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Long-term Prognosis and Management of Noncompaction Cardiomyopathy

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Noncompaction Cardiomyopathy

Abstract

Currently NCCM is viewed as a primary cardiomyopathy, with mostly autosomal dominant inheritance pattern [1]. The clinical presentation varies from asymptomatic to supra-ventricular arrhythmias, severe heart failure (HF), malignant ventricular arrhythmias, and thromboembolic events [2, 3]. Since the first report by Engberding and Bender in 1984 of a case of a 33 years old women with heart failure and palpitations and later in 1990 case series of 8 patients by Chin et al., there is increasing amounts of publications and more awareness among clinicians. But the majority of these are small series of cases or solely case reports with high incidence of adverse cardiac events, probably reflecting the biased patient selection in tertiary referral centers. Therefore, the long-term prognosis remains conflicting and inconsistent. In this chapter, we report our single center experience with excellent long-term survival in patients without heart failure in the modern heart failure treatment area with individualized drug treatment and, in selected patients, ICD implantation for primary or secondary prophylaxes of sudden cardiac death [4, 5]. We also provide an overview of the most up-to-date prognosis studies.

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Authors and Affiliations

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

    Emrah Kaya & Martijn Otten

  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. Emrah Kaya
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  2. Martijn Otten
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  3. Kadir Caliskan
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Correspondence to Kadir Caliskan .

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Editors and Affiliations

  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., Caliskan, K. (2019). Long-term Prognosis and Management of Noncompaction Cardiomyopathy. In: Caliskan, K., Soliman, O., ten Cate, F. (eds) Noncompaction Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-030-17720-1_9

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  • DOI: https://doi.org/10.1007/978-3-030-17720-1_9

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