Tamura Atsushi, Yoshizaki Takamichi, Kusadokoro Sho
Department of Cardiovascular Surgery, Kawaguchi Municipal Medical Center, Kawaguchi, Japan.
Kyobu Geka. 2017 Aug;70(9):762-764.
We report a case of severe aortic valve stenosis in a patient with Tangier disease. A 64-year-old female was diagnosed with Tangier disease on the basis of gene mutation. The serum levels of total cholesterol and high-density lipoprotein were 124 mg/dl and 4.3 mg/dl, respectively. She had a symptom of dyspnea and echocardiography revealed severe aortic valve stenosis with the maximum gradient of 60.5 mmHg. Chest computed tomography showed severe calcification of the ascending aorta and the aortic root. Aortic valve replacement using a bioprosthetic valve was performed. Several reports have been made on coronary artery revascularization in Tangier disease patients, but one on surgical treatment for aortic valve stenosis is extremely rare.
我们报告一例患有Tangier病的患者出现严重主动脉瓣狭窄的病例。一名64岁女性基于基因突变被诊断为Tangier病。其血清总胆固醇和高密度脂蛋白水平分别为124mg/dl和4.3mg/dl。她有呼吸困难症状，超声心动图显示严重主动脉瓣狭窄，最大压力阶差为60.5mmHg。胸部计算机断层扫描显示升主动脉和主动脉根部严重钙化。使用生物人工瓣膜进行了主动脉瓣置换术。关于Tangier病患者冠状动脉血运重建已有多篇报道，但关于主动脉瓣狭窄手术治疗的报道极为罕见。
