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主动脉病:病因、遗传学、鉴别诊断、预后和管理。

Aortopathies: etiologies, genetics, differential diagnosis, prognosis and management.

机构信息

Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke's Medical Centers, University of Wisconsin School of Medicine and Public Health, Milwaukee, WI, USA.

出版信息

Am J Med. 2013 Aug;126(8):670-8. doi: 10.1016/j.amjmed.2013.01.029. Epub 2013 Jun 22.

DOI:10.1016/j.amjmed.2013.01.029
PMID:23800581
Abstract

Aortic root and ascending aortic dilatation are indicators associated with risk of aortic dissection, which varies according to underlying etiologic associations, indexed aortic root size, and rate of progression. Typical aortic involvement is most commonly seen in syndromic cases for which there is increasing evidence that aortic aneurysm represents a spectrum of familial inheritance associated with variable genetic penetrance and phenotypic expression. Aortic root and ascending aortic dimensions should be measured routinely with echocardiography. Pharmacologic therapy may reduce the rate of progression. Timing of surgical intervention is guided by indexed aortic size and rate of change of aortic root and ascending aorta dimensions. Lifelong surveillance is recommended.

摘要

主动脉根部和升主动脉扩张是与主动脉夹层风险相关的指标,其风险因潜在病因关联、索引主动脉根部大小和进展速度而有所不同。典型的主动脉受累最常见于综合征病例,越来越多的证据表明,主动脉瘤代表与可变遗传外显率和表型表达相关的家族遗传谱。应常规使用超声心动图测量主动脉根部和升主动脉的尺寸。药物治疗可能会降低进展速度。手术干预的时机取决于索引主动脉大小以及主动脉根部和升主动脉尺寸的变化速度。建议进行终身监测。

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