Abstract
Background and HypothesisThe acquired von Willebrand syndrome (AvWS), which predisposes to bleeding events, is often related to valvular heart diseases. We investigated possible implications of AvWS and factor VIII levels in patients with moderate to severe mitral regurgitation (MR) undergoing transcatheter mitral valve repair (TMVR).Methods and Results123 patients with moderate to severe MR were prospectively enrolled. Complete measurements of von Willebrand Factor activity (vWFAct), von Willebrand Factor antigen (vWFAg), and factor VIII expression before and 4 weeks after TMVR were available in 85 patients. At baseline, seven patients had a history of gastrointestinal bleeding, two patients suffered bleeding events during their hospital stay, and one patient had a bleeding 4 weeks after TMVR. Even though vWFAct, vWFAct/vWFAg ratio and vWFAg values did not change after TMVR, we observed a significantly lower vWFAct/vWFAg ratio in patients with primary MR as compared to patients with secondary MR both at baseline (p = 0.022) and 4 weeks following the TMVR procedure (p = 0.003). Additionally, patients with a mean mitral valve gradient ≥4 mmHg after TMVR had significantly lower vWFAct/vWFAg ratios as compared to patients with a mean mitral valve gradient <4 mmHg (p = 0.001).ConclusionsMR of primary etiology was associated with lower vWFAct/vWFAg ratio, hinting toward HMWM loss due to shear stress caused by eccentric regurgitation jets. In addition, morphological changes leading to postprocedural transmitral gradients ≥4 mmHg were related to lower vWFAct/vWFAg ratio 4 weeks after the procedure. Alterations of the vWFAct/vWFAg ratio in turn did not translate into a greater risk for bleeding events.
Highlights
IntroductionInherited von Willebrand Disease (vWD) represents the most common inherited bleeding disorder, which was first described by Erik von Willebrand in 1926.3 In contrast to the inherited form, acquired von Willebrand syndrome (AvWS) is a rare disorder[4] with an estimated prevalence of 0.04%, this figure might be underestimated.[5] AvWS is often associated with cardiovascular diseases especially congenital and valvular heart diseases.[6] AvWS is similar to inherited type 2A vWD showing decreased Von Willebrand factor (vWF)-dependent platelet adhesion due to a loss of high-molecular-weight von Willebrand Factor multimers.[7]
Von Willebrand factor is produced in megakaryocytes and endothelial cells and functions as a large human adhesive glycoprotein.[1,2]Inherited von Willebrand Disease represents the most common inherited bleeding disorder, which was first described by Erik von Willebrand in 1926.3 In contrast to the inherited form, acquired von Willebrand syndrome (AvWS) is a rare disorder[4] with an estimated prevalence of 0.04%, this figure might be underestimated.[5]
Heyde syndrome comprises the combination of AvWS, valvular heart disease and bleeding tendency – in particular gastrointestinal bleeding-8 as a result of an increased clearance of Von Willebrand factor (vWF) related to shear-induced proteolysis of high-molecular-weight vWF multimers when passing the abnormal valve.[9,10]
Summary
Inherited von Willebrand Disease (vWD) represents the most common inherited bleeding disorder, which was first described by Erik von Willebrand in 1926.3 In contrast to the inherited form, acquired von Willebrand syndrome (AvWS) is a rare disorder[4] with an estimated prevalence of 0.04%, this figure might be underestimated.[5] AvWS is often associated with cardiovascular diseases especially congenital and valvular heart diseases.[6] AvWS is similar to inherited type 2A vWD showing decreased vWF-dependent platelet adhesion due to a loss of high-molecular-weight von Willebrand Factor multimers.[7]. Heyde syndrome comprises the combination of AvWS, valvular heart disease and bleeding tendency – in particular gastrointestinal bleeding-8 as a result of an increased clearance of vWF related to shear-induced proteolysis of high-molecular-weight vWF multimers when passing the abnormal valve.[9,10]. The acquired von Willebrand syndrome (AvWS), which predisposes to bleeding events, is often related to valvular heart diseases. Alterations of the vWFAct/vWFAg ratio in turn did not translate into a greater risk for bleeding events
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