Key result
A two-stage operation of Caesarian section followed two days later by a modified Bentall procedure safely managed acute type A aortic dissection in a pregnant patient with Marfan syndrome.
Case Report (n=1)
No
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A two-stage surgical strategy (Caesarian section followed by delayed aortic repair) can be safely performed for acute type A aortic dissection in late pregnancy complicated by Marfan syndrome.
妊娠後期に急性A型大動脈解離をきたしたMarfan症候群の1治験例を経験したので報告する．症例は32歳女性の初産婦．妊娠29週目に突然前胸部痛を発症し前医を受診．CT上，急性A型大動脈解離と診断され手術目的に当科へ搬送された．可及的早期の心大血管修復が必要であったが，人工心肺使用の際のヘパリン投与による子宮・胎盤剥離面からの大量出血の危険性を考慮し，帝王切開・子宮全摘術を先行させ，2日後にBentall手術＋上行弓部大動脈人工血管置換術を二期的に施行した．母子ともに術後経過は良好であった．妊娠後期に大動脈解離をきたしたMarfan症候群においては，その治療戦略が非常に重要になる．二期的手術，すなわち，帝王切開を先行し，ICUにて鎮静・人工呼吸管理下に厳重に循環動態をコントロールし，産科的出血が落ち着いてから心大血管修復を行うという戦略をとることで，安全に手術および周術期管理を行うことができた．
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畠山 正治 (2009) conducted a case report in Acute type A aortic dissection in late pregnancy with Marfan syndrome (n=1). Two-stage operation (Caesarian section followed by modified Bentall procedure) was evaluated on Maternal and fetal survival. A two-stage operation of Caesarian section followed two days later by a modified Bentall procedure safely managed acute type A aortic dissection in a pregnant patient with Marfan syndrome.
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