title: Epicardial myocardial strain abnormalities may identify the earliest stages of arrhythmogenic cardiomyopathy.
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.
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK. patricia.reant@chu-bordeaux.fr.|CHU de Bordeaux, CIC1401 (Pessac), University of Bordeaux, Bordeaux, France. patricia.reant@chu-bordeaux.fr.|Hôpital Cardiologique Haut-Lévêque, 1 avenue de Magellan, 33604, Pessac Cedex, France. patricia.reant@chu-bordeaux.fr.
authors.forename: Patricia
authors.initials: P
authors.lastname: Réant
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.|Haga Teaching Hospital, The Hague, The Netherlands.
authors.forename: Arnaud D
authors.initials: AD
authors.lastname: Hauer
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: Silvia
authors.initials: S
authors.lastname: Castelletti
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: Antonis
authors.initials: A
authors.lastname: Pantazis
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: Stefania
authors.initials: S
authors.lastname: Rosmini
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: Mun Hong
authors.initials: MH
authors.lastname: Cheang
authors.affiliation: CHU de Bordeaux, CIC1401 (Pessac), University of Bordeaux, Bordeaux, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Peyrou
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: Maite
authors.initials: M
authors.lastname: Tomé-Esteban
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: Petros
authors.initials: P
authors.lastname: Syrris
authors.affiliation: CHU de Bordeaux, CIC1401 (Pessac), University of Bordeaux, Bordeaux, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Lafitte
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: James C
authors.initials: JC
authors.lastname: Moon
authors.affiliation: Inherited Cardiac Disease Unit and Division of Cardiovascular Imaging, The Heart Hospital, Institute of Cardiovascular Science, University College London, London, UK.
authors.forename: William J
authors.initials: WJ
authors.lastname: McKenna
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology; D016022:Case-Control Studies; D042241:Early Diagnosis; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D008131:London; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction; D010496:Pericardium / Q000000981:diagnostic imaging* / Q000503:physiopathology; D011237:Predictive Value of Tests; D013314:Stress, Mechanical; D013318:Stroke Volume; D016277:Ventricular Function, Left; D055815:Young Adult
keywords: Arrhythmogenic cardiomyopathy; Cardiac imaging; Echocardiography; Myocardial strain; Speckle tracking