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The added value of abnormal regional myocardial function for risk prediction in arrhythmogenic right ventricular cardiomyopathy

Kirkels, FP; Rootwelt-Norberg, C; Bosman, LP; Aabel, EW; Muller, SA; Castrini, AI; Taha, K; ... Teske, AJ; + view all (2023) The added value of abnormal regional myocardial function for risk prediction in arrhythmogenic right ventricular cardiomyopathy. European Heart Journal Cardiovascular Imaging , 24 (12) pp. 1710-1718. 10.1093/ehjci/jead174. Green open access

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Abstract

Aims A risk calculator for individualized prediction of first-time sustained ventricular arrhythmia (VA) in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients has recently been developed and validated (www.ARVCrisk.com). This study aimed to investigate whether regional functional abnormalities, measured by echocardiographic deformation imaging, can provide additional prognostic value. Methods From two referral centres, 150 consecutive patients with a definite ARVC diagnosis, no prior sustained VA, and an echo- and results cardiogram suitable for deformation analysis were included (aged 41 ± 17 years, 50% female). During a median follow-up of 6.3 (interquartile range 3.1–9.8) years, 37 (25%) experienced a first-time sustained VA. All tested left and right ventricular (LV and RV) deformation parameters were univariate predictors for first-time VA. While LV function did not add predictive value in multivariate analysis, two RV deformation parameters did; RV free wall longitudinal strain and regional RV deformation patterns remained independent predictors after adjusting for the calculator-predicted risk [hazard ratio 1.07 (95% CI 1.02–1.11); P = 0.004 and 4.45 (95% CI 1.07–18.57); P = 0.040, respectively] and improved its discriminative value (from C-statistic 0.78 to 0.82 in both; Akaike information criterion change > 2). Importantly, all patients who experienced VA within 5 years from the echocardiographic assessment had abnormal regional RV deformation patterns at baseline. Conclusions This study showed that regional functional abnormalities measured by echocardiographic deformation imaging can further refine personalized arrhythmic risk prediction when added to the ARVC risk calculator. The excellent negative predictive value of normal RV deformation could support clinicians considering the timing of implantable cardioverter defibrillator implantation in patients with intermediate arrhythmic risk.

Type: Article
Title: The added value of abnormal regional myocardial function for risk prediction in arrhythmogenic right ventricular cardiomyopathy
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/ehjci/jead174
Publisher version: http://dx.doi.org/10.1093/ehjci/jead174
Language: English
Additional information: This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com.
Keywords: Arrhythmogenic cardiomyopathy, echocardiography, risk stratification, strain imaging, sudden cardiac death, ventricular arrhythmia, Humans, Female, Male, Arrhythmogenic Right Ventricular Dysplasia, Myocardium, Arrhythmias, Cardiac, Prognosis, Echocardiography, Ventricular Function, Right
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Health Informatics
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Health Informatics > Clinical Epidemiology
URI: https://discovery.ucl.ac.uk/id/eprint/10184223
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