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Published in: Journal of Cardiovascular Magnetic Resonance 1/2015

Open Access 01-12-2015 | Research

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

Authors: Patricia Reant, Gabriella Captur, Mariana Mirabel, Arthur Nasis, Daniel M. Sado, Viviana Maestrini, Silvia Castelletti, Charlotte Manisty, Anna S. Herrey, Petros Syrris, Maite Tome-Esteban, Sharon Jenkins, Perry M. Elliott, William J. McKenna, James C. Moon

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2015

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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/m2 vs. 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.

Conclusions

Septal convexity is an additional previously undescribed feature of subclinical HCM.
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Metadata
Title
Abnormal septal convexity into the left ventricle occurs in subclinical hypertrophic cardiomyopathy
Authors
Patricia Reant
Gabriella Captur
Mariana Mirabel
Arthur Nasis
Daniel M. Sado
Viviana Maestrini
Silvia Castelletti
Charlotte Manisty
Anna S. Herrey
Petros Syrris
Maite Tome-Esteban
Sharon Jenkins
Perry M. Elliott
William J. McKenna
James C. Moon
Publication date
01-12-2015
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2015
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/s12968-015-0160-y

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