Perioperative haemostatic management and bleeding outcomes in congenital factor VII deficiency: a retrospective cohort study.

BACKGROUND

Congenital factor VII (FVII) deficiency is characterised by a poor and inconsistent relationship between FVII activity (FVII:C) and bleeding phenotype, making perioperative haemostatic management challenging. We aimed to describe perioperative management strategies, identify factors associated with haemostatic treatment, and evaluate perioperative outcome.

METHODS

We conducted a retrospective cohort study of patients with congenital FVII deficiency (severe FVII:C < 10%, moderate 10-19%, mild ≥20% according to ISTH) undergoing surgical procedures or delivery at Charité - Universitätsmedizin Berlin between January 2015 and June 2023. Procedural bleeding risk was classified as minor, low or high according to 2022 ESC non-cardiac surgery guidelines. Haemostatic management, bleeding and thromboembolic outcomes were assessed; univariate logistic regression explored factors associated with haemostatic treatment.

RESULTS

Ninety-three procedures were analysed in 70 patients; 73% were performed in patients with mild, 14% with moderate and 13% with severe deficiency. Haemostatic treatment was administered in 71% of procedures, most commonly tranexamic acid (56%) and factor replacement therapy (38%). Lower FVII:C was associated with factor replacement therapy (OR 0.96, 95% CI: 0.92-0.99; P = 0.006), bleeding risk and bleeding history were not. Perioperative bleeding occurred in 9 (9.7%) procedures, all in patients with mild deficiency (FVII:C 24-45%) and all during high-risk procedures. Two arterial thromboembolic events (2.2%) occurred.

CONCLUSIONS

In clinical practice, perioperative treatment in congenital FVII deficiency appeared driven primarily by FVII:C, whereas bleeding events clustered among patients with mild deficiency undergoing high-risk procedures. As treatment itself may have influenced this pattern, our findings highlight the need for an individualised perioperative risk assessment that integrates FVII:C, bleeding phenotype and procedural risk, rather than relying on FVII:C alone.