Given that the activity of vWF (i) encompasses the interaction between platelets and endothelial cells8 and (ii) is related to its cleaving protease ADAMTS13,27 it is possible that the interplay of the vWF-ADAMTS13 axis has a role in endothelial dysfunction in PAH (Figure 3). Here, ADAMTS13 is linked to pulmonary arterial hypertension.