PO0033 - Prevention of perioperative hemorrhage during open cardiac surgery in children with high titer inhibitor by Emicizumab combined with rhFVII：one case report

Wednesday, July 13, 2022

1:00 PM – 2:00 PM

Location: ExCel Center, ICC Capital Suite Room 1

Presenting Author(s)

Xiaoqin Feng, MD,PhD

Associate Professor
Nanfang Hospital, Southern Medical University
Guangzhou, Guangdong, China (People's Republic)

Co-Author(s)

SZ

Shaoyi Zheng

Professor
Cardiothoracic Surgery，Nanfang Hospital, Souther Medical University
Guangzhou, Guangdong, China (People's Republic)
JS

Jing Sun

Professor
Hematology, Nanfang Hospital, Southern Medical University
Guanghzou, Guangdong, China (People's Republic)
ZW

Zhenkang Wang

Professor
Cardiothoracic Surgery, Nanfang Hospital, Southern Medical University
Guangzhou, Guangdong, China (People's Republic)
HL

Huiping Li

Attending physician
Pediatrics, Nanfang Hospital, Southern Medical University
Guangzhou, Guangdong, China (People's Republic)
ZC

Zhenping CHEN

Professor
Beijing Children’s Hospital, Capital Medical University
Beijing, Beijing, China (People's Republic)
qL

qiang Li

Technicain
Clinical Laboratory，Nanfang Hospital，Southern Medical University
Guangzhou, Guangdong, China (People's Republic)
JL

Jinxi Lai

Technician
Guangzhou Zhili medical laboratory
Guangzhou, Guangdong, China (People's Republic)
JD

Jing Du

Nurse
Pediatrics, Nanfang Hospital, Southern Medical University
Guangzhou, Guangdong, China (People's Republic)

Background: Prevention of bleeding during major surgery in patients with high titer inhibitors is a great challenge. Emicizumab or rhFVII is possible valid options.

Aims: To explore the efficacy of Emicizumab combined with rhFVII in preventing hemorrhage in children with high titer inhibitors during the perioperative period of major surgery.

Methods:

Method: The patient was 2 years old boy, 13kg,diagnosed severe hemophilia A with ventricular septal defect (massive left-to-right shunt), accompanied by severe pulmonary hypertension. After the 20th exposure days, he was found to combine inhibitor around 27- 35BU/ml.He received a total of 4 doses of Emicizumab(30mg, IH, 1/W )for prophylactic application. The operation of ventricular septal repair and mitral valvuloplasty under cardiopulmonary bypass were performed after systemic heparinization. Arteries were blocked for 69 min. In the perioperative period,rhFVII 1-2 mg/dose was used, q2h-q12h, and continued to used Emicizumab 30mg/W for 2 weeks after surgery. Laboratory data such as PT, D-Dimmer, FVII level, CAT and clinical bleeding were monitored.

Results:

Results: After 3 doses of Emicizumab, the equivalent FVIII levels reached 9.8%-15.7%, respectively. Intraoperative bleeding was about 60ml. However, there was persistent pericardial oozing blood after operation. He was given rhFVII 2mg/dose, q2h IV, infusion of RBC and fresh frozen plasma for supportive treatment, and then the bleeding gradually decreased. The dose of rFVII was gradually tappered. One week after the operation, the inhibitor titer was 14.3BU/ml. The drainage tube was removed 5 days after the operation, and the patient recovered well. CAT and plasma FVII levels are shown in Figures 1 and 2.

Conclusion(s):

Discussion: For the cardiac surgery under cardiopulmonary bypass after heparinization in children with high titer inhibitors, the use of Emicizumab combined with rFVII to prevent perioperative bleeding, still requires high-dose of rhFVII in the early postoperative period, and overall effective prevention bleeding can be achieved.