Strauss ER, et al. - The goals of this overview are to summarize pathophysiology of rare bleeding disorders (RBDs) and laboratory monitoring pertinent to cardiovascular (CV) surgery, available factor replacement agents, and to provide the framework for perioperative coagulation management of RBD patients. The obtained data reveal that perioperative management of RBDs in CV surgery is further complicated by heparin anticoagulation, haemodilution, and consumption of procoagulant and anticoagulant proteins associated with cardiopulmonary bypass (CPB).