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Malignant Arrhythmias and Sudden Cardiac Death in Patients with Noncompaction Cardiomyopathy: Prevalence, Prevention, and Use of Implantable Cardiac Defibrillators

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Noncompaction Cardiomyopathy

Abstract

Noncompaction cardiomyopathy (NCCM) was first described almost a half century ago, it initially began as an unusual autopsy finding. NCCM has a highly variable clinical presentation and is usually diagnosed when the condition becomes symptomatic or when complications occur in patients. These complications are end-stage heart failure, lethal arrhythmias and thromboembolic events [8–10]. Sudden cardiac death (SCD) is its most striking and visible consequence. The mortality in patients with NCCM has been reported in 18% of adults and 0–13% in children. Most SCD within the NCCM population are due to arrhythmias caused by ventricular tachycardia (VTs) and ventricular fibrillation. The implantable cardioverter defibrillator (ICD) is the single most effective therapy to prevent SCD. However, appropriate risk stratification in these patients are  not yet established. 

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Authors and Affiliations

  1. Thoraxcenter, Department of Cardiology, Erasmus MC University Medical Center, Rotterdam, The Netherlands

    Emrah Kaya, Martijn Otten, Sing-Chien Yap & Tamas Szili-Torok

  2. Department of Cardiology, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands

    Emrah Kaya

  3. Erasmus MC University Medical Center, Department of Cardiology, Rotterdam, The Netherlands

    Kadir Caliskan

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  1. Erasmus MC University Medical Center, Department of Cardiology, Rotterdam, The Netherlands

    Kadir Caliskan

  2. Erasmus MC University Medical Center, The Thoraxcenter, Rotterdam, The Netherlands

    Osama I. Soliman

  3. Erasmus MC University Medical Center, Department of Cardiology, Rotterdam, The Netherlands

    Folkert J. ten Cate

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Kaya, E., Otten, M., Yap, SC., Szili-Torok, T., Caliskan, K. (2019). Malignant Arrhythmias and Sudden Cardiac Death in Patients with Noncompaction Cardiomyopathy: Prevalence, Prevention, and Use of Implantable Cardiac Defibrillators. In: Caliskan, K., Soliman, O., ten Cate, F. (eds) Noncompaction Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-030-17720-1_5

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