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马凡氏综合征32例外科治疗 被引量:2

Surgical Treatment for 32 Cases of Marfan's Syndrome
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摘要 目的 观察马凡氏综合征心血管病变手术治疗效果。方法 收集在我院手术治疗的马凡氏综合征患者 32例 ,对比分析他们手术前及手术后 6~ 12个月心功能变化情况。结果 Bentall(升主动脉带瓣管道置换 )手术者 2 9例 ,升主动脉带瓣管道并全主动脉弓置换 3例 ,同期行二尖瓣置换两例。手术死亡 3例 ,失去追踪 3例 ,2 6例参与研究的患者手术后 6~ 12个月心功能较术前比较 ,左心室大小、左心室射血分数、左心室舒张末期容量指数及左心室收缩末期容量指数等指标均明显改善 (P <0 0 1)。结论 外科手术是治疗马凡氏综合征心血管病变行之有效的方法。 Objective To observe the operative outcomes of Marfan's syndrome.Methods Collecting 32 cases of Marfan's syndrome operated in our hospital and analyzing the changes of cardiac function between preoperation and 6~12 months postoperation. Results There were 29 cases of Bentall(replacement of the ascending aortic with flap valve canal) operation, 3 cases of them were Bentall with total aortic arch replacement; two case were replaced the mitral valve in the homochronous operation; 3 cases died from the Bentall operation,other 3 cases were lost to follow up. The size of left ventricle(LV), left ventricular ejection fraction(LVEF), left ventricular end-diastolic volume index (LVEDVI) and left ventricular end-systolic volume index(LVESVI) were remarkable improved in postoperation. Conclusions Surgical treatment is an effective way for the cardiovascular lesions of Marfan's syndrome.
出处 《中国医师杂志》 CAS 2002年第10期1077-1079,共3页 Journal of Chinese Physician
关键词 马凡氏综合征 BENTALL手术 主动脉全弓置换 遗传病 结缔组织疾病 Marfan's syndrome Bentall Operation Total aortic arch replacement
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  • 2Hayward C, Porteous ME, Brock DJ, et al. Mutation screening of all 65 exons of the fibrillin-1 gene in 60 patients with Marfan syndrome: report of 12 novel mutations[J]. Hum mutat, 1997, 10(4): 280~289 被引量:1
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