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Observational Study
. 2025 Jun 30;26(7):1233-1241.
doi: 10.1093/ehjci/jeaf043.

CarDiac magnEtic Resonance for prophylactic Implantable cardioVerter defibrillAtor ThErapy in Non-Dilated Left Ventricular Cardiomyopathy: a sub-study from the DERIVATE registry

Isabella Leo  1   2 , Santo Dellegrottaglie  1 , Alessandra Scatteia  1 , Daniele Torella  2 , Raffaele Abete  3 , Giovanni Donato Aquaro  4 , Andrea Baggiano  5 , Andrea Barison  4 , Jan Bogaert  6 , Leonardo Calo'  7 , Giovanni Camastra  8 , Samuela Carigi  9 , Nazario Carrabba  10 , Grazia Casavecchia  11 , Stefano Censi  12 , Gloria Cicala  13 , Carlo N De Cecco  14 , Manuel De Lazzari  15 , Gabriella Di Giovine  3 , Monica Dobrovie  6 , Marta Focardi  16 , Laura Fusini  5   17 , Nicola Gaibazzi  18 , Annalaura Gismondi  16 , Matteo Gravina  19 , Marco Guglielmo  20   21 , Chiara Lanzillo  7 , Massimo Lombardi  22 , Valentina Lorenzoni  23 , Jordi Lozano-Torres  24   25   26 , Davide Margonato  3 , Chiara Martini  13   27 , Francesca Marzo  9 , Pier-Giorgio Masci  28 , Ambra Masi  29 , Claudio Moro  30 , Giuseppe Muscogiuri  31 , Saima Mushtaq  5 , Alberto Nese  32 , Alessandro Palumbo  13 , Anna Giulia Pavon  33 , Patrizia Pedrotti  29 , Martina Perazzolo Marra  15 , Silvia Pradella  34 , Cristina Presicci  13 , Mark G Rabbat  35   36 , Claudia Raineri  37 , Jose' F Rodriguez-Palomares  24   25   26 , Stefano Sbarbati  38 , Uwe Joseph Schoepf  39 , Angelo Squeri  12 , Nicola Sverzellati  27 , Rolf Symons  6 , Emily Tat  35 , Mauro Timpani  40 , Giancarlo Todiere  4 , Adele Valentini  41 , Akos Varga-Szemes  39 , Alessandra Volpe  5 , Andrea Igoren Guaricci  42 , Juerg Schwitter  31   43 , Gianluca Pontone  5   44
Affiliations
Observational Study

CarDiac magnEtic Resonance for prophylactic Implantable cardioVerter defibrillAtor ThErapy in Non-Dilated Left Ventricular Cardiomyopathy: a sub-study from the DERIVATE registry

Isabella Leo et al. Eur Heart J Cardiovasc Imaging. .

Abstract

Aims: Accurate risk stratification for patients with non-dilated left ventricular cardiomyopathy (NDLVC) remains challenging due to lack of dedicated clinical trials. This post hoc analysis aims to delineate the arrhythmic risk and assess the incremental value of cardiac magnetic resonance (CMR) imaging in the CarDiac magnEtic Resonance for prophylactic Implantable-cardioVerter defibrillAtor ThErapy (DERIVATE) study cohort meeting the NDLVC diagnostic criteria.

Methods and results: Patients with NDLVC from the DERIVATE registry were identified in the absence of left ventricular (LV) dilatation and in the presence of non-ischaemic LV scarring ('fibrotic NDLVC') or isolated LV systolic dysfunction (LV ejection fraction < 50%) without fibrosis ('hypokinetic NDLVC'). The primary endpoint was all-cause mortality. Major adverse arrhythmic cardiac events (MAACE) were the secondary endpoint and included sudden cardiac death (SCD) and aborted SCD. One hundred and ninety-seven NDLVC patients were identified from the cohort of the DERIVATE study (mean age: 59 ± 14 years; male: 135). Over a median follow-up of 2.7 years, 15 (8%) patients died and 8 (4%) experienced MAACE. Patients with 'hypokinetic' NDLVC had significantly lower rates of MAACE than non-ischaemic dilated cardiomyopathy (NIDCM) (P = 0.001), while patients with 'fibrotic' NDLVC had same rate of both primary (P = 0.48) and secondary endpoints (P = 0.616) compared with NIDCM patients. Multivariable analysis identified late gadolinium enhancement (LGE) with midwall distribution as an independent predictor of MAACE in NDLVC patients (hazard ratio 6.7, 95% confidence interval: 1.33-33.67; P = 0.021).

Conclusion: NDLVC patients exhibit a heterogeneous risk profile for arrhythmic events. The presence of midwall LGE, similarly to NIDCM, is a significant predictor of MAACE, highlighting the importance of CMR imaging for risk stratification.

Keywords: cardiac magnetic resonance; dilated cardiomyopathy; non-dilated left ventricular cardiomyopathy; sudden cardiac death.

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Conflict of interest statement

Conflict of interest: G.P. received honorarium as speaker and/or research funding from GE Healthcare, Bracco, HeartFlow, Menarini, Novartis, and Alexion. J.S. receives research support by Bayer Healthcare, Schweiz, AG. L.F. received speaker honoraria from Philips.

Figures

Graphical Abstract
Graphical Abstract
Risk of major adverse arrhythmic cardiac events in patients with dilated and non-dilated left ventricular cardiomyopathy. DCM, dilated cardiomyopathy; MAACE, major adverse arrhythmic cardiac event; NDLVC, non-dilated left ventricular cardiomyopathy.
Figure 1
Figure 1
Kaplan–Meier curves for secondary endpoints in DCM and NDLVC patients (A) and in NDLVC with and without LGE (B). Kaplan–Meier curves for secondary endpoints in DCM and NDLVC LGE-negative (C) and LGE-positive (D) patients. DCM, dilated cardiomyopathy; LGE, late gadolinium enhancement; MAACE, major adverse arrhythmic cardiac event; NDLVC, non-dilated left ventricular cardiomyopathy.
See this image and copyright information in PMC

Comment in

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