Tricuspid Valve Replacement for a Patient with Corrected Congenital Transposition of the Great Vessels and Protein C Deficiency / 日本心臓血管外科学会雑誌

ABSTRACT

Protein C (PC) deficiency is an inherited thrombotic disorder with a prevalence of 0.19% among the general population. PC deficiency is associated with an increased risk of thrombosis when other risk factors are present, such as trauma, surgery, or infection, and is an important cause of mechanical valve thrombosis. We performed tricuspid valve replacement with a 29mm Carpentier-Edwards Perimount valve in a 20-year-old man with PC deficiency. The patient had corrected transposition of the great vessels with severe tricuspid insufficiency, as well as a history of cerebral infarction. In the perioperative period, we used only heparin sodium as the anticoagulant. When we restarted administration of warfarin, changing over from heparin, transient increases of serum plasmin inhibitor-plasmin complex (PIC) and thrombin antithrombin complex (TAT) levels were observed. Despite an increased dose of heparin, an appropriate activated partial thromboplastin time (APTT) was not obtained. This suggested a hypercoagulatory state, but the postoperative course was uneventful. Management of perioperative anticoagulation, prevention of late thrombotic events, and prosthetic valve selection in this particular situation are discussed.