Long-term treatment of pulmonary arterial hypertension (PAH) in adult patients w/ WHO functional class II-III (including idiopathic & heritable PAH, PAH associated w/ connective tissue disorders, & PAH associated w/ corrected simple congenital heart disease), either as combination therapy in patients insufficiently controlled w/ an endothelin receptor antagonist &/or a phosphodiesterase type 5 inhibitor, or as monotherapy in patients who are not candidates for these therapies.
Initially 200 mcg bd, approx 12 hr apart. Should be up-titrated to the highest individually tolerated dose in increments of 200 mcg bd, usually at wkly intervals. Max: 1,600 mcg bd. Moderate hepatic impairment Initially 200 mcg once daily, increase at wkly intervals by increments of 200 mcg once daily.
View Uptravi overdosage for action to be taken in the event of an overdose.
Administration
May be taken with or without food: Swallow whole, do not split/crush/chew.
Contraindications
Hypersensitivity. Severe CHD or unstable angina. MI w/in the last 6 mth. Decompensated cardiac failure if not under close medical supervision. Severe arrhythmias. Cerebrovascular events (eg, transient ischaemic attack, stroke) w/in the last 3 mth. Congenital or acquired valvular defects w/ clinically relevant myocardial function disorders not related to pulmonary HTN. Concomitant use w/ strong CYP2C8 inhibitors (eg, gemfibrozil).
May cause hypotension; hyperthyroidism. Perform thyroid function tests as clinically indicated. Patients w/ pulmonary veno-occlusive disease. Concomitant use w/ moderate CYP2C8 inhibitors. Minor influence on the ability to drive & use machines. Should not be administered in patients w/ severe liver impairment (Child-Pugh class C), severe renal impairment (eGFR <30 mL/min/1.73 m2), & in patients undergoing dialysis. Women of childbearing potential should practice effective contraception while on treatment. Not recommended during pregnancy & in women of childbearing potential not using contraception. Should not be used during breast-feeding. Elderly >75 yr.
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