Pulmonary arterial hypertension: Focused therapeutic update

PURPOSE: The purpose of this publication is to outline evidence-based approaches to the diagnosis, assessment, and treatment of pulmonary arterial hypertension (PAH) utilizing contemporary guidelines and clinical trials. SUMMARY: PAH is a rare, progressive cardiopulmonary disease hallmarked by elevations in pulmonary vascular resistance leading to right heart failure. PAH is distinct from other forms of pulmonary hypertension, which include 4 additional groups with different hemodynamic and pathophysiologic mechanisms. Diagnosis of PAH requires exclusion of common cardiac and pulmonary disorders and is confirmed by right heart catheterization with distinct hemodynamic criteria. Disease severity and prognosis in PAH are evaluated using multidimensional assessment tools, which guide evidence-based medication selections. The landscape for evidence-based PAH treatment has evolved over the past decade and includes agents within the nitric oxide, endothelin, prostacyclin, and activin signaling pathways. The standard of care includes initiating a dual oral combination or a 3-drug combination including a parenteral agent at diagnosis in patients with group 1 PAH in those with low- to intermediate- or high-risk status, respectively. Reassessment and modification of the PAH medication regimen includes adding agents from different pathways or switching within a treatment pathway for those agents with clinical trials demonstrating clinical improvements with these approaches. CONCLUSION: The management of PAH has progressed significantly over the past decade with the availability of new treatment approaches and novel therapeutics. It is imperative for healthcare professionals caring for patients with PAH to stay abreast of the current management approaches in this population to improve patient outcomes in this rare disease.