The discovery of novel signaling pathways-such as the TGF-β superfamily, growth factors, and tyrosine kinases-has advanced the understanding of PAH and opened opportunities for disease-modifying therapies. Targeting these pathways, including BMPR2 restoration, TGF-β inhibition, and tyrosine kinase blockade, has shown encouraging results beyond the vasodilator-focused standard of care. These innovations may reshape PAH management by improving outcomes, and potentially altering disease progression.