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Sudden cardiac death in cardiomyopathies: acting upon “acceptable” risk in the personalized medicine era

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Abstract

Patients with cardiomyopathies are confronted with the risk of sudden cardiac death (SCD) throughout their lifetime. Despite the fact that SCD is relatively rare, prognostic stratification is an integral part of physician–patient discussion, with the goal of risk modification and prevention. The current approach is based on a concept of “acceptable risk.” However, there are intrinsic problems with an algorithm-based approach to risk management, magnified by the absence of robust evidence underlying clinical decision support tools, which can make high- versus low-risk classifications arbitrary. Strategies aimed at risk reduction range from selecting patients for an implantable cardioverter defibrillator (ICD) to disqualification from competitive sports. These clinical options, especially when implying the use of finite financial resources, are often delivered from the physician’s perspective citing decision-making algorithms. When the burden of intervention-related risks or financial costs is deemed higher than an “acceptable risk” of SCD, the patient’s perspective may not be appropriately considered. Designating a numeric threshold of “acceptable risk” has ethical implications. One could reasonably ask “acceptable to whom?” In an era when individual choice and autonomy are pillars of the physician–patient relationship, the subjective aspects of perceived risk should be acknowledged and be part of shared decision-making. This is particularly true when the lack of a strong scientific evidence base makes a dichotomous algorithm-driven approach suboptimal for unmitigated translation to clinical practice.

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Funding

IO is supported by the European Union’s Horizon 2020 Research and Innovation Programme under Grant Agreement no. 777204: “SILICOFCM — In Silico trials for drug tracing the effects of sarcomeric protein mutations leading to familial cardiomyopathy”; by the Italian Ministry of Health (Left ventricular hypertrophy in aortic valve disease and hypertrophic cardiomyopathy: genetic basis, biophysical correlates and viral therapy models (RF-2013–02356787)) and NET-2011–02347173 (Mechanisms and treatment of coronary microvascular dysfunction in patients with genetic or secondary left ventricular hypertrophy); and by the Ente Cassa di Risparmio di Firenze (bando 2016) “juvenile sudden cardiac death: just know and treat.” GF is supported by the charity Cardiac Risk in the Young (CRY).

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  1. Gherardo Finocchiaro and Emma F. Magavern equally contributed as first author

Authors and Affiliations

  1. Cardiothoracic Centre, Guy’s and St Thomas’ Hospital, London, UK

    Gherardo Finocchiaro & Gerald Carr-White

  2. King’s College London, London, UK

    Gherardo Finocchiaro, Georgios Georgioupoulos & Gerald Carr-White

  3. Royal Brompton Hospital, Sydney St, London, SW3 6NP, UK

    Gherardo Finocchiaro & Antonis Pantazis

  4. Cardiovascular Clinical Academic Group, St George’s, University of London, London, UK

    Gherardo Finocchiaro

  5. The London School of Medicine and Dentistry, William Harvey Research Institute, Barts, London, UK

    Emma F. Magavern

  6. Department of Clinical Pharmacology, Cardiovascular Medicine, Barts Health NHS Trust, London, UK

    Emma F. Magavern

  7. Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy

    Niccolo’ Maurizi & Iacopo Olivotto

  8. Cardiovascular Department, Azienda Sanitaria Giuliano Isontina (ASUGI), University of Trieste, Trieste, Italy

    Gianfranco Sinagra

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Finocchiaro, G., Magavern, E.F., Georgioupoulos, G. et al. Sudden cardiac death in cardiomyopathies: acting upon “acceptable” risk in the personalized medicine era. Heart Fail Rev 27, 1749–1759 (2022). https://doi.org/10.1007/s10741-021-10198-3

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