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Kardiologie up2date 2020; 16(03): 259-272
DOI: 10.1055/a-1057-0680
Entzündliche Herzerkrankungen und Kardiomyopathien

Linksventrikuläre Non-Compaction-Kardiomyopathie

Nicolas Verheyen
,
Herbert Juch
,
Klemens Ablasser
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Die linksventrikuläre Non-Compaction-Kardiomyopathie ist eine genetische Erkrankung des Myokards, die durch eine auffällige Hypertrabekularisierung gekennzeichnet ist. Im Einzelfall ist die Abgrenzung zur Hypertrabekularisierung bei anderen Herzerkrankungen schwierig. Dieser Beitrag gibt einen Überblick über die multimodale Diagnostik einschließlich Echokardiografie, kardialer Magnetresonanztomografie sowie Genetik, die zur Diagnosestellung beitragen kann.
Kernaussagen

  • Die Magnetresonanztomografie ist sehr häufig falsch-positiv – bis zu ein Viertel aller Gesunden erfüllen hier die Bildgebungskriterien für linksventrikuläre Nichtkompaktierung (LVNC).

  • Die LVNC-Kardiomyopathie ist definiert durch linksventrikuläre Nichtkompaktierung einhergehend mit klinisch manifester Herzinsuffizienz, die nicht anderweitig erklärbar ist.

  • Eine linksventrikuläre Nichtkompaktierung kann bei allen spezifischen Kardiomyopathien als morphologische Variante auftreten.

  • Eine genetische Definition der LVNC-Kardiomyopathie ist derzeit nicht möglich, weil keine reproduzierbaren Genotyp-Phänotyp-Korrelationen beschrieben sind.

  • Zur vollständigen Diagnostik der LVNC-Kardiomyopathie gehört im Hinblick auf die mögliche weitere Abklärung und Therapie von Familienmitgliedern eine genetische Abklärung.

  • Es gibt keine evidenzbasierte spezifische Therapie für die LVNC-Kardiomyopathie, sondern es wird eine allgemeine Herzinsuffizienz-Therapie entsprechend internationaler Richtlinien empfohlen.

  • Expert*innen empfehlen bei Vorliegen einer LVNC-Kardiomyopathie bei einer linksventrikulären Ejektionsfraktion < 40% oder bei Vorhofflimmerarrhythmie, unabhängig vom CHA2DS2-Score, eine Antikoagulation zur Schlaganfallprophylaxe.

Schlüsselwörter

Kardiomyopathie - Hypertrabekularisierung - Genetik - Echokardiografie - Therapie


Publication History

Article published online:
10 September 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Stuttgart · New York

 

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