Coronary artery bypass graft in a patient with Von Willebrand disease type 1

Abstract

Von Willebrand disease (VWD), is the most common hereditary bleeding disorder (HBD) and affects ap - proximately 1–2% of the population. Type 1 accounts for 70–80% of all cases and involves partially reduced levels of functional VWF. It is usually manifested by mild to moderate mucocutaneous bleeding. A 63-year-old patient with multivessel coronary artery disease and diagnosed with Von Willebrand’s disease type 1 after a previous NSTEMI infarction (10 days earlier) was admitted to the cardiac surgery department for a planned CABG procedure. The coagulation factor values on admission to the ward were successively (VIII 15%; VWF: Ag 12%). Coronary angiography revealed MV CAD. The patient was consulted with the HEART Team. The decision was made to qualify the patient for CABG using the OPCAB technique. A detailed plan for securing the haemostasis was established. Haemate P 500/1200 and 1000/2400, a lyophilized concentrate of humans VIII and von Willebrand Factors, were secured. During surgery, LITA- LAD and Ao-RCA bridges were performed using Medtronic Starfish® Heart Positioners. In the post-operation period, no complications were noted. Plasma levels of VIII and VW factors were measured daily and antihemorrhagic prophylaxis was given accordingly to measured values. The patient was discharged on the eighth day after surgery in good condition, with the haematological recommendations. Currently, there are no guidelines for the management of patients with Von Willebrand’s disease undergoing cardiac surgery. Incorrectly conducted pharmacotherapy may result in an elevated level of VW factor and additional exposure to the occurrence of acute coronary syndromes and heart attacks.