Pulmonary Hypertension

•Persistent/recurrent Chronic Thromboembolic Pulmonary Hypertension (CTEPH) (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class.

•Pulmonary Arterial Hypertension (PAH) (WHO Group 1) to improve exercise capacity, improve WHO functional class and to delay clinical worsening.

DOSAGE AND ADMINISTRATION
•Initiate treatment at 1 mg taken three times a day.

•For patients who may not tolerate the hypotensive effect of Adempas, consider a starting dose of 0.5 mg, three times a day.

•Increase dosage by 0.5 mg at intervals of no sooner than 2-weeks as tolerated to a maximum of 2.5 mg three times a day.

DOSAGE FORMS AND STRENGTHS
Tablets: 0.5 mg, 1 mg, 1.5 mg, 2 mg and 2.5 mg

WARNING: EMBRYO-FETAL TOXICITY
See full prescribing information for complete boxed warning.
•Do not administer Adempas to a pregnant female because it may cause fetal harm.

•Females of reproductive potential: Exclude pregnancy before start of treatment, monthly during treatment, and 1 month after treatment discontinuation. Prevent pregnancy during treatment and for one month after treatment discontinuation by use of acceptable methods of contraception.

•For females, Adempas is available only through a restricted program called the Adempas REMS Program.

See local monograph for additional information.

WARNINGS AND PRECAUTIONS:

1. Other ERAs cause hepatotoxicity and liver failure. Obtain baseline liver enzymes and monitor as clinically indicated.
2. Decreases in hemoglobin.
3. Pulmonary edema in patients with pulmonary veno-occlusive disease. If confirmed, discontinue treatment
4. Decreases in sperm count have been observed in patients taking ERAs

DOSAGE AND ADMINISTRATION:
 10 mg once daily. Doses higher than 10 mg once daily have not been studied in patients with PAH and are not recommended

DOSAGE FORMS AND STRENGTHS
Tablet: 10 mg

 See local monograph for additional information.

Initial U.S. Approval:  2013

Mechanism of Action: The major pharmacologic actions of treprostinil are direct vasodilation of pulmonary and systemic arterial vascular beds, inhibition of platelet aggregation, and inhibition of smooth muscle cell proliferation.

INDICATIONS AND USAGE:  Orenitram is a prostacyclin vasodilator indicated for:
Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity. The study that established effectiveness included predominately patients with WHO functional class II-III symptoms and etiologies of idiopathic or heritable PAH (75%) or PAH associated with connective tissue disease (19%). (1.1)

As the sole vasodilator, the effect on exercise is small. Orenitram has not been shown to add to other vasodilator therapy.

HOW SUPPLIED: Extended-Release Tablets: 0.125 mg, 0.25 mg, 1 mg and 2.5 mg.

Initial U.S. Approval:  2015

Mechanism of Action: Selexipag is an oral prostacyclin receptor (IP receptor) agonist that is structurally distinct from prostacyclin. Selexipag is hydrolyzed by carboxylesterase 1 to yield its active metabolite, which is approximately 37-fold as potent as selexipag. Selexipag and the active metabolite are selective for the IP receptor versus other prostanoid receptors (EP_1-4, DP, FP and TP).

INDICATIONS AND USAGE:
1.1 Pulmonary Arterial Hypertension
UPTRAVI is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression and reduce the risk of hospitalization for PAH.
Effectiveness was established in a long-term study in PAH patients with WHO Functional Class II-III symptoms.

Patients had idiopathic and heritable PAH (58%), PAH associated with connective tissue disease (29%), PAH associated with congenital heart disease with repaired shunts (10%

HOW SUPPLIED:
UPTRAVI is available in the following strengths:

– 200 mcg [Light yellow tablet debossed with 2]

– 400 mcg [Red tablet debossed with 4]

– 600 mcg [Light violet tablet debossed with 6]

– 800 mcg [Green tablet debossed with 8]

– 1000 mcg [Orange tablet debossed with 10]

– 1200 mcg [Dark violet tablet debossed with 12]

– 1400 mcg [Dark yellow tablet debossed with 14]

– 1600 mcg [Brown tablet debossed with 16]