Blackshear Joseph L
Department of Cardiovascular Diseases, Mayo Clinic Florida, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.
Curr Treat Options Cardiovasc Med. 2018 Feb 3;20(1):6. doi: 10.1007/s11936-018-0595-1.
Gastrointestinal bleeding with severe aortic stenosis was originally described in the 1950s by Heyde, although for years, the association was debated. Further discovery of mechanisms and the ubiquity and severity of acquired von Willebrand syndrome in the left ventricular assist device therapy have removed any doubts. At this time, gastrointestinal bleeding from intestinal angiodysplasia in patients with turbulence-related proteolysis of the highest molecular weight multimers of von Willebrand factor is now known to occur in patients with aortic stenosis, and also subaortic obstruction and associated mitral insufficiency in hypertrophic cardiomyopathy, isolated mitral and aortic insufficiency, endocarditis, and in patients with prosthetic valve dysfunction, either from stenosis or insufficiency. The degree of loss of high molecular weight multimers correlates with lesion severity, and tests of von Willebrand factor function have been proposed as important biomarkers of the severity of valve dysfunction, including in-lab testing for paravalvular leak during transcatheter aortic valve replacement. Bleeding tends to recur after endoscopic or surgical therapy, but cardiac repair is curative in the great majority.
严重主动脉瓣狭窄伴发的胃肠道出血最初是在20世纪50年代由海德描述的,尽管多年来这种关联一直存在争议。随着机制的进一步发现以及获得性血管性血友病综合征在左心室辅助装置治疗中的普遍性和严重性,所有疑问都已消除。目前已知,在主动脉瓣狭窄患者中,以及肥厚型心肌病的主动脉瓣下梗阻和相关二尖瓣关闭不全、单纯二尖瓣和主动脉瓣关闭不全、心内膜炎患者,以及人工瓣膜功能障碍(无论是狭窄还是关闭不全)患者中,都会发生由于血管性血友病因子高分子量多聚体的湍流相关蛋白水解导致的肠道血管发育异常引起的胃肠道出血。高分子量多聚体的丢失程度与病变严重程度相关,血管性血友病因子功能检测已被提议作为瓣膜功能障碍严重程度的重要生物标志物,包括在经导管主动脉瓣置换术中对瓣周漏的实验室检测。内镜或手术治疗后出血往往会复发,但大多数情况下心脏修复是治愈性的。