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Grunds\u00e4tzlich kann im Kontext genetischer Erkrankungen eine Unterscheidung zwischen Kanalopathien und Kardiomyopathien getroffen werden. Schwerpunkt der vorliegenden Arbeit sind die Kanalopathien Long- und Short-QT-Syndrom, Brugada-Syndrom sowie die katecholaminerge polymorphe ventrikul\u00e4re Tachykardie (CPVT). Eine fr\u00fchzeitige Diagnose dieser Erkrankungen ist unerl\u00e4sslich, lassen sich doch durch die medikament\u00f6se Therapie, die Aufkl\u00e4rung \u00fcber Verhaltensma\u00dfnahmen und gegebenenfalls die Implantation eines Kardioverter-Defibrillators die Prognose und Lebensqualit\u00e4t der Patienten signifikant verbessern. Der Beitrag beleuchtet die pathophysiologischen und genetischen Grundlagen dieser Kanalopathien, beschreibt deren klinische Manifestation und kommentiert die Grundlagen f\u00fcr Diagnose, Risikostratifikation und Therapie.<\/jats:p>","DOI":"10.1007\/s00108-024-01751-x","type":"journal-article","created":{"date-parts":[[2024,7,8]],"date-time":"2024-07-08T18:02:46Z","timestamp":1720461766000},"page":"787-797","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Kardiale Kanalopathien im\u00a0Kontext heredit\u00e4rer Arrhythmiesyndrome","Cardiac channelopathies in the context of hereditary arrhythmia syndromes"],"prefix":"10.1007","volume":"65","author":[{"given":"Moritz T.","family":"Huttelmaier","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas H.","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,7,8]]},"reference":[{"issue":"40","key":"1751_CR1","doi-asserted-by":"publisher","first-page":"3997","DOI":"10.1093\/eurheartj\/ehac262","volume":"43","author":"K Zeppenfeld","year":"2022","unstructured":"Zeppenfeld\u00a0K, Tfelt-Hansen\u00a0J, de Riva\u00a0M, Winkel\u00a0BG, Behr\u00a0ER, Blom\u00a0NA et\u00a0al (2022) 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. 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