1型血管性血友病患者的冠状动脉旁路移植术

Sebastian Krych, Katarzyna Krzyżak, Tomasz Styn, Witold Bratkowski, Michał Jamrozik, M. Knapik, Małgorzata Jekiełek, T. Hrapkowicz
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摘要

血管性血友病(VWD)是最常见的遗传性出血性疾病(HBD),大约影响1-2%的人口。1型占所有病例的70-80%,涉及部分功能性VWF水平降低。通常表现为轻度至中度皮肤粘膜出血。一名63岁的多支冠状动脉疾病患者,在先前的非stemi梗死(10天前)后被诊断为1型血管性血友病,住进心脏外科接受计划的CABG手术。入院时凝血因子值依次为(VIII 15%;VWF: Ag 12%)。冠状动脉造影显示为中枢性冠心病。病人接受了心脏科的会诊。决定采用OPCAB技术对患者进行冠脉搭桥。制定了详细的止血方案。血液p500 /1200和1000/2400,冻干浓缩人VIII和血管性血友病因子,是安全的。在手术期间,LITA- LAD和Ao-RCA桥使用美敦力海星®心脏定位器进行。术后无并发症发生。每日测定血浆VIII和VW因子水平,并根据测量值给予抗出血预防。患者术后第8天出院,血液学检查结果良好。目前,对于接受心脏手术的血管性血友病患者的管理尚无指南。不正确的药物治疗可能导致VW因子水平升高,增加急性冠状动脉综合征和心脏病发作的风险。
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Coronary artery bypass graft in a patient with Von Willebrand disease type 1
Von Willebrand disease (VWD), is the most common hereditary bleeding disorder (HBD) and affects ap - proximately 1–2% of the population. Type 1 accounts for 70–80% of all cases and involves partially reduced levels of functional VWF. It is usually manifested by mild to moderate mucocutaneous bleeding. A 63-year-old patient with multivessel coronary artery disease and diagnosed with Von Willebrand’s disease type 1 after a previous NSTEMI infarction (10 days earlier) was admitted to the cardiac surgery department for a planned CABG procedure. The coagulation factor values on admission to the ward were successively (VIII 15%; VWF: Ag 12%). Coronary angiography revealed MV CAD. The patient was consulted with the HEART Team. The decision was made to qualify the patient for CABG using the OPCAB technique. A detailed plan for securing the haemostasis was established. Haemate P 500/1200 and 1000/2400, a lyophilized concentrate of humans VIII and von Willebrand Factors, were secured. During surgery, LITA- LAD and Ao-RCA bridges were performed using Medtronic Starfish® Heart Positioners. In the post-operation period, no complications were noted. Plasma levels of VIII and VW factors were measured daily and antihemorrhagic prophylaxis was given accordingly to measured values. The patient was discharged on the eighth day after surgery in good condition, with the haematological recommendations. Currently, there are no guidelines for the management of patients with Von Willebrand’s disease undergoing cardiac surgery. Incorrectly conducted pharmacotherapy may result in an elevated level of VW factor and additional exposure to the occurrence of acute coronary syndromes and heart attacks.
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