Medical Policy

Effective Date:09/20/2005 Title:Pulmonary Artery Hypertension Oral Therapy
Revision Date:01/01/2017 Document:BI119:00
CPT Code(s):J1325; J3285; J7686; Q4074
Public Statement

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Revatio (Sildenafil Citrate), Tracleer (Bosentan), Letairis (Ambrisentan), Adcirca (Tadalafil), Adempas (Riociguat), Flolan (Epoprostenol), Veletri (Epoprostenol), Ventavis (Iloprost), Remodulin (Treprostinil), Tyvaso (Treprostinil), Uptravi (Selexipag) and Opsumit (Macitentan) have been approved by the FDA for the treatment of severe heart and lung disease known as pulmonary arterial hypertension and are covered by the plan subject to the criteria below.

2)    All drugs for pulmonary arterial hypertension require prior authorization.

3)    Orenitram (Treprostinil) oral tablets are not covered by the plan.

4)    The oral drugs are covered under the pharmacy benefit as specialty drugs and must be obtained through the specialty pharmacy. The inhaled and infused drugs are covered under the medical benefit.

Medical Statement

There are four (4) primary drug categories addressed in this policy that are approved to treat pulmonary arterial hypertension (PAH).  Coverage criteria for each of the drugs within each category are as below and prescribed by a pulmonologist or cardiologist.

1)    Phosphodiesterase Type 5 (PDE-5) inhibitors

a.     Revatio (Sildenafil)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class II-III symptoms  AND

                                         ii.     Confirmed by right heart catheterization  AND

                                       iii.     No concurrent nitrate therapy  AND

                                       iv.     Patient 18 years of age or older AND

                                        v.     If prescribing oral tablets, only generic is covered AND

                                       vi.     If prescribing oral suspension, must document why generic oral tablet cannot be used  AND

                                     vii.      If prescribing IV dosage form, must document why oral dosage form cannot be used.

b.     Adcirca (Tadalafil)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group 1, NYHA Class II-III symptoms  AND

                                         ii.     Confirmed by right heart catheterization AND

                                       iii.     No concurrent nitrate therapy  AND

                                       iv.     Patient is 18 years of age or older

2)    Endothelin Receptor Antagonists

a.     Tracleer (Bosentan)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2) , WHO Group I, NYHA Class II-IV symptoms  AND

                                         ii.     Confirmed by right heart catheterization  AND

                                       iii.     Patient is not pregnant AND

                                       iv.     Patient not receiving concomitant cyclosporine or glyburide therapy AND

                                        v.     Provider and patient enrolled in TAP Restricted Distribution Program

b.     Letairis (Ambrisentan)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class II-III symptoms  AND

                                         ii.     Confirmed by right heart catheterization  AND

                                       iii.     Patient is not pregnant AND

                                       iv.     Patient is not diagnosed with idiopathic pulmonary fibrosis AND

                                        v.     Provider and female patients are enrolled in Letairis REMS program

c.     Opsumit (Macitentan)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class II-III symptoms AND

                                         ii.     Confirmed by right heart catheterization AND

                                       iii.     Patient is not pregnant AND

                                       iv.     Provider and female patients are enrolled in Opsumit REMS program

3)    Prostacyclin Analogues/Prostacyclin Agonists

a.     Epoprostenol (Flolan, Veletri)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class III-IV symptoms AND

                                         ii.     Confirmed by right heart catheterization

b.     Ventavis (Iloprost)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class III symptoms AND

                                         ii.     Confirmed by right heart catheterization

c.     Treprostinil (Remodulin, Tyvaso, Orenitram)

                                          i.     Remodulin and Tyvaso are covered if meeting the criteria below; Orenitram is not covered.

                                         ii.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class II-III symptoms AND

                                       iii.     Confirmed by right heart catheterization

d.     Selexipag (Uptravi)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class II-III symptoms AND

                                         ii.     Confirmed by right heart catheterization

4)    Guanylate Cyclase Stimulators

a.     Adempas (Riociguat)

                                          i.     Diagnosis of pulmonary arterial hypertension (I27.0, I27.2), WHO Group I, NYHA Class II-III symptoms AND

                                         ii.     Confirmed by right heart catheterization AND

                                       iii.     Patient is not pregnant  OR

                                       iv.     Diagnosis of persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) WHO Group 4 following surgery or when surgery is not an option AND

                                        v.     Patient is not pregnant

Initial authorization shall be for six (6) months.  Reauthorization for continued use shall be reviewed at least every 12 months to confirm that the patient continues to meet coverage criteria and has achieved an objective response to therapy demonstrated by one of the following:

1)    Improvement in exercise capacity from baseline (e.g. improved 6MW, O2 saturation, etc.) OR

2)    Improvement in the signs/symptoms of PAH or lack of disease deterioration

Limits

As specialty drugs, these products are limited to no more than a 30 day supply per fill. 

Reference

1.     Tracleer Product Information. Actelion Pharmaceuticals US, Inc., South San Francisco, CA. November 2001.

2.     Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002; 346:896-903.

3.     Olin BR, editor. Drugs Facts and Comparisons (electronic online version). St. Louis: J.B. Lippincott Company, 2004.

4.     USPDI Drug Information for the HealthCare Professional (online through Stat! Ref). Thomson MICROMEDEX, Greenwood Village, Colorado; 2004.

5.     McEvoy GK, editor. AHFS Drug Information (online through Stat! Ref). American Society of Health-Systems Pharmacists, Bethesda, Maryland; 2004.

6.     Medical Economics, Inc., PDR Electronic Library. Thomson Medical Economics, Montvale, NJ; 2003.

7.     McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension. ACCP evidence-based clinical practice guidelines. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=15249497&dopt=Abstract

8.     Galie N, Rubin LJ. Introduction: new insights into a challenging disease. J Am Coll Cardiology 2004; 43(Suppl S):1S.

9.     Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension. ACCP evidence-based clinical practice guidelines. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=15249494&dopt=Abstract

10. Chockalingam A, Gnanavelu G, Venkatesan S, et al. Efficacy and optimal dose of sildenafil in primary pulmonary hypertension. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=15721505&dopt=Abstract

11. FDA Approval letter for Adcirca at: http://www.accessdata.fda.gov/drugