P; et al. Reant, P; Captur, G; Mirabel, M; Nasis, A; M Sado, D; Maestrini, V; Castelletti, S; Manisty, C; Herrey, AS; Syrris, P; Tome-Esteban, M; Jenkins, S; Elliott, PM; McKenna, WJ; Moon, JC (2015) Abnormal septal convexity into the left ventricle occurs in subclinical hypertrophic cardiomyopathy. J Cardiovasc Magn Reson, 17. p. 64. ISSN 1532-429X https://doi.org/10.1186/s12968-015-0160-y
SGUL Authors: Tome, Maria Teresa

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Abstract

BACKGROUND: Sarcomeric 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. METHODS: Cardiovascular 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. RESULTS: Septal 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/m(2) vs. 23.7 ± 5.8 ml/m(2), p ≤ 0.05). Other morphologic measurements (LV angles, sphericity index, and eccentricity index) were not different between G + LVH- and controls. CONCLUSIONS: Septal convexity is an additional previously undescribed feature of subclinical HCM.

Item Type: Article
Additional Information: © Reant et al. 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Keywords: Adolescent, Adult, Asymptomatic Diseases, Cardiomyopathy, Hypertrophic, Case-Control Studies, Female, Genetic Markers, Genetic Predisposition to Disease, Heart Ventricles, Humans, Magnetic Resonance Imaging, Cine, Male, Middle Aged, Mutation, Phenotype, Predictive Value of Tests, Stroke Volume, Ventricular Function, Left, Young Adult, Heart Ventricles, Humans, Cardiomyopathy, Hypertrophic, Genetic Predisposition to Disease, Genetic Markers, Magnetic Resonance Imaging, Cine, Stroke Volume, Case-Control Studies, Predictive Value of Tests, Ventricular Function, Left, Phenotype, Mutation, Adolescent, Adult, Middle Aged, Female, Male, Young Adult, Asymptomatic Diseases, Nuclear Medicine & Medical Imaging, 1102 Cardiovascular Medicine And Haematology
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cardiac (INCCCA)
Journal or Publication Title: J Cardiovasc Magn Reson
ISSN: 1532-429X
Language: eng
Dates:
DateEvent
30 July 2015Published
23 June 2015Accepted
Publisher License: Creative Commons: Attribution 4.0
PubMed ID: 26219660
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/108644
Publisher's version: https://doi.org/10.1186/s12968-015-0160-y

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