家族性主动脉疾病的外科治疗

H. B. Jmaà
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引用次数: 0

摘要

怀孕可能会增加有遗传倾向的年轻女性在怀孕三个月和围产期发生主动脉夹层的风险。必须对这种疾病进行紧急处理。之后,必须通过基因检测筛查儿童的主动脉家族性疾病,因为在儿童时期快速发展为夹层或动脉瘤的风险很高,其处理也很有挑战性。我们报告了一位孕妇因主动脉夹层而接受手术的病例,以及她的儿子四年后因升主动脉瘤和主动脉瓣关闭不全而接受手术的病例。患有家族性主动脉根部动脉瘤的儿童,如果主动脉瓣反流较轻,可以进行瓣膜疏通和主动脉置换术。但必须进行长期的临床和超声心动图随访。
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Surgical Management of Familial Aortic Disease
Pregnancy may increase the risk of aortic dissection in young women genetically predisposed in the third trimester and the peri-partum period. This disease must be managed with emergency. Later, aortic familial diseases must be screened in the children by genetic tests, because of the high risk of rapid developing dissection or aneurysm in the childhood, whose management is challenging. We report the cases of a pregnant woman operated for aortic dissection, and her son operated four years later for aneurysm of the ascending aorta, and aortic insufficiency. Children with familial aortic root aneurysm may have intervention of valve sparing and aortic replacement if the aortic regurgitation is minimal. But, long-term clinical and echocardiographic follow-up is mandatory.
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