Pulmonary arterial hypertension in the emergency department: A focus on medication management

Pulmonary arterial hypertension (PAH) is a chronic progressive incurable condition associated with a high degree of morbidity and mortality. With over five drug classes FDA approved in the last decade, the significant advancements in the pharmacologic management of PAH has improved long-term outcomes. Drug therapies have been developed to directly target the underlying pathogenesis of PAH including phosphodiesterase type-5 inhibitors (PDE-5i), endothelin-receptor antagonists (ERAs), guanylyl-cyclase inhibitors, prostacyclin analogues, and prostacyclin receptor agonists. Although these agents offer remarkable benefits, there are significant challenges with their use such as complexities in medication dosing, administration, and adverse effects. Given these consequences, PAH medications are classified as high-risk, and the transitions of care process to and from the hospital setting are a vulnerable area for medication errors in this population. Thus, it is crucial for the emergency department provider to appropriately identify, manage, and triage these patients through close collaboration with a multidisciplinary team to ensure safe and effective medication management for PAH patients in the acute care setting.