What is the most appropriate next step for a PAH patient demonstrating severe symptoms and deteriorating functional capacity?

In the management of pulmonary arterial hypertension (PAH), patients who are showing severe symptoms and have a deteriorating functional capacity require immediate and intensive treatment. This urgency stems from the significant impact that PAH can have on a patient’s quality of life and overall health, as well as the potential for rapid clinical decline.

Initiating intravenous epoprostenol therapy is an appropriate next step because this medication is a prostacyclin analog that has potent vasodilatory effects and the ability to inhibit platelet aggregation. Epoprostenol is often considered a standard of care for patients with severe PAH due to its ability to significantly improve hemodynamic parameters and functional capacity rapidly. The intravenous route allows for higher and more controlled dosing, which is especially crucial in a patient who is experiencing severe symptoms.

Switching to oral sildenafil or starting a new oral endothelin receptor antagonist may not provide the immediate response needed in a patient with severe symptoms. While these treatments can be part of the overall management plan for PAH, they generally take longer to exert their effects compared to intravenous therapies. Increasing the dose of inhaled treprostinil could also have limited efficacy in rapidly improving a patient’s condition, depending on their current dosage and response to the medication.

Thus