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„Non-compaction“-Kardiomyopathie

Noncompaction cardiomyopathy

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Zusammenfassung

Die „Non-compaction“-Kardiomyopathie (NCCM) ist eine genetische Erkrankung des Myokards. Sie wird durch eine 2-schichtige Ventrikelwand mit einer epikardialen dünnen kompakten Schicht und einer inneren dicken nichtkompakten Schicht mit prominenten Trabekeln und intratrabekulärem tiefen Rezessus, die mit der inneren Wand des Ventrikels, nicht aber mit dem Koronarsystem kommunizieren, charakterisiert. Vor der ersten Beschreibung als isolierte linkventrikuläre Kardiomyopathie (ILVNC) im Jahre 1984 von Engberding und Bender wurden die morphologischen Eigenschaften der NCCM nur als Begleitmerkmal anderer angeborener Herzerkrankungen wie Atresie von Semilunarklappen angesehen. Die Krankheit betrifft zwar in der Regel den linken Ventrikel, aber die Zahl der berichteten Fälle über Beteiligung des rechten Ventrikels ist gestiegen. Die NCCM kann ohne rechtzeitige Diagnose und Therapie im fortgeschrittenen Stadium zur Herzinsuffizienz führen. Lebensbedrohliche Komplikationen wie maligne Arrhythmien mit plötzlichem Herztod und Embolien sind bei Patienten mit NCCM bekannt. Eine multimodale Diagnostik einschließlich der Echokardiographie und der kardialen Magnetresonanztomographie (CMR) sowie einer fokussierten Analyse der Symptome und des EKG kann zu einer gültigen Diagnosestellung beitragen.

Abstract

Noncompaction cardiomyopathy (NCCM) is a genetic myocardial disorder, which is characterized by a two-layered ventricle wall with a thin compact outer layer and a noncompacted inner layer, with prominent trabeculations and deep intratrabecular recesses communicating with the ventricle cavity without any contact to the coronary system. Before the initial description as isolated left ventricle cardiomyopathy (ILVCN) in 1984 by Engberding and Bender, the morphological characteristics had been described only in association with other congenital cardiac disorders, such as atresia of the semilunar valves. The disease usually involves the myocardium of the left ventricle but involvement of the right ventricular has recently been shown. Due to delayed diagnosis and therapy, in advanced stages NCCM can result in heart failure. Life-threatening complications, such as malignant arrhythmia with sudden cardiac death and embolic events have been observed in patients with NCCM. A multimodal investigation including echocardiography and cardiac magnet resonance tomography (CMR) as well as a focused analysis of symptoms can allow a valid diagnosis.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. J. Shariati, T. Schlosser und R. Erbel geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Westdeutsches Herz- und Gefäßzentrum, Klinik für Kardiologie, Universitätsklinikum Duisburg-Essen, Hufelandstr. 55, 45122, Essen, Dautschland

    J. Shariati & R. Erbel

  2. Institut für Diagnostische und Interventionelle Radiologie und Neuroradiologie, Universitätsklinikum Duisburg-Essen, Essen, Deutschland

    T. Schlosser

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  1. J. Shariati
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Correspondence to J. Shariati.

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Shariati, J., Schlosser, T. & Erbel, R. „Non-compaction“-Kardiomyopathie. Herz 40, 583–590 (2015). https://doi.org/10.1007/s00059-015-4233-3

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