Central venous access devices implantation in children with severe hemophilia a: data from the children comprehensive care center of China

Qian Xu; Chunli Wang; Wei Cheng; Yingzi Zhen; Yaguang Ding; Guoqing Liu; Wanru Yao; Zhenping Chen; Zhiqiang Li; Runhui Wu

doi:10.1016/j.heliyon.2023.e13666

Central venous access devices implantation in children with severe hemophilia a: data from the children comprehensive care center of China

Heliyon. 2023 Feb 11;9(3):e13666. doi: 10.1016/j.heliyon.2023.e13666. eCollection 2023 Mar.

Authors

Qian Xu^{1

2

3

4

5}, Chunli Wang⁶, Wei Cheng⁷, Yingzi Zhen⁶, Yaguang Ding⁶, Guoqing Liu^{1

2

3

4}, Wanru Yao^{1

2

3

4}, Zhenping Chen^{1

2

3

4}, Zhiqiang Li⁷, Runhui Wu^{1

2

3

4}

Affiliations

¹ Department II of Hematology Center, Haemophilia Comprehensive Care Center, Beijing Children's Hospital, Capital Medical University; National Center for Children's Health, Beijing 100045, China.
² Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, Beijing 100045, China.
³ Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing Children's Hospital, Capital Medical University, Beijing 100045, China.
⁴ National Key Discipline of Pediatrics, Capital Medical University, Beijing 100045, China.
⁵ Department of Hematology & Oncology, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu 611731, China.
⁶ Nursing Department, Beijing Children's Hospital, Capital Medical University; National Center for Children's Health, Beijing 100045, China.
⁷ Department of Cardiovascular Surgery II, Beijing Children's Hospital, Capital Medical University; National Center for Children's Health, Beijing 100045, China.

Abstract

Objectives: To report the perioperative management experience of central venous access devices (CVAD) in Chinese children with severe hemophilia A (SHA) in China.

Methods: This retrospective study included SHA children who underwent Port-A-Cath or peripherally inserted central catheter (PICC) implantation between 2020/01 and 2021/07. Collected data included baseline characteristics, factor replacement regimen and CVAD-related complications.

Results: Nine patients had nine ports placed, and eight patients underwent 10 PICCs placement. Patients without or with low-titer inhibitor (<5 BU) received a port. The median preoperative and postoperative plasma-derived factor VIII (pd-FVIII) doses were 53.0 (44.4-61.1) and 315.9 (88.2-577.8) IU/kg. The median port duration was 189 (15-512) days, with infection incidence of 0.06 per 1000 CVAD days. Patients with high-titer inhibitors (>10 BU) received PICC. The median recombinant factor VIIa (rFVIIa) dose was 87.47 μg/kg before and for 5-7 doses after implantation over 2-3 days. The median PICC duration was 226.5 days, with infection incidence of 0.12 per 1000 catheter-days.

Conclusions: CVADs can be safely implanted in China. PICC implantation is a practical and safe option for SHA children with high-titer inhibitors.

Keywords: Children; Factor replacement regimen; Hemophilia A; Peripherally inserted central catheter; Port-A-Cath; Vascular access devices.