Marfan氏综合征的心血管病变(附2例尸检报告)

Marfan氏综合征是一较罕见的家族遗传性疾病。国内有关尸检资料报道不多。今报告我们尸检诊断的2例,并对本征病因机理、病理变化、并发症和预后等加以分析。

CARDIOVASCULAR CHANGES IN THE MARFAN''''S SYNDROME

2 cases of Marfan's syndrome diagnosed by autopsy arereported. The common features in the cardiovascular changes are:lesions occurring mainly in cardiovascular system, including dilatationof the aortic rootage, mucous degeneration and necrosis in the vasculartunica media, reduction of smooth muscles, rupture and defectiveformation of elactic fiber, vascular dissections and proliferation of thetunica inner. In case 1, vascular dissections are very extensive, invol-ving aorta with its branches. Lesions just like that in the aorta alsooccur in the pulmonary artery, with extensive thrombosis in thebranches. The patient died of the heart failure. In case 2, vasculardissections only involved aorta, thoracic aorta and innominate artery.The patient died of the aortic rupture. The relevant literatures werereviewed and the etiology, pathogenesis, morphology and complicationsof the Marfan's syndrome were discussed.