心脏手术中血友病患者的替代疗法：3例报告

doi:10.24920/003929

Chinese Medical Sciences Journal ›› 2022, Vol. 37 ›› Issue (1): 79-81.doi: 10.24920/003929

心脏手术中血友病患者的替代疗法：3例报告

陈小坤¹,苗齐²,朱铁楠³,张超纪^2,^*()

¹中国医学科学院北京协和医学院北京协和医院外科,北京 100730,中国
²中国医学科学院北京协和医学院北京协和医院心外科,北京 100730,中国
³中国医学科学院北京协和医学院北京协和医院血液科,北京 100730,中国

收稿日期:2021-05-07 接受日期:2021-10-15 出版日期:2022-03-31 发布日期:2022-03-02
通讯作者: 张超纪 E-mail:chaojizhang@163.com

Replacement Therapy for Hemophilia Patients Undergoing Cardiac Surgery: Report of Three Cases

Xiaokun Chen¹,Qi Miao²,Tienan Zhu³,Chaoji Zhang^2,^*()

¹Department of Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
²Department of Cardiac Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
³Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

Received:2021-05-07 Accepted:2021-10-15 Published:2022-03-31 Online:2022-03-02
Contact: Chaoji Zhang E-mail:chaojizhang@163.com

摘要/Abstract

摘要：

血友病是一种X连锁隐性遗传性出血性疾病。近年来随着长效凝血因子及非因子治疗的问世,血友病的诊疗越来越多样化,使得血友病患者的预期寿命得到延长。但血友病患者血液中的凝血因子减少并不会减慢动脉粥样硬化的进展。随着预期寿命的增加,越来越多的血友病患者将患上与年龄相关的心血管疾病,并且将需要外科手术干预。由于心脏手术需要肝素化和体外循环,血友病患者的止血和凝血平衡极具挑战性。本文报告了三例成功接受心脏手术的血友病患者。

关键词: 血友病, 心脏手术, 替代疗法

Abstract:

Hemophilia is an X-linked recessive inherited bleeding disorder. Despite the improved treatment in recent years with the advent of replacement therapies, the progression of atherosclerosis is not slowed down after the reduction of clotting factors in hemophilia. As life expectancy increases, more hemophilia patients will suffer from age-related cardiovascular diseases. Since cardiac surgery needs heparinization and cardiopulmonary bypass (CPB), it is extremely challenging to balance hemostasis and coagulation in patients with hemophilia. Here we report three cases of hemophilia patients who underwent cardiac surgery successfully.

Key words: Hemophilia, cardiac surgery, replacement therapy

Xiaokun Chen, Qi Miao, Tienan Zhu, Chaoji Zhang. Replacement Therapy for Hemophilia Patients Undergoing Cardiac Surgery: Report of Three Cases[J].Chinese Medical Sciences Journal, 2022, 37(1): 79-81.

Characteristics	Case 1		Case 2		Case 3
Age/sex	57 years/M		62 years/M		49 years/M
Weight (kg)	65		68		70
Diagnosis	CAD		AA		HOCM
Type of hemophilia	B		B		A
Pre-test
Drugs	FⅨ		PCC		FⅧ
Dosage	1000IU		30IU/Kg		3500IU
Factor activity APTT (s)	FⅨ (%)	APTT	FⅨ (%)	APTT	FⅧ (%)	APTT
0	3.6	47.3	2.2	44.4	10.6	43.5
15min	14.9	-	35.4	33.1	25.8	-
2h	-	-	31.7	29.6	104.2	-
4h	11.2	-	28.1	29.5	94.9	-
8h	9.1	-	27.8	29.3	79.7	-
12h	8.6	-	20.5	30.9	67.6	30.3
24h	11.5	37.5	-	31.9	57.0	31.8
Replacement therapy
Drugs	FⅨ		PCC		FⅧ
Dosage	Preoperative	6000 IU	Preoperative	4200 IU	Preoperative	2800 IU
	12h	1500 IU	12h	2200 IU	12h	2800 IU
	D1-D2	3000 IU	D1	2200 IU	D1-D2	2800 IU
	D3-D4	2000 IU	D2-D3	2000 IU	D3-D4	1000 IU
	D5	change to PCC	D4-D6	1400 IU	D5-D7	1000 IU
			D7-D14	800 IU
ACT (s)
Before CPB	596		530		585
Postoperation	104		95		108
Time of CPB (min)	92		171		79
Bleeding (mL)	300		500		500

1.	Lin PS, Yao YT. Perioperative management of hemophilia A patients undergoing cardiac surgery: a literature review of published cases. J Cardiothorac Vasc Anesth 2021; 35(5):1341-50. doi: 10.1053/j.jvca.2020.06.074. doi: 10.1053/j.jvca.2020.06.074
2.	Kanellopoulou T, Nomikou E. Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B. J Card Surg 2018; 33(2):76-82. doi: 10.1111/jocs.13530. doi: 10.1111/jocs.13530 pmid: 29439283
3.	Shalabi A., Kachel E, Kogan A, et al. Cardiac surgery in patients with hemophilia: is it safe?. J Cardiothorac Surg 2020; 15(1):76. doi: 10.1186/s13019-020-01123-0 doi: 10.1186/s13019-020-01123-0 pmid: 32384896

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心脏手术中血友病患者的替代疗法：3例报告

Replacement Therapy for Hemophilia Patients Undergoing Cardiac Surgery: Report of Three Cases

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