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- W4241036390 abstract "Marfan症候群（MFS）に心室中隔欠損（VSD）を合併し，急速に心不全が進行し，手術前後で心機能が低下した乳児例を経験した．症例は2か月の女児で，VSDによる高肺血流性心不全と診断され，当院を紹介となった．家族歴，身体所見からMFSが疑われた．高肺血流に心機能低下も伴うため，段階的に修復術を施行された．肺動脈絞扼術直後と心内修復術直後にそれぞれ心機能は低下したが，徐々に回復した．3歳時に水晶体脱臼を認め，父がMFSであることから改訂Ghent基準を満たした．MFSは潜在的な心機能障害を有する可能性があり，治療介入が必要な先天性心疾患を合併する例では，術前の心不全の進行や術後管理に注意し，慎重な経過観察や治療戦略を考慮すべきである．" @default.
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- W4241036390 date "2020-06-01" @default.
- W4241036390 modified "2023-09-26" @default.
- W4241036390 title "An Infant with Marfan Syndrome and Ventricular Septal Defect and Progressive Heart Failure" @default.
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- W4241036390 doi "https://doi.org/10.9794/jspccs.36.159" @default.
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