Arrhythmogene rechtsventrikul?re Kardiomyopathie

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• 作者：Prof. Dr. T. Wichter (1)
  M. Paul (2)
  
• 关键词：Ventrikul?re Tachykardie ; Pl?tzlicher Herztod ; Rechtsventrikul?re Dysplasie ; Diagnosekriterien ; Kardiale Bildgebung ; Ventricular tachycardia ; Sudden cardiac death ; Right ventricular dysplasia ; Diagnostic criteria ; Cardiac imaging
• 刊名：Der Kardiologe
• 出版年：2014
• 出版时间：February 2014
• 年：2014
• 卷：8
• 期：1
• 页码：85-98
• 全文大小：1,075 KB
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• 作者单位：Prof. Dr. T. Wichter (1)
  M. Paul (2)
  
  1. Klinik für Innere Medizin/Kardiologie, Herzzentrum Osnabrück/Bad Rothenfelde, Niels-Stensen-Kliniken, Marienhospital Osnabrück, Bischofsstr. 1, 49074, Osnabrück, Deutschland
  2. Klinik für Kardiologie, Department für Kardiologie und Angiologie, Universit?tsklinikum Münster, Münster, Deutschland
  
• ISSN：1864-9726

文摘

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary, genetically determined heart muscle disease and a major cause of ventricular tachyarrhythmia and sudden cardiac death in young, apparently healthy individuals and athletes. In ARVC, mutations in genes encoding for desmosomal cell contact proteins result in myocardial atrophy with subsequent fibrofatty replacement, predominantly affecting the right ventricular myocardium. Left ventricular involvement can also occur early in the disease process. The structural abnormalities lead to global and/or regional dysfunction of the right or both ventricles and to the dominant clinical manifestation with ventricular tachyarrhythmia. A primary manifestation of ARVC with clinically relevant signs and symptoms of heart failure is rare. Genetic counselling is indicated in all index patients with ARVC and their families. Genetic testing, however, is recommended only under particular circumstances (e.g. cascade screening). An early and correct diagnosis is crucial for risk stratification, treatment and prognosis of ARVC. The catalogue of diagnostic criteria (major and minor criteria) includes electrocardiogram (ECG) findings of depolarization and repolarization, arrhythmia, morphological and functional imaging, histopathology and genetics. These diagnostic criteria are currently under prospective validation in clinical studies and registries.