PAH, or pulmonary arterial hypertension, is a disease resulting from restricted flow through the pulmonary arterial circulation that leads to increased pulmonary vascular resistance and, ultimately, right heart failure.2
Clinically, PAH is defined by an increase in mean pulmonary arterial pressure (PAP) to ≥25 mmHg at rest, a mean pulmonary arterial wedge pressure of ≤15 mmHg, and an elevated pulmonary vascular resistance (PVR) >3 Wood units.3
Early in the disease, PVR and PAP may be elevated, but due in part to RV compensation, CO remains within normal range for an indeterminate time. Patients may not be diagnosed until they present with overt symptoms, such as dyspnea, fatigue, and edema in the lower extremities.6,8
OPSUMIT is an endothelin receptor antagonist (ERA) indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression. Disease progression included: death, initiation of intravenous (IV) or subcutaneous prostanoids, or clinical worsening of PAH (decreased 6-minute walk distance, worsened PAH symptoms and need for additional PAH treatment). OPSUMIT also reduced hospitalization for PAH.
Effectiveness was established in a long-term study in PAH patients with predominantly WHO Functional Class II-III symptoms treated for an average of 2 years. Patients were treated with OPSUMIT monotherapy or in combination with phosphodiesterase-5 inhibitors or inhaled prostanoids. Patients had idiopathic and heritable PAH (57%), PAH caused by connective tissue disorders (31%), and PAH caused by congenital heart disease with repaired shunts (8%).
Pregnancy: OPSUMIT may cause fetal harm when administered to a pregnant woman. OPSUMIT is contraindicated in females who are pregnant. If OPSUMIT is used during pregnancy, apprise the patient of the potential hazard to a fetus.