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Prior Authorization Protocol
FLOLANR, VELETRIR (epoprostenol), ORENITRAMTM, REMODULINR, TYVASOR (treprostinil), VENTAVISR (iloprost), UPTRAVIR (selexipag)


NATL

Interim Guidelines; Final Review and Approval by the P&T Committee Pending

Coverage of drugs is first determined by the member’s pharmacy or medical benefit. Please consult with or refer to the Evidence of Coverage document.
  1. FDA Approved Indications:
    Flolan:
    • Treatment of pulmonary arterial hypertension (World Health Organization (WHO) Group 1) to improve exercise capacity.
    Veletri:
    • Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity.
    Orenitram:
    • Treatment of pulmonary arterial hypertension (PAH) (WHO Group1) to improve exercise capacity.
    Remodulin:
    • Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to diminish symptoms associated with exercise.
    • For patients who require transition from Flolan, to reduce the rate of clinical deterioration.
    Tyvaso:
    • Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise ability.
    Ventavis:
    • Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve a composite endpoint consisting of exercise tolerance, symptoms (New York Heart Association (NYHA) Class) and lack of deterioration.

    Uptravi: 

    • Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to delay disease progression and reduce the risk of hospitalization for PAH. 
       
  2. Health Net Approved Indications and Usage Guidelines:
    • Confirmed diagnosis of PAH (WHO Group l)
  3. 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.
  4. General Information:
    • 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: CTEPH
      • Group 5 PH: miscellaneous, unclear causes
    • Studies establishing effectiveness of Flolan included predominantly patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue diseases.
    • Studies establishing the effectiveness of Veletri included predominantly patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH or PAH associated with connective tissue diseases.
    • The study that established the effectiveness of Orenitram included predominantly patients with WHO functional class II-III symptoms and etiologies of idiopathic or heritable PAH (75%) or PAH associated with connective tissue disease (19%).
    • Studies establishing effectiveness of Remodulin included patients with NYHA Functional Class II- IV symptoms and etiologies of idiopathic or heritable PAH (58%), PAH associated with congenital systemic-to-pulmonary shunts (23%), or PAH associated with connective tissue diseases (19%)
    • Studies establishing effectiveness of Tyvaso included predominately patients with NYHA Functional Class III symptoms and etiologies of idiopathic or heritable PAH (56%) or PAH associated with connective tissue diseases (33%).
    • Studies establishing effectiveness of Ventavis included predominately patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH (65%) or PAH associated with connective tissue diseases (23%).
    • Studies establishing effectiveness of Uptravi included patients with symptomatic (WHO Functional Class I [0.8%], II [46%], III [53%], and IV [1%] ) PAH. Idiopathic or heritable PAH was the most common etiology in the study population (58%) followed by PAH associated with connective tissue disease (29%), PAH associated with congenital heart disease with repaired shunts (10%), drugs and toxins (2%), and HIV (1%).
    • The chronic use of Flolan and Veletri in patients with congestive heart failure due to severe left ventricular systolic dysfunction is contraindicated. Flolan and Veletri should not be used chronically in patients who develop pulmonary edema during dose initiation.
    • Orenitram is contraindicated in patients with severe hepatic impairment (Child Pugh Class C).
    • Ventavis should not be initiated in patients with systolic blood pressure below 85 mmHg.
    • Ventavis is intended to be used with the I-neb AAD System. Ventavis is available through specialty pharmacies. The Ventavis patient enrollment phone number is 1-866-228-3546.
  5. Therapeutic Alternatives:
    Drug Dosing Regimen Dose Limit/ Maximum Dose

    LetairisTM (ambrisentan)

    5 to 10 mg PO QD

    10 mg PO QD

    RevatioTM (sildenafil citrate)*
    Tablets and Oral Suspension:
    5 mg or 20 mg PO TID
    Injection:
    2.5 mg or 10 mg IV bolus TID
    Tablets:
    20 mg PO TID
    Injection:
    10 mg IV bolus TID

    TracleerR (bosentan)

    Initiate:
    62.5 mg PO BID for 4 weeks.
    Maintenance:
    Up to 125 mg PO BID

    125 mg PO BID

    AdcircaTM (tadalafil)*

    40 mg PO QD

    40 mg PO QD

    OpsumitR(macitentan)*

    10 mg PO QD

    10 mg PO QD

    AdempasR(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

    * Requires Prior Authorization
  6. Recommended Dosing Regimen and Authorization Limit:
    Drug Dosing Regimen Authorization Limit

    Flolan
    Veletri

    Initiate chronic infusion rate at 2 ng/kg/min continuous IV infusion via central catheter and increase in increments of 2 ng/kg/min every 15 min
    until dose-limiting pharmacological effects are elicited or until a tolerance limit is established or further increases are not clinically warranted.

