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Observational Study
. 2025 Sep 30;26(10):1609-1619.
doi: 10.1093/ehjci/jeaf198.

Redefining the risk of major arrhythmic events in non-ischaemic cardiomyopathy: insights from the DERIVATE-NICM study

Andrea Igoren Guaricci  1 , Nazario Carrabba  2 , Salvatore Mario Romano  3 , Marco Chiostri  4 , Laura Fusini  5   6 , Andrea Baggiano  5   7 , Saima Mushtaq  5 , Alessandra Volpe  5 , Raffaele Abete  8 , Giovanni Donato Aquaro  9 , Andrea Barison  9 , Paolo Basile  1 , Jan Bogaert  10 , Leonardo Calo'  11 , Giovanni Camastra  12 , Samuela Carigi  13 , Grazia Casavecchia  14 , Stefano Censi  15 , Gloria Cicala  16 , Marco Matteo Ciccone  1 , Carlo N De Cecco  17 , Manuel De Lazzari  18 , Gabriella Di Giovine  8 , Monica Dobrovie  10 , Marta Focardi  19 , Nicola Gaibazzi  20 , Annalaura Gismondi  19 , Matteo Gravina  21 , Marco Guglielmo  22 , Chiara Lanzillo  11 , Massimo Lombardi  23 , Valentina Lorenzoni  24 , Jordi Lozano-Torres  25 , Davide Margonato  8 , Chiara Martini  16 , Francesca Marzo  13 , Pier-Giorgio Masci  26 , Ambra Masi  27 , Claudio Moro  28 , Giuseppe Muscogiuri  5 , Alberto Nese  5 , Alessandro Palumbo  16 , Anna Giulia Pavon  29 , Patrizia Pedrotti  27 , Martina Perazzolo Marra  18 , Silvia Pradella  30 , Cristina Presicci  16 , Mark G Rabbat  31 , Claudia Raineri  32 , Jose' F Rodriguez-Palomares  25 , Guillem Casas  25 , Eduardo Rodenas-Alesina  25 , Alessandro Giustiniani  25 , Stefano Sbarbati  33 , U Joseph Schoepf  34 , Angelo Squeri  15 , Nicola Sverzellati  16 , Rolf Symons  10 , Emily Tat  31 , Mauro Timpani  5 , Giancarlo Todiere  9 , Adele Valentini  35 , Akos Varga-Szemes  34 , Juerg Schwitter  29   36 , Gianluca Pontone  5   37
Affiliations
Observational Study

Redefining the risk of major arrhythmic events in non-ischaemic cardiomyopathy: insights from the DERIVATE-NICM study

Andrea Igoren Guaricci et al. Eur Heart J Cardiovasc Imaging. .

Abstract

Aims: Selection of the patients for implantable cardioverter defibrillator primary prevention therapy in non-ischaemic cardiomyopathy (NICM) needs to be improved. To evaluate the additional prognostic value of a new cardiac magnetic resonance (CMR) score based on late gadolinium enhancement (LGE) pattern distribution (DERIVATE Risk Score 2.0) when compared with previously published DERIVATE Risk Score 1.0, which is based solely on quantitative parameters, in a cohort of NICM patients enrolled in the DERIVATE registry.

Methods and results: One thousand three hundred and eighty-four NICM patients with chronic heart failure and left ventricular ejection fraction (LVEF) < 50% were evaluated for primary sudden cardiac death prevention therapy. Major adverse arrhythmic cardiac events (MAACEs) were the primary endpoint. During a median follow-up of 959 days, MAACE occurred in 128 (9.2%) patients. In the multivariate analyses, male gender [hazard ratio (HR): 1.605 (95% confidence interval, CI: 1.051-2.451); P = 0.028], LVEF per point % [HR: 0.977 (95% CI: 0.961-0.993); P = 0.005] and presence and location of midwall LGE [weighted HR: 1.066 (95% CI: 1.045-1.086), P < 0.001] were independent predictors of MAACE. A multi-parametric CMR-weighted predictive-derived score (DERIVATE Risk Score 2.0) provided a higher additional prognostic value vs. transthoracic echocardiography-LVEF cut-off of 35% when compared with the previous published DERIVATE Risk Score 1.0 with a net reclassification improvement of 54.52% (95% CI: 36.52-72.52%; P < 0.001). These findings were confirmed in the validation cohort.

Conclusion: The presence of midwall LGE, but also the location of scar, confers an added and independent MAACE risk to a large NICM population influencing the choice of treatment.

Keywords: cardiac magnetic resonance; heart failure; implantable cardioverter defibrillator therapy; non-ischaemic dilated cardiomyopathy.

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Conflict of interest statement

Conflict of interest: C.N.D.C. received a grant from Siemens and serves as Associate Editor, EHJ CVI. G.P. received institutional fees from General Electric, Bracco, Heartflow, Medtronic, Bayer, Bhoeringher, and serves as Senior Associate Editor, EHJ CVI. J.S. received research support from Bayer Healthcare Switzerland. U.J.S. received a grant from Astellas, Bayer, General Electric, and Siemens Healthcare, personal fees from Guerbet, speaking honorarium from Heartflow. A.V.-S. received a grant from Siemens Healthcare and personal fees from Elucid Bioimaging. The other authors have nothing to disclose.

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