Abstract
Left ventricular noncompaction (LNVC), first described in 1984, is a rare congenital cardiomyopathy that is thought to be caused by arrest of normal embryogenesis of the endocardium and the myocardium, and characterized by multiple prominent trabeculations with deep intertrabecular recesses. LVNC can be associated with other congenital cardiac abnormalities such as atrial septal defect and ventricular septal defect, but it can occur in isolation. The clinical manifestations of the disease are variable, ranging from no symptoms to signs of heart failure, systemic emboli, and ventricular arrhythmias. The diagnosis is established by two dimensional echocardiography or magnetic resonance imaging. We report the case of a 44-year-old man with LVNC and discuss the role of multimodal imaging in the diagnosis and assessment of the disease.
Zusammenfassung
Die linksventrikuläre Noncompaction-Kardiomyopathie (LVNC) ist eine seltene kongenitale Erkrankung, die erstmals 1984 beschrieben wurde. Man nimmt an, dass ihr eine Unterbrechung der normalen Embryonalentwicklung des Endokards und Myokards zugrunde liegt. Charakteristisch sind multiple prominente Trabekel mit tiefen intertrabekulären Recessus. Die LVNC kann mit anderen kongenitalen Herzfehlern wie Vorhof- und Ventrikelseptumdefekten vergesellschaftet sein oder auch isoliert auftreten. Klinisch manifestiert sie sich uneinheitlich: Das Bild reicht von Symptomfreiheit bis zu Zeichen einer Herzinsuffizienz, systemischen Emboli und ventrikulären Herzrhythmusstörungen. Die Diagnose wird mithilfe der 2-dimensionalen Echokardiographie und Magnetresonanztomographie gestellt. In der vorliegenden Kasuistik berichten wir über einen 44-jährigen Patienten mit LVNC. Die Rolle, die die multimodale Bildgebung in der Diagnose und Beurteilung der Erkrankung spielt, wird erörtert.
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Bouhzam, N., Kurtz, B., Cribier, A. et al. Multimodal imaging in the diagnosis of isolated left ventricular noncompaction. Herz 37, 804–808 (2012). https://doi.org/10.1007/s00059-012-3647-4
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DOI: https://doi.org/10.1007/s00059-012-3647-4