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Supravalvuläre Aortenstenose: Morphologie der Aortenklappe und deren Bedeutung für die chirurgische Therapie

Supravalvular aortic stenosis: Morphology and surgical importance of the aortic valve

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Zeitschrift für Herz-,Thorax- und Gefäßchirurgie Aims and scope

Zusammenfassung

Ein großer Teil der Patienten mit supravalvulärer Aortenstenose zeigt pathologische Veränderungen der Aortenklappe selbst. Eine ausgeprägte Redundanz der Klappensegel, hervorgerufen durch die verengte sinutubuläre Leiste als Aufhängepunkt derselben, kann zu frühzeitigen degenerativen Veränderungen der Klappensegel führen. Die sogenannte “supra”-valvuläre Stenose beeinträchtigt also stets Struktur und Funktion der gesamten Aortenwurzel. Ein korrektiver chirurgischer Eingriff sollte diesem Rechnung tragen, indem die Leiste über allen drei Sinus durchtrennt und adäquat erweitert wird.

Summary

Techniques of surgical repair of supravalvar aortic stenosis vary from simple enlargement using a single patch to more extensive procedures. In this review we describe the development of surgical therapy and elucidate the structural basis in the context of anatomical restoration of the aortic root. Thickened and rigid aortic valvar leaflets, as well as partial adhesion of the leaflets to the stenosing sinutubular ridge, were seen frequently, together with markedly abnormal dimensions of the aortic root. A marked redundancy of the free edge of the leaflets relative to the diameter of the sinutubular junction was observed in anatomic specimens. The entire valvar apparatus, therefore, is always affected in hearts with so-called supravalvular stenosis. We recommend that ideal surgical restoration of the aortic root should take account of all the anatomic components by enlarging all three sinuses of Valsalva at the sinutubular junction.

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Stamm, C., Busch, T., Felderhoff, T. et al. Supravalvuläre Aortenstenose: Morphologie der Aortenklappe und deren Bedeutung für die chirurgische Therapie. Z. Herz-, Thorax-, Gefäßchir. 11, 282–288 (1997). https://doi.org/10.1007/BF03045204

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