三期的に全大動脈置換を行った若年者Marfan症候群の1例

Successful Staged Total Aortic Replacement in a Young Patient with Marfan Syndrome：Report of a Case

はじめに　Marfan症候群は結合組織の異常に起因する常染色体優性遺伝性疾患であり，心血管系では大動脈拡張，瘤形成，大動脈解離などの重篤な合併症を引き起こすことが知られている．そのため大動脈径の定期的な経過観察が推奨されており，病変の進行に応じて予防的に広範囲の人工血管置換術が施行されることもある1）．われわれは，右下肢灌流障害を伴うStanford B型大動脈解離と診断された若年Marfan症候群患者に対し，有症状の下肢虚血に対する血行再建術を先行し，続いて自己弁温存型大動脈基部置換術，全弓部置換術，胸腹部大動脈置換術を段階的に施行し，良好な転帰を得た1例を経験したため報告する．

We report a case of a young male patient diagnosed with Marfan syndrome who presented with lower limb ischemia due to Stanford type B aortic dissection. Initial imaging revealed significant dilation of the aortic root and occlusion of the right iliac artery. Surgical treatment was performed in three stages：revascularization of the lower limbs, valve-sparing aortic root and total arch replacement, and finally thoracoabdominal aortic replacement. This stepwise approach prioritized organ perfusion and allowed for safe and effective repair of the extensive aortic pathology. The postoperative course was uneventful, and the patient remains in good condition. This case highlights the importance of staged surgical strategy in complex aortic disease associated with Marfan syndrome.