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Maintaining the balance of BMPR2/BMP and TGFβ pathways is critical for normal vascular homoeostasis. Shifting the balance in disease in these pathways will be critical in reducing/reversing the vascular remodelling associated with PAH pathobiology. The central pathways targeted by the therapeutics in this review are detailed, but these may not be exclusive as many will have multiple benefits. EC, endothelial cells; SMC, smooth muscle cells; Fibs, fibroblasts.
