Shimizu Masatoshi, Masai Hiroyuki, Miwa Yoichi
Department of Cardiology, National Hospital Organization Kobe Medical Center.
Intern Med. 2007;46(8):481-5. doi: 10.2169/internalmedicine.46.6026. Epub 2007 Apr 17.
A 69-year-old woman presented with a harsh systolic murmur and severe anemia. Echocardiography demonstrated hypertrophic obstructive cardiomyopathy with a peak pressure gradient of 154 mmHg. Endoscopic examinations disclosed an angiodysplasia and multiple diverticula in the colon, but no active bleeding was noted in these lesions. A selective defect of large multimers of von Willebrand factor was detected by electrophoresis. After collection of anemia and Ca antagonist therapy, left ventricular obstruction was relieved and cessation of the occult gastrointestinal bleeding was obtained. This is the first report whereby acquired type 2A von Willebrand syndrome was caused by hypertrophic obstructive cardiomyopathy.
一名69岁女性因出现粗糙的收缩期杂音和严重贫血前来就诊。超声心动图显示为肥厚型梗阻性心肌病,峰值压力阶差为154 mmHg。内镜检查发现结肠有血管发育异常和多个憩室,但这些病变未见活动性出血。通过电泳检测到血管性血友病因子大分子量多聚体存在选择性缺陷。在纠正贫血并给予钙拮抗剂治疗后,左心室梗阻得以缓解,隐匿性胃肠道出血停止。这是第一例由肥厚型梗阻性心肌病导致获得性2A型血管性血友病综合征的报告。