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Akute Herzinsuffizienz bei Ebstein-Anomalie und Linksherzhypertrophie

Acute heart failure with Ebstein’s anomaly and left ventricular hypertrophy

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Zusammenfassung

Wir berichten von einer 51-jährigen Patientin mit progredienter Dyspnoe. Echokardiographisch zeigte sich eine linksventrikuläre Hypertrophie mit hochgradig eingeschränkter linksventrikulärer Ejektionsfraktion. Darüber hinaus fiel eine zur Herzspitze hin verlagerte Trikuspidalklappe, vereinbar mit dem Vorliegen einer Ebstein-Anomalie, auf. In der kardiovaskulären Magnetresonanztomographie bestätigten sich die echokardiographischen Befunde einer hochgradig reduzierten Ejektionsfraktion und Verlagerung der Trikuspidalklappenebene, darüber hinaus ließen sich zahlreiche prominente ventrikuläre Trabekel mit tiefen intertrabekulären Recessus darstellen. Ein Anhalt für eine Myokarditis oder myokardiale Narbe fand sich nicht. Die Kombination der echokardiographischen und kernspintomographischen Befunde führte uns zu der Diagnose der Non-Compaction-Kardiomyopathie. Die Therapie der Non-Compaction-Kardiomyopathie ist nicht spezifisch, sondern entspricht der Behandlung der Herzinsuffizienz.

Abstract

We report on a case of a 51-year-old woman suffering from progressive dyspnoea. Echocardiography showed left ventricular hypertrophy and severely reduced left ventricular ejection fraction. In addition, a downward displacement of the tricuspid valve could be seen, as described in patients with Ebstein’s anomaly. Cardiovascular magnetic resonance imaging confirmed our echocardiographic findings of severely reduced left ventricular ejection fraction and downward displacement of the tricuspid valve. Furthermore, numerous prominent ventricular trabeculations and deep intertrabecular recesses were demonstrated. There was no evidence of myocarditis or myocardial scar. The combination of echocardiography and cardiovascular magnetic resonance imaging led us to the diagnosis of non-compaction cardiomyopathy. There is no specific treatment option for non-compaction cardiomyopathy; treatment includes all options available for the treatment of heart failure.

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Authors and Affiliations

  1. Medizinische Klinik II, Klinik für Kardiologie und Angiologie, Marienhospital Herne, Klinikum der Ruhr-Universität Bochum, Hölkeskampring 40, 44625, Herne, Deutschland

    M. Christ, M. van Bracht, T. Butz, G. Plehn, A. Meissner & H.-J. Trappe

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  1. M. Christ
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  2. M. van Bracht
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  3. T. Butz
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  4. G. Plehn
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  5. A. Meissner
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  6. H.-J. Trappe
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Correspondence to M. Christ.

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Christ, M., van Bracht, M., Butz, T. et al. Akute Herzinsuffizienz bei Ebstein-Anomalie und Linksherzhypertrophie. Kardiologe 3, 502–506 (2009). https://doi.org/10.1007/s12181-009-0162-9

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  • DOI: https://doi.org/10.1007/s12181-009-0162-9

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