    6 months or to member's renewal period,
    whichever is longer.

    Ventavis

    2.5 - 5 mcg inhaled 6 to 9 times per day (no more than every two hours)
    Maximum daily dose:
    45 mcg (5 mcg nine times per day)

    6 months or to member's renewal period,
    whichever is longer.

    Tyvaso

    Initial dosage:
    3 breaths (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

    Length of Benefit

    Remodulin

    Initial Therapy:
    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.
    6 months or to member's renewal period,
    whichever is longer.

    Orenitram

    Initial dosage: 0.25 mg PO BID
    Increase by 0.25 or 0.5 mg BID every 3 to 4 days to achieve optimal clinical response. Maximum studied dose was 21 mg PO BID.

    Length of Benefit

    Uptravi

    Initial dosage: 200 mcg PO BID
    Increase the dose by 200 mcg BID at weekly intervals to the highest tolerated dose up to 1600 mcg BID.

    Length of benefit

  7. Product Availability:
    Flolan Powder for reconstitution: 0.5 mg and 1.5 mg
    Veletri Powder for reconstitution: 0.5 mg and 1.5 mg
    Orenitram Tablet: 0.125 mg, 0.25 mg, 1 mg, and 2.5 mg
    Remodulin for Injection:20 mL vial containing 20 mg (1 mg/ml), 50 mg (2.5 mg/ml), 100 mg (5 mg/ml), or 200 mg (10 mg/mL).
    Tyvaso Solution for oral inhalation: 2.9 mL ampule containing 1.74 mg treprostinil (0.6 mg per mL) 
    Ventavis Inhalation Solution: 1 mL ampules (10 mcg iloprost per 1 mL and 20 mcg iloprost per 1 mL)
    Uptravi Tablet: 200 mcg, 400 mcg, 600 mcg, 800 mcg, 1000 mcg, 1200 mcg, 1400 mcg, 1600 mcg
  8. References:
    1. Flolan [Prescribing Information] Research Triangle Park, NC: GlaxoSmithKlein; April 2015.
    2. Veletri [Prescribing Information] South San Francisco, CA: Actelion Pharmaceuticals US, Inc.; March 2015.
    3. Orenitram [Prescribing Information] Research Triangle Park, NC: United Therapeutics; October 2014.
    4. Uptravi [Prescribing Information]. South San Francisco, CA: Actelion Pharmaceuticals US, Inc.; December 2015.
    5. Remodulin [Prescribing Information] Research Triangle Park, NC: United Therapeutics; December 2014.
    6. Tyvaso [Prescribing Information] Research Triangle Park, NC: United Therapeutics; August 2014.
    7. Ventavis [Prescribing Information]. South San Francisco, CA: Actelion Pharmaceuticals US, Inc.; January 2015.
    8. Epoprostenol. American Hospital Formulary Service Drug Information. Available at: http://www.medicinescomplete.com/mc/ahfs/current/. Accessed June 4, 2015.
    9. Treprostinil. American Hospital Formulary Service Drug Information. Available at: http://www.medicinescomplete.com/mc/ahfs/current/. Accessed June 4, 2015.
    10. Iloprost. American Hospital Formulary Service Drug Information. Available at: http://www.medicinescomplete.com/mc/ahfs/current/. Accessed June 4, 2015.
    11. MicromedexR Healthcare Series [Internet database]. Greenwood Village, CO: Thomson Healthcare. Updated periodically. Accessed January 2016.
    12. Medscape Web site. The Classification of Pulmonary Arterial Hypertension. Updated 2006. Available at: http://www.medscape.org/viewarticle/544175. Accessed January 6, 2016.
    13. Clinical Pharmacology Web site. Available at: http://clinicalpharmacology-ip.com/default.aspx. Accessed January 6, 2016.
The material provided to you are guidelines used by this plan to authorize, modify or determine coverage for persons with similar illnesses or conditions. Specific care and treatment may vary depending on individual need and the benefits covered under your contract.