结缔组织源性心血管疾病

Cardiovascular disease of connective tissue origin.

作者信息

Bowen J, Boudoulas H, Wooley C F

出版信息

Am J Med. 1987 Mar;82(3):481-8. doi: 10.1016/0002-9343(87)90448-7.

Abstract

Inherited abnormalities of connective tissue elements often cause changes in the structure and function of the cardiovascular system. Well-known heritable disorders of connective tissue in which cardiovascular abnormalities are prominent include the Marfan syndrome and the Ehlers-Danlos syndrome. Connective tissue abnormalities also occur without the associated features of a recognized syndrome. These include isolated valvular prolapse and anuloaortic ectasia. In this review, the cardiovascular features of connective tissue abnormalities--both the recognized syndromes and the isolated abnormalities--are described, important concepts in the diagnosis and treatment of these disorders are reviewed, and the classification of inherited connective tissue abnormalities of the cardiovascular system is discussed.

摘要

结缔组织成分的遗传性异常常导致心血管系统结构和功能的改变。心血管异常较为突出的著名遗传性结缔组织疾病包括马方综合征和埃勒斯-当洛综合征。结缔组织异常也可在无公认综合征相关特征的情况下出现。这些包括孤立性瓣膜脱垂和主动脉环扩张。在本综述中，描述了结缔组织异常（包括公认的综合征和孤立性异常）的心血管特征，回顾了这些疾病诊断和治疗中的重要概念，并讨论了心血管系统遗传性结缔组织异常的分类。