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乙型血友病患者的冠状动脉搭桥术。

Coronary artery bypass grafting in a patient with haemophilia B.

作者信息

Palanzo D A, Sadr F S

机构信息

Department of Perfusion, Lehigh Valley Hospital, Allentown, PA 18105, USA.

出版信息

Perfusion. 1995 Jul;10(4):265-70. doi: 10.1177/026765919501000410.

DOI:10.1177/026765919501000410
PMID:7488773
Abstract

Patients with coagulation disorders present the entire open-heart surgical team with an increased challenge. A patient with a known history of moderately severe Factor IX deficiency (2.4% activity) was evaluated for coronary artery disease. Cardiac catheterization revealed a 99% right coronary artery lesion, a long 99% circumflex lesion and normal left ventricular function. Sextuple coronary artery bypass grafting was performed with the aid of aprotinin and Factor IX transfusions. The patient's platelet count after cardiopulmonary bypass was 65,000/mm3, down from a preoperative level of 172,000/mm3, requiring the transfusion of six units of pooled platelets immediately postoperation. The patient was extubated five and a half hours after arriving in the Intensive Care Unit, and his chest-tube drainage after the first 24 hours was 373 ml. Other than a transient episode of atrial fibrillation on the third postoperative day, the patient had an uneventful postoperative course and was discharged on the sixth postoperative day. With the use of aproptinin and the newer monoclonal antibody-purified Factor IX concentrates that have been developed, many of the added risks of performing open-heart surgery on patients with haemophilia B are greatly reduced if not eliminated.

摘要

凝血功能障碍患者给整个心脏外科手术团队带来了更大的挑战。一名有中度严重因子IX缺乏(活性为2.4%)已知病史的患者因冠状动脉疾病接受评估。心脏导管检查显示右冠状动脉病变99%,回旋支病变99%且长,左心室功能正常。在抑肽酶和输注因子IX的辅助下进行了六支冠状动脉搭桥术。体外循环后患者的血小板计数为65,000/mm³,低于术前的172,000/mm³水平,术后立即需要输注六个单位的混合血小板。患者在进入重症监护病房五个半小时后拔管,术后头24小时的胸腔引流液为373毫升。除术后第三天出现短暂的房颤发作外,患者术后过程平稳,术后第六天出院。随着抑肽酶和新开发的单克隆抗体纯化因子IX浓缩物的使用,对B型血友病患者进行心脏直视手术的许多额外风险即使没有消除也大大降低了。

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J Thromb Thrombolysis. 2019 Aug;48(2):299-302. doi: 10.1007/s11239-019-01888-9.
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Consensus review of the treatment of cardiovascular disease in people with hemophilia A and B.甲型和乙型血友病患者心血管疾病治疗的共识性综述。
Cardiol Rev. 2015 Mar-Apr;23(2):53-68. doi: 10.1097/CRD.0000000000000045.