Marfan Sendromlu Gebede Anestezi Yönetimi

Abstract

Marfan Sendromu (MS) 1/5000 oranında otozomal dominant geçiş gösteren bir bağ doku hastalığıdır. Fibrillin-1 (FBN1) geninin 15q21 kromozomunda mutasyon vardır. İskelet sistemi anormallikleri, lens dislokasyonu, dural ektazi ve aort dilatasyonu ile karakterizedir. Hastaların %80’ninde kardiyovasküler sistemde birtakım değişiklikler mevcut olup bunlar; aort dilatasyonu, aort yetmezliği, mitral-triküspit valv prolapsusu ve regürjitasyonudur. Majör ölüm nedenleri arasında aortik anevrizma rüptürü ve diseksiyonu yer alır. Gebelikteki fizyolojik değişiklikler aort diseksiyonu patogenezi ve progresyonuna katkıda bulunmaktadır.

Gebelikte artmış aort kompliyansı ve bunun sonucunda aort dilatasyonu sözkonusudur. Gebelik ve doğum MS’lu hastalarda yaşamı tehdit eden diseksiyonlara yol açar; özellikle de aort çapı 40 mm’ den geniş ise. MS’lu gebelerde lumbal spinal deformiteler ve yaygın artrodezisler, epidural ve spinal anestezi başarısızlığını artırır. Benzer şekilde dural ektaziler dural delinme riskinin artmasına neden olur. Bu olgu sunumuyla MS’lu gebe kadında elektif sezaryen operasyonunda genel anestezi uygulamamızı sunmayı amaçladık.

Keywords

• Marfan Sendromu, diseksiyon, genel anestezi

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