Prior Auth Protocol

FDA Approved Indications:

For the treatment of pulmonary arterial hypertension (PAH), World Health Organization (WHO) Group 1 to delay disease progression.

Health Net Approved Indications and Usage Guidelines:

Confirmed diagnosis of PAH (WHO Group l)

Coverage is Not Authorized For:

Non-FDA approved indications, which are not listed in the Health Net Approved Indications and Usage Guidelines section, unless there is sufficient documentation of efficacy and safety in the published literature.

General Information:

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.
In light of potential teratogenic risks (pregnancy category X), for all female patients, Opsumit is available only through a restricted program called the Opsumit Risk Evaluation and Mitigation Strategy (REMS). Male patients are not enrolled in the REMS. Further information is available at www.OPSUMITREMS.com or 1-866-228-3546. Information on Opsumit certified pharmacies or wholesale distributors are available through Actelion Pathways at 1-866-228-3546.
WHO classifies patients into 5 groups based on etiologies of pulmonary hypertension
- Group 1 PAH: sporadic idiopathic pulmonary arterial hypertension (IPAH), heritable IPAH, PAH caused by diseases of the pulmonary arterioles and drug and toxin-induced PAH
- Group 2 PH: due to cardiac origin
- Group 3 PH: due to severe lung diseases or hypoxemia
- Group 4 PH: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
- Group 5 PH: miscellaneous, unclear causes

Therapeutic Alternatives:

Drug	Dosing Regimen	Dose Limit/ Maximum Dose
Flolan^R, Veletri^R(epoprostenol)*	Initiate chronic infusion rate at 2 ng/kg/min continuous IV infusion via central venous catheter and increase in increments of 2 ng/kg/min every 15 min until dose-limiting pharmacological effects are elicited or until an intolerance limit is established or further increases are not clinically warranted.	Titrate as needed and tolerated, avoid abrupt withdrawal. Should dose-limiting effects occur, reduce infusion rate by 2 ng/kg/min every 15 minutes.
(sildenafil citrate)* Revatio^R	Tablets and Oral Suspension: 5 mg or 20 mg PO TID taken approximately 4 to 6 hours apart Injection: 2.5 mg or 10 mg IV bolus TID	Tablets: 20 mg PO TID Injection: 10 mg IV bolus TID
Remodulin^R (treprostinil)*	Initiate at 1.25 ng/kg/min SC continuous infusion (undiluted) or IV continuous infusion (diluted). The infusion rate should be increased in increments of 1.25 ng/kg/min per week for the first four weeks then 2.5 ng/kg/min per week for the remaining duration of infusion depending on clinical response. Avoid abrupt discontinuation. Transition from Flolan: Initial Remodulin dose is 10% of the current Flolan dose. Dose should be increased as Flolan dose is decreased.	40 ng/kg/min. Titrate as tolerated. Avoid abrupt withdrawal.
Tracleer^R (bosentan)*	Initiate: 62.5 mg PO BID for 4 weeks. Maintenance: up to 125 mg PO BID (if body wt.< 40 kg and age > 12 y/o initial and maintenance is 62.5 mg PO BID)	125 mg PO BID
Ventavis^R (iloprost)*	2.5 - 5 mcg inhaled PO 6 to 9 times per day (no more than every two hours) *Do not initiate therapy in patients with systolic blood pressure (SBP) below 85 mmHg**	45 mcg (5 mcg nine times per day)
Letairis^R (ambrisentan)	5 to 10 mg PO QD	10 mg PO QD
Tyvaso^R (treprostinil)*	Initial dosage: Inhale 3 breaths PO (18 mcg) per treatment session. If 3 breaths are not tolerated, reduce to 1 or 2 breaths. Administer in 4 separate treatment sessions each day approximately four hours apart, during waking hours. Dosage should be increased by an additional 3 breaths at approximately 1-2 week intervals, if tolerated. Titrate to target maintenance dosage of 9 breaths (54 mcg) per treatment session.	9 breaths (54 mcg) QID
Adcirca^R (tadalafil)*	40 mg PO QD	40 mg PO QD
Adempas^R(riociguat)*	1 mg PO TID If hypotension is a problem, may initiate at 0.5 mg PO TID; Increase by 0.5 mg every 2 weeks as tolerated up to a max dose of 2.5 mg PO TID.	2.5 mg PO TID
Orenitram^TM(treprostinil)*	Initial dosage: 0.25 mg PO BID or 0.125 mg PO TID Increase by 0.25 or 0.5 mg PO BID or O.125 mg PO TID every 3 to 4 days to achieve optimal clinical response. Maximum studied dose was 21 mg PO BID.	21 mg PO BID

* Requires Prior Authorization

Recommended Dosing Regimen and Authorization Limit:

Drug	Dosing Regimen	Authorization Limit
Opsumit	10 mg PO QD Doses higher than 10 mg once daily have not been studied in patients with PAH and are not recommended.	Length of Benefit

Product Availability:

Tablet: 10 mg

References:

Opsumit [Prescribing Information] South San Francisco, CA: Actelion Pharmaceuticals US, Inc.; April 2015.
Micromedex^R Healthcare Series [Internet database]. Greenwood Village, CO: Thomson Healthcare. Updated periodically. Accessed June 1, 2015.
Medscape Web site. The Classification of Pulmonary Arterial Hypertension. Updated 2006. Available at: http://www.medscape.org/viewarticle/544175. Accessed June 14, 2014.
Clinical Pharmacology Web site. Available at:http://clinicalpharmacology-ip.com/default.aspx. Accessed June 1, 2015.