SELEXIPAG TAB
Clinical Criteria Summary
Exclusion Criteria
- Concomitant use with strong CYP2C8 inhibitors (e.g., gemfibrozil)
- Concomitant use with other prostacyclins such as epoprostenol, treprostinil, and iloprost
Inclusion Criteria
- Definitive diagnosis of PAH confirmed by right-heart catheterization including hemodynamic diagnosis: mean pulmonary artery pressure [mPAP] >25 mmHg, pulmonary artery wedge pressure [PAWP] or left ventricular end diastolic pressure [LVEDP] ≤15 mm Hg, and pulmonary vascular resistance (PVR) >3 Wood units
- World Health Organization (WHO) Group 1 PAH
- Under the care of a VA authorized, experienced provider in the management of PAH
- Patient is treated with or has been assessed for adjunct therapies such as oxygen, diuretics, digoxin and oral anticoagulant
- If acute vasoreactivity test positive, calcium channel antagonist therapy has been tried unless right heart failure is present
- Inadequate response (e.g., progression, deterioration) or a documented contraindication or inability to tolerate a (phosphodiesterase type 5) PDE-5 inhibitor AND an endothelin receptior antagonist (ERA) prior to consideration of selexipag
Dosage and Administration
- Initial dose: 200 mcg orally twice daily
- Titration: Increase dose by 200 mcg twice daily at weekly intervals to the highest tolerated dose, up to a maximum of 1600 mcg twice daily
- Interruptions/Discontinuations: If medication is missed for 3 days or longer, restart at a reduced dose and re-titrate
Efficacy and Patient Selection
- Efficacy established in patients with WHO Group 1 PAH and functional class II or III symptoms
- Not established for patients with functional class IV symptoms; continuous infusion of a prostacyclin (e.g., intravenous epoprostenol) remains preferred therapy for severely ill patients
- Treatment of non-WHO Group 1 pulmonary hypertension is aimed at the underlying disease; current evidence does not support use in non-WHO Group 1 PAH
Safety and Tolerability
- Safety profile consistent with other systemic vasodilators; primarily affects tolerability (e.g., headache, diarrhea, jaw pain, nausea, myalgia, vomiting, extremity pain, flushing, arthralgia, rash)
- Initiated at a low starting dose and titrated slowly as tolerated
Combination Therapy
- Combination therapy with prostacyclins and selexipag has not been studied
- Appropriateness of combination PAH therapy should be assessed by a provider who specializes in PAH, considering potential drug interactions, potentially additive adverse effects, and current evidence
Renewal and Monitoring
- Routine evaluation by the PAH provider to assess both effectiveness and tolerability is mandatory
- Patients with a poor response should be considered for continuous infusion of prostacyclin
- Provider must specify criteria (e.g., NYHA functional class assessment) and a timepoint (e.g., 1, 3, or 6 months) for evaluation and continuation of treatment prior to beginning therapy