Key result
Staged total aortic replacement achieves safe recovery across 5 operations in a Marfan syndrome patient.
Case Report (n=1)
No
Staged surgical intervention for extensive aortic disease in Marfan syndrome can yield favorable outcomes despite the need for multiple complex procedures.
May support individualized staging in select Marfan aortopathy cases; leaves open need for larger series on durability and generalizability.
症例はMarfan症候群の34歳，女性．27歳時にAAE，AR4度，上行大動脈瘤に対して大動脈基部置換と上行大動脈置換を行った．29歳時に急性B型大動脈解離を発症し，真腔狭小化による下肢虚血のため解離発症1カ月後にY型人工血管で腹部大動脈置換を行った．解離腔の拡大に伴い30歳時に下行大動脈置換を，32歳時に胸腹部大動脈置換を行い，今回，残存弓部大動脈と右鎖骨下動脈起始部の瘤化のため，弓部大動脈全置換と右鎖骨下動脈再建を行った．全経過7年間に5回の手術で大動脈全置換となった．全経過を通じて合併症はなく，最終の手術後1年半の現在，健在である．Marfan症候群において基部置換後に解離を発症し大動脈全置換となった症例は少なく，また分枝動脈の真性瘤の発生も稀である．本症例は短期間に種々の血管病変が出現したが，それぞれの病変の発生に対し分割手術を行うことにより良好な結果が得られた．今後は吻合部あるいは分枝動脈の厳重な経過観察を要すると考えられた．
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Inaoka et al. (2009) conducted a case report in Marfan syndrome with extensive aortic disease (n=1). Staged total aortic replacement was evaluated on Surgical success and patient survival. Staged total aortic replacement performed in 5 consecutive operations over 7 years was safe and resulted in satisfactory recovery for a patient with Marfan syndrome and extensive aortic disease.
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