Nomura K, Nakamura Y, Yamagishi M, Yamamoto K, Hanada R
Saitama Children Medical Center, Japan.
Kyobu Geka. 1994 Dec;47(13):1071-4.
We performed an open heart surgery for ventricular septal defect and right coronary cusp prolapse in a 5-year-old boy with hemophilia A. He has only 3.5% of factor VIII activity. This paper describes a successful perioperative management of hemophilia A. Previous infusion study of factor VIII concentrate showed an administration of 500 units of factor VIII increased its level from 3.5 to 25% two hours after the infusion. With this result we administered 1,500 units of concentrate 14 and 2 hours before the operation, factor VIII level was 201% after the induction of anesthesia. Sternotomy was uneventfully performed. Subarterial VSD was patch closed and right coronary cusp hung down into the defect was plicated. After intra cardiac repair, we administered 2,000 units of VIII factor concentrate, followed by an injection of protamin and its level was 240% at CCU. During the initial 3 postoperative day, patient was maintained with 1,500 units of factor VIII every 12 hours. Chest drains were removed on 2 postoperative day with no evidence of bleeding. Additional infusion of 1,000 units per a day was continued on postoperative days 4 through 17th. Subsequently factor VIII level was maintained more than 60%, so that we achieved successful perioperative course in a patient with hemophilia A.
我们为一名患有甲型血友病的5岁男孩进行了室间隔缺损和右冠状动脉瓣脱垂的心脏直视手术。他的凝血因子VIII活性仅为3.5%。本文描述了甲型血友病成功的围手术期管理。先前对凝血因子VIII浓缩物的输注研究表明，输注500单位的凝血因子VIII后两小时，其水平从3.5%升至25%。基于此结果，我们在手术前14小时和2小时分别给予1500单位的浓缩物，麻醉诱导后凝血因子VIII水平为201%。胸骨切开术顺利进行。动脉下室间隔缺损用补片修补，脱垂至缺损处的右冠状动脉瓣进行了折叠处理。心脏内修复后，我们给予2000单位的VIII因子浓缩物，随后注射鱼精蛋白，在冠心病监护病房时其水平为240%。术后最初3天，每12小时给予患者1500单位的凝血因子VIII以维持。术后第2天拔除胸腔引流管，无出血迹象。术后第4天至第17天，每天继续额外输注1000单位。随后凝血因子VIII水平维持在60%以上，因此我们在一名甲型血友病患者身上实现了成功的围手术期过程。
