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症例1:38歳,女性.5年前,妊娠38週時に強い背部痛があったが無事出産した.3年前の検診で縦隔影の拡大を指摘され,当科で精査しMarfan症候群とDeBakey Ⅲb型の大動脈解離と診断した.最近,大動脈解離の拡大傾向があり待機手術の予定.
症例2:32歳,女性.10歳時に水晶体脱臼がありMarfan症候群と診断された.1991年6月(妊娠32週)右頸部に疼痛を伴う拍動性腫瘤が出現し当科に紹介された.急性大動脈弁閉鎖不全を合併したDeBakey Ⅰ型の急性大動脈解離と診断し,緊急手術(帝王切開と上行弓部大動脈置換+大動脈弁置換+右冠動脈バイパス)を行った.術後経過良好で母子ともに無事退院に至った.Marfan症候群の妊娠中の大動脈解離発症は一般に予後が悪く,Mar-fan症候群における妊娠の可否決定には本人・家族との十分な意志疎通が必要で,妊娠続行の場合の管理には各科での綿密な連携が欠かせない.
Two women with Marfan syndrome who developed aortic dissection during pregnancy are reported. Case 1: A 38 year-old woman had experienced severe back pain five years before (at the age of 33) when she was in the 38th week of gestation, followed by labour with no problem. Three years ago, she was examined in our hospital for enlarged thoracic aorta pointed out in a somatoscopy and was diagnosed as having Marfan syndrome and aortic dissection of DeBakey type Ⅲb. Because her aortic dissection has been growing larger recently, surgical repair is scheduled electively.
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