VWF and pulmonary hypertension: The current findings that a proportion of pulmonary EC express FVIII and vWF on the cell surface, together with recent evidence that resting EC can constitutively express membrane constituents involved in sustained FVIII/IXa-dependent activation of FX [42], lead us to hypothesise that dysregulated pulmonary endothelial FVIII processing may contribute to exuberant local intravascular thrombus formation in these pulmonary hypertension phenotypes.