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Die isolierte Noncompaction Kardiomyopathie (NCCM) ist eine seltene, genetisch determinierte Herzmuskelerkrankung, bei der der Remodellingprozess des linksventrikulären (LV) Myokards während der Embryonalzeit gestört ist, sodass in den betroffenen Abschnitten der LV-Wand eine schmale kompakte und eine dickere nicht kompakte Myokardschicht verbleiben. Die Genetik der NCCM ist dabei durch eine große Heterogenität charakterisiert. Die Diagnostik der NCCM kann durch Echokardiographie oder kardiale Magnetresonanztomographie erfolgen, wobei die Diagnosekriterien immer noch einer Diskussion unterliegen. Die betroffenen Patienten weisen ein breites Spektrum an Befunden und Symptomen auf: Arrhythmien, Herzinsuffizienz und kardioembolische Ereignisse. In der Risikostratifikation erwiesen sich neben der schweren LV-Funktionsstörung ein kompletter Linksschenkelblock und Vorhofflimmern als signifikante Risikomarker für schwere klinische Ereignisse. Die Therapie der NCCM erfolgt individuell und orientiert sich an den Symptomen und Untersuchungsbefunden. Sie umfasst eine medikamentöse Behandlung, im Bedarfsfall auch Ablationsbehandlungen oder gerätebasierte Therapien und bei schwerster Herzinsuffizienz eine Listung zur Herztransplantation. Bei der Betreuung von Patienten mit NCCM sollte neben der regelmäßigen kardiologischen Kontrolle auch ein Familienscreening mit Erhebung der Anamnese, körperlicher Untersuchung, EKG-Registrierung und Echokardiographie durchgeführt werden.
Herzschrittmachertherapie + Elektrophysiologie – Springer Journals
Published: Sep 25, 2012
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