Treatment and Palliation of Symptoms in Patients With Advanced Pulmonary Arterial Hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a complex disease in which the illness as well as the associated treatment can be particularly burdensome. Previously considered to be uniformly fatal, PAH treatment and management has improved with an extended survival. However, this can come with symptom burden. Burden can be associated with the disease itself in addition to the treatment aimed at ameliorating these symptoms. This article explores both the use of PAH-directed therapy as well as traditionally supportive treatment strategies for palliation of symptoms in those with PAH. As highlighted in the patient case described throughout this issue, patients are constantly weighing the benefits and burdens of treatment during each step of their journey.

Real‐world treatment patterns, healthcare resource utilization, and cost among adults with pulmonary arterial hypertension in the United States

“…Medications that target the endothelin pathway include three endothelin receptor antagonists (ERAs): bosentan, ambrisentan, and macitentan. Prostacyclin and IP receptor agonists target the prostacyclin pathway and are available parenterally through a continuous infusion either intravenously (epoprostenol and treprostinil) or subcutaneously (treprostinil), or via inhalation (iloprost and treprostinil) or orally (treprostinil and selexipag) 10 . Various multiparametric risk assessment tools are used to predict survival in people with PAH and to guide treatment decisions with an aim for achieving a low risk status 11,12 …”

“…Prostacyclin and IP receptor agonists target the prostacyclin pathway and are available parenterally through a continuous infusion either intravenously (epoprostenol and treprostinil) or subcutaneously (treprostinil), or via inhalation (iloprost and treprostinil) or orally (treprostinil and selexipag). 10 Various multiparametric risk assessment tools are used to predict survival in people with PAH and to guide treatment decisions with an aim for achieving a low risk status. 11,12 Because three signaling pathways can be targeted by the currently available medications, increasing attention has been placed on using combination therapy, the use of two or more classes of drugs simultaneously, for the treatment of PAH.…”