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Personalized sudden cardiac death risk prediction in genetic heart diseases: Beyond one-size-fits-all

  • University of Amsterdam
  • Amsterdam UMC
  • European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN GUARDHEART)

Research output: Contribution to journalReview articleAcademicpeer-review

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Abstract

Sudden cardiac death (SCD) risk prediction in genetic heart diseases is essential to identify patients who will benefit from implantable cardioverter-defibrillator (ICD) implantation. Although many prediction tools have been developed, risk prediction remains challenging due to variability in underlying arrhythmic substrates and statistical modeling approaches. This review addresses 2 major challenges in current clinical practice. First, the use of surrogate SCD end points, such as appropriate ICD therapy, can potentially and actually does lead to overestimation of the “true” SCD risk. This may result in unnecessary ICD implantation in low-risk patients and exposing them to device-related complications. Second, most risk models are static and do not account for temporal changes in risk. We provide an overview of SCD prediction models and offer recommendations to address these challenges. This review underscores the need for disease-specific surrogate end points and dynamic risk models that reflect individual risk over time.
Original languageEnglish
Pages (from-to)e62-e74
JournalHeart rhythm
Volume23
Issue number1
Early online date2025
DOIs
Publication statusPublished - 1 Jan 2026

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

  1. SDG 3 - Good Health and Well-being
    SDG 3 Good Health and Well-being

Keywords

  • Genetic heart diseases
  • ICD implantation
  • Risk prediction tools
  • Sudden cardiac death
  • Surrogate SCD end points

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