Future Development of Arrhythmogenic Risk Scores in Patients with Heart Failure and Inherited Dilated Cardiomyopathy. A Scientific Statement of the Heart Failure Association of the ESC

The risk of sudden cardiac death (SCD) in the general population of patients with dilated cardiomyopathy (DCM) has progressively declined with the implementation of novel medical strategies. However, still cases occur in young individuals and the challenge of risk stratification remains unsolved. Traditional criteria, including left ventricular ejection fraction, have demonstrated their profound weakness to identify subjects at high risk of SCD in this specific context. The increasing availability of genetic information has allowed identification of certain genotypes with a high arrhythmic risk that deserve a more individualized approach. Recent European guidelines recognized the contribution of genetic information in clinical decision-making. Gene-specific risk stratification tools have been developed, and in some cases externally validated, which can support clinicians in the decisions on SCD primary prevention interventions. However, they are generally based on basic variables, whereas the growing amount of knowledge on novel methods for risk prediction, and in particular the solid data on the predictive value of cardiac magnetic resonance tissue characterization (i.e. late gadolinium enhancement) are not incorporated in available scores, and more in general, are not systematically part of the clinical work-up. In this scientific statement, we summarized the current state of the art concerning the risk stratification of SCD in DCM, with particular emphasis on genetic forms, highlight the weaknesses of the available strategies and the potential actions needed for improving them. Available risk stratification tools are discussed, and methodologies that should be incorporated in future prognostication models are summarized. Lastly, a point-by-point summary of the key prerequisites for developing the future arrhythmogenic risk scores in patients with DCM is provided.

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Publication Details

Journal
European Journal of Heart Failure
Published
2025-09-24
DOI
https://doi.org/10.1002/ejhf.70042
Citations
1
Primary Topic
Cardiomyopathy and Myosin Studies
Type
article
Field-Weighted Citation Impact
1.39
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article

Future Development of Arrhythmogenic Risk Scores in Patients with Heart Failure and Inherited Dilated Cardiomyopathy. A Scientific Statement of the Heart Failure Association of the ESC

Job A.J. Verdonschot, Dana Dawson, Lorenzo Monserrat, Marta Gigli et al.
1 citations
European Journal of Heart Failure
Cardiomyopathy and Myosin Studies
1.39
article

Future Development of Arrhythmogenic Risk Scores in Patients with Heart Failure and Inherited Dilated Cardiomyopathy. A Scientific Statement of the Heart Failure Association of the ESC

Job A.J. Verdonschot, Dana Dawson, Lorenzo Monserrat, Marta Gigli, Antonios Pantazis, Andrea Mazzanti, Brian P. Halliday, Upasana Tayal, Folkert W. Asselbergs, Barbara Bauce, Pablo García‐Pavía, Sanjay Prasad, Stéphane Heymans, Maite Tome, Luisa Mestroni, J. Peter van Tintelen, Philippe Charron, Gianfranco Sinagra, Petar Seferović, Marco Metra
article en
1 citations

Abstract

The risk of sudden cardiac death (SCD) in the general population of patients with dilated cardiomyopathy (DCM) has progressively declined with the implementation of novel medical strategies. However, still cases occur in young individuals and the challenge of risk stratification remains unsolved. Traditional criteria, including left ventricular ejection fraction, have demonstrated their profound weakness to identify subjects at high risk of SCD in this specific context. The increasing availability of genetic information has allowed identification of certain genotypes with a high arrhythmic risk that deserve a more individualized approach. Recent European guidelines recognized the contribution of genetic information in clinical decision-making. Gene-specific risk stratification tools have been developed, and in some cases externally validated, which can support clinicians in the decisions on SCD primary prevention interventions. However, they are generally based on basic variables, whereas the growing amount of knowledge on novel methods for risk prediction, and in particular the solid data on the predictive value of cardiac magnetic resonance tissue characterization (i.e. late gadolinium enhancement) are not incorporated in available scores, and more in general, are not systematically part of the clinical work-up. In this scientific statement, we summarized the current state of the art concerning the risk stratification of SCD in DCM, with particular emphasis on genetic forms, highlight the weaknesses of the available strategies and the potential actions needed for improving them. Available risk stratification tools are discussed, and methodologies that should be incorporated in future prognostication models are summarized. Lastly, a point-by-point summary of the key prerequisites for developing the future arrhythmogenic risk scores in patients with DCM is provided.

European Journal of Heart FailureVol. 27(11)
Serbian Academy of Sciences and Arts (RS), University of Padua (IT), Inserm (FR), Utrecht University (NL), University of Aberdeen (GB), Guy's and St Thomas' NHS Foundation Trust (GB), University of Pavia (IT), Royal Brompton & Harefield NHS Foundation Trust (GB), Maastricht University Medical Centre (NL), Aberdeen Royal Infirmary (GB), St George’s University Hospitals NHS Foundation Trust (GB), Harefield Hospital (GB), Spanish National Centre for Cardiovascular Research (ES), University Medical Center Utrecht (NL), Maastricht University (NL), National Institute for Health and Care Research (GB), Sorbonne Université (FR), University of Belgrade (RS), Istituti Clinici Scientifici Maugeri (IT), Stockholm South General Hospital (SE), Assistance Publique – Hôpitaux de Paris (FR), Grade Medical (Czechia) (CZ), Lung Institute (US), Surgical Specialties (Canada) (CA), Hospital Universitario Puerta de Hierro Majadahonda (ES), Medical Solutions (GB), ERN GUARD-Heart (NL), Fondation pour l’innovation en Cadiométabolisme et Nutrition (FR), Azienda Sanitaria Universitaria Integrata di Trieste (IT), University College London (GB), Imperial College London (GB), University of Colorado Anschutz Medical Campus (US), University of Brescia (IT), University of Amsterdam (NL)
Openalex Percentile: Top 15%
Cardiomyopathy and Myosin Studies
1.39
DOISourceOpenAlex
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