Perioperative management of rare coagulation factor deficiency states in cardiac surgery

Area of Science:

• Cardiology
• Hematology
• Anesthesiology

Background:

• Rare bleeding disorders (RBDs) encompass hereditary deficiencies in clotting factors like fibrinogen, Factor II, V, VII, VIII, XI, and XIII.
• Increased longevity and improved healthcare access are leading to a rise in cardiovascular (CV) surgeries among RBD patients.
• Existing clinical data on managing RBDs in CV surgery is limited, necessitating better understanding of hemostatic strategies.

Purpose of the Study:

• To review the pathophysiology and laboratory monitoring of RBDs relevant to CV surgery.
• To summarize available biological and pharmacological factor replacement agents.
• To establish a framework for perioperative coagulation management in RBD patients undergoing CV surgery.

Main Methods:

• Literature review focusing on pathophysiology, laboratory diagnostics, and hemostatic agents for RBDs.
• Analysis of challenges in perioperative management, including heparin anticoagulation and cardiopulmonary bypass (CPB).
• Synthesis of information to propose a management framework.

Main Results:

• RBDs do not protect against atherosclerosis, increasing the need for CV surgery in affected individuals.
• Perioperative management is complex due to factors like heparin, hemodilution, and protein consumption during CPB.
• Understanding of available hemostatic agents and their application is crucial.

Conclusions:

• A comprehensive understanding of RBD pathophysiology and available hemostatic agents is essential for safe CV surgery.
• Standardized perioperative management protocols are needed for RBD patients.
• This review provides a framework to guide clinicians in managing coagulation during CV surgery in patients with rare bleeding disorders.