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

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.