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Risk stratification in hypertrophic cardiomyopathy

Risikostratifizierung bei hypertropher Kardiomyopathie

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Abstract

Sudden cardiac death (SCD) is the most devastating complication of hypertrophic cardiomyopathy (HCM). The greatest challenge in the management of HCM is identifying those at increased risk, since an implantable cardioverter-defibrillator (ICD) is a potentially life-saving therapy. We sought to summarize the available data on SCD in HCM and provide a clinical perspective on the current differing and somewhat conflicting data on risk stratification, with balanced guidance regarding rational clinical decision-making. Additionally, we sought to determine the status of the current implementation of guidelines compiled by HCM experts worldwide. The HCM Risk-SCD model helps improve the risk stratification of HCM patients for primary prevention of SCD by calculating an individual risk estimate that contributes to the clinical decision-making process. Improved risk stratification is important for decision-making before ICD implantation for the primary prevention of SCD.

Zusammenfassung

Der plötzliche Herztod („sudden cardiac death“, SCD) stellt die schwerwiegendste Komplikation der hypertrophen Kardiomyopathie (HCM) dar. Die größte Herausforderung in der Behandlung der HCM ist es, gefährdete Patienten zu erkennen, denn ein implantierbarer Defibrillator („implantable cardiac defibrillator“, ICD) ist ein potenziell lebensrettender Therapieansatz. Ziel der Autoren war es, die verfügbaren Daten zum SCD bei HCM zusammenzufassen und die aktuellen unterschiedlichen und sich teils widersprechenden Daten zur Risikostratifizierung aus klinischer Sicht mit einer ausgewogenen Anleitung zur rationalen klinischen Entscheidungsfindung zu erörtern. Darüber hinaus war es das Ziel, den aktuellen Stand der Etablierung der durch HCM-Spezialisten erstellten Leitlinien zu bestimmen. Das HCM Risk-SCD Modell trägt dazu bei, die Risikostratifizierung von HCM-Patienten zur Primärprävention eines SCD durch Berechnung eines individuellen Risikoschätzwerts zu verbessern, welcher einen Beitrag zum klinischen Entscheidungsfindungsprozess leistet. Eine verbesserte Risikostratifizierung ist für die Entscheidungsfindung vor einer ICD-Implantation zur Primärprävention des SCD von großer Bedeutung.

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Authors and Affiliations

  1. Department of Cardiology, Abderrahmane Mami Hospital, Ariana, Tunisia

    S. Marrakchi, I. Kammoun, E. Bennour, L. Laroussi & S. Kachboura

  2. El Manar University, 8, Rue Zohra Touati El Manar 1, Tunis, 2092, Tunisia

    S. Marrakchi, I. Kammoun, E. Bennour, L. Laroussi & S. Kachboura

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  2. I. Kammoun
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S. Marrakchi, I. Kammoun, E. Bennour, L. Laroussi and S. Kachboura declare that they have no competing interests.

This article does not contain any studies with human participants or animals performed by any of the authors.

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Marrakchi, S., Kammoun, I., Bennour, E. et al. Risk stratification in hypertrophic cardiomyopathy. Herz 45, 50–64 (2020). https://doi.org/10.1007/s00059-018-4700-8

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