# Modern Treatment of Valvulopathies in Patients with Congenital Hemophilia

## TL;DR

This paper reviews how heart valve surgeries can be safely performed in people with hemophilia through careful planning and multidisciplinary care.

## Contribution

The paper is the first to synthesize modern surgical approaches for valvular disease in hemophiliacs.

## Key findings

- Valve repair and bioprosthetic valves are preferred to avoid lifelong anticoagulation in hemophiliacs.
- The On-X mechanical valve may be used when bioprosthetic valves are not an option due to less intensive anticoagulation needs.
- Maintaining a specific coagulation factor level is essential for safe antithrombotic treatment in these patients.

## Abstract

Hemophiliacs can develop cardiovascular diseases, including valvulopathies of various etiologies and severities. Some require surgical treatment. Performing cardiac surgery in hemophiliacs is a challenge because they maintain an increased risk of bleeding throughout their lives. Our review shows that with a multidisciplinary team and careful planning, cardiac surgery can be safely performed in these patients. Valve repair and bioprosthetic valves should be preferred over mechanical valves to avoid life-long anticoagulation. In patients who cannot receive a bioprosthetic valve, the use of the On-X mechanical valve might be considered because it requires less intensive anticoagulation after 3 months of treatment. Antithrombotic treatment is feasible in hemophiliacs only if the coagulation factor level is kept constantly above a specific trough limit. Our review is valuable because, for the first time, the available data on the modern surgical treatment of valvular disease in hemophiliacs have been synthesized and systematized.