Zusammenfassung
Die angeborenen Ausflussbehinderungen des linken Ventrikels können im Bereich der Klappe durch Verwachsungen der Kommissuren (valvuläre Stenose, Abb. 1), unterhalb der Klappe (subvalvulär) und oberhalb der Klappe (supravalvulär) gelegen sein. Die subvalvulären Stenosen sind fibrotisch (Ringleistenstenose), muskulär (hypertrophe obstruktive Kardiomyopathie, HOCM) oder fibromuskulär bedingt. Bei der valvulären Stenose ist die Aortenklappe oft bikuspidal angelegt mit erhöhter Endokarditisgefahr. Begleitfehlbildungen können eine Aortenbogenhypoplasie, eine Aortenisthmusstenose oder eine Mitralstenose sein. Die angeborenen Ausflussbehinderungen des linken Ventrikels führen zu einem Druckgradienten zwischen dem linken Ventrikel und dem poststenotisch gelegenen Teil des linken Ventrikels bzw. der Aorta. Bei den subvalvulären und valvulären Stenosen entspringen die Koronararterien aus einem Bereich mit vermindertem Druck. Bei den supravalvulären Stenosen ist der Zufluss zu den Koronararterien durch Verwachsungen zwischen den Klappensegeln und der Aortenwand häufig eingeschränkt. Bei hochgradiger Stenose kann es, insbesondere bei körperlicher Belastung mit Abfall des aortalen Mitteldrucks, zu einer schweren Koronarinsuffizienz kommen.
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Breuer, J., Apitz, J., Schmaltz, A.A. (2016). Primär nichtzyanotische Vitien. In: Hoffmann, G., Lentze, M., Spranger, J., Zepp, F. (eds) Pädiatrie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54671-6_202-1
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Primär nichtzyanotische Vitien- Published:
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DOI: https://doi.org/10.1007/978-3-642-54671-6_202-2
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Primär nichtzyanotische Vitien- Published:
- 08 April 2016
DOI: https://doi.org/10.1007/978-3-642-54671-6_202-1