Abstract
von Willebrand factor (VWF) is an adhesive protein involved in primary hemostasis and facilitates platelet adhesion to sites of vascular injury, thereby promoting thrombus formation. VWF exists in plasma as multimers of increasing size, with the largest (high molecular weight; HMW) expressing the greatest functional activity. A deficiency of VWF is associated with a bleeding disorder called von Willebrand disease (VWD), whereas an excess of VWF, in particular the HMW forms, is associated with thrombosis. ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif-13), also known as VWF-cleaving protease, functions to moderate the activity of VWF by cleaving multimers of VWF and limiting the expression of the largest multimers of VWF. A deficiency of ADAMTS13 is therefore associated with an excess of (HMW forms of) VWF, and thus thrombosis. Indeed, any disturbance of the VWF/ADAMTS13 ratio or ‘axis’ may be associated with pathophysiological processes, including prothrombotic tendency. However, both thrombosis or bleeding may be associated with such disturbances, depending on the presenting events. This review evaluates the relationship of VWF and ADAMTS13 with cardiac disease, including cardiac failure, and associated pathophysiology.
Highlights
Cardiac failure is becoming increasingly common with improvements in therapeutic targets in an aging population [1]
von Willebrand factor (VWF) activity is controlled through cleavage by a disintegrin and metalloproteinase with thrombospondin type 1 motif-13 (ADAMTS13), known as VWF-cleavage protease
We aim to describe the associations of VWF, ADAMTS13, and cardiac disease in some detail
Summary
Cardiac failure is becoming increasingly common with improvements in therapeutic targets in an aging population [1]. In the main, studies reporting a single ADAMTS13 parameter report activity levels. Circulating endothelial cells have been shown to predict vascular events in patients with established coronary artery disease and affect myocardial infarct size following a myocardial ischaemic event [16,17,18]. Alterations in both VWF and ADAMTS13 levels have been implicated in patients with atrial fibrillation (AF) [19,20]. We aim to describe the associations of VWF, ADAMTS13, and cardiac disease in some detail
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