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.

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.