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01121 Journal of Nara Medical Association >
Vol.52 No.1 >
このアイテムの引用には次の識別子を使用してください:
http://hdl.handle.net/10564/646
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タイトル: | 妊娠後期に僧帽弁腱索断裂によるうっ血性心不全を発症したMarfan症候群の1例 |
その他のタイトル: | A CASE OF MARFAN SYNDROME ASSOCIATED WITH CONGESTIVE HEART FAILURE DUE TO RUPTURED CHORDAE TENDINEAE IN LATE PREGNANCY |
著者: | 山本, 広光 中谷, 秀隆 中嶋, 民夫 土肥, 直文 上村, 史朗 橋本, 俊雄 土肥, 和紘 |
キーワード: | Marfan syndrome mitral valve prolapse infectious endocarditis |
発行日: | 2001年2月28日 |
出版者: | 奈良医学会 奈良県立医科大学 |
引用: | Journal of Nara Medical Association Vol.52 No.1 p.9-14 |
抄録: | We report a rare case of Marfan syndrome associated with congestive
heart failure due to ruptured chordae tendineae in late pregnancy. A 29-year-old woman,
who was 32 weeks pregnant, was admitted to our hospital with complaints of dyspnea on
exertion. Although she had had severe myopia, she was never found to have a cardiac
murmur. On physical examination, arachnodactyly was revealed, and both the wrist sign
and the thumb sign were positive. A chest radiography showed bilateral pleural effusion,
cardiomegaly, and scoliosis. Echocardiography showed mitral valve prolapse (MVP) with
ruptured chordae tendineae and severe mitral regurgitation. The patient was diagnosed
as having Marfan syndrome with congestive heart failure due to ruptured chordae
tendineae. She was treated with diuretics and a daily continuous intravenous drip of
vasodilator (ISDN, 3.6 mg/h), dopamine (5.0μg/kg/min) and dobutamine (10.0
μg/kg/min). Due to the appearance of pulmonary edema on the 5th hospital day, positive
end-expiratory pressure (PEEP) and an extracorporeal ultrafiltration method (ECUM)
were performed. Although an emergency cesarean section was performed on the 6th
hospital day, congestive heart failure and low-grade fever persisted. On the 27th hospital
day, mitral valve replacement (MVR) was performed. Subsequently, the patient's
congestive heart failure gradually improved. Histological analysis of the mitral valve
extracted on MVR revealed muciform changes and infiltration of inflammatory cells in
the mitral valve leaflets. These findings are consistent with changes of mitral valve
leaflet related to the Marfan syndrome having both mitral valve prolapse and infectious
endocarditis. |
URI: | http://hdl.handle.net/10564/646 |
ISSN: | 13450069 |
出現コレクション: | Vol.52 No.1
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