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Epicardial myocardial strain abnormalities may identify the earliest stages of arrhythmogenic cardiomyopathy

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Abstract

The aim of this cohort study was to evaluate the value of echocardiographic multilayer strain analysis in the identification of arrhythmogenic cardiomyopathy (AC) in its earliest stages in which sudden cardiac death can occurs. Twenty seven asymptomatic relatives of AC probands (mean age 39.6 ± 19.5 years, 37 % male) with a desmosomal pathogenic mutation but no additional criteria for AC (group II) were compared to age and sex-matched healthy controls (group I). In addition, 70 patients harboring a pathogenic desmosomal mutation with “definitive” diagnosis of AC (group IV), and 19 subjects with “borderline” diagnosis (group III) were also studied. A standard echocardiographic evaluation plus left (LV) and right ventricular global and regional transmural, endocardial, and epicardial longitudinal strain (LS) analysis, was performed. In group II, while LV ejection fraction, fractional shortening, and S′ were not significantly reduced compared to controls, transmural global LS was significantly reduced to 19.3 ± 1.8 % in group II versus 20.9 ± 1.1 % in controls (p = 0.0003). Compared to controls, group II presented significant (p < 0.05) regional LS decrease in the basal infero-lateral, antero-lateral, latero-apical, infero-septal, and septo-apical segments. Moreover, LS of the latero-apical and the basal antero-lateral segments was significantly altered in the epicardium (p < 0.05) but not significantly in the endocardium. Global and regional LV LS analysis allows detection of AC in an early or non-diagnostic stage of the disease. Moreover, epicardial LS analysis allows the detection of abnormalities earlier than endocardial LS.

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Abbreviations

AC:

Arrhythmogenic cardiomyopathy

CMR:

Cardiovascular magnetic resonance

EF:

Ejection fraction

LV:

Left ventricle

LGE:

Late gadolinium enhancement

LS:

Longitudinal strain

ROC:

Receiver operating characteristics

RV:

Right ventricle

TFC:

Task Force Criteria

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Acknowledgements

This work was undertaken at University College London Hospitals/University College London, which receives a proportion of funding from the Department of Health’s NIHR Biomedical Research Centre funding scheme. Professors JCM and WJM are funded by the Higher Education Funding Council for England. Dr. PR was supported by the Fédération Francaise de Cardiologie. Dr. AH was supported by the Leiden University Medical Center. Dr. SC was funded by the 2014 ESC research grant. We thank Mrs. Shaughan Dickie and Mrs. Sarah Anderson for their help.

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Authors and Affiliations

  1. Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK

    Patricia Réant, Arnaud D. Hauer, Silvia Castelletti, Antonis Pantazis, Stefania Rosmini, Mun Hong Cheang, Maite Tomé-Esteban, Petros Syrris, James C. Moon & William J. McKenna

  2. CHU de Bordeaux, CIC1401 (Pessac), University of Bordeaux, Bordeaux, France

    Patricia Réant, Jérôme Peyrou & Stéphane Lafitte

  3. Haga Teaching Hospital, The Hague, The Netherlands

    Arnaud D. Hauer

  4. Hôpital Cardiologique Haut-Lévêque, 1 avenue de Magellan, 33604, Pessac Cedex, France

    Patricia Réant

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  1. Patricia Réant
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Correspondence to Patricia Réant.

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Réant, P., Hauer, A.D., Castelletti, S. et al. Epicardial myocardial strain abnormalities may identify the earliest stages of arrhythmogenic cardiomyopathy. Int J Cardiovasc Imaging 32, 593–601 (2016). https://doi.org/10.1007/s10554-015-0813-9

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  • DOI: https://doi.org/10.1007/s10554-015-0813-9

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