The diagnosis and management of pulmonary arterial hypertension (PAH) includes several advances, such as broader recognition of extra-pulmonary vascular organ system involvement, validated point-of-care clinical assessment tools, and a focus on early-initiation of multiple pharmacotherapeutics in appropriate patients. Indeed, a principal goal in PAH today is early diagnosis for prompt initiation of treatment to achieve minimal symptom burden, optimize the patient’s biochemical, hemodynamic, and functional profile, and limit adverse events. To accomplish this end, clinicians must be familiar with novel risk factors and the revised hemodynamic definition for PAH. Fresh insights into the role of developmental biology (i.e., perinatal health) may also be useful for predicting incident PAH in early adulthood. Emergent or underutilized approaches to PAH management include a novel TGF-β ligand trap pharmacotherapy, remote pulmonary artery pressure monitoring, next-generation imaging using inert gas-based magnetic resonance and other technologies, right atrial pacing, and pulmonary artery denervation. These and other PAH state-of-the-art advances are summarized here for the wider pulmonary medicine community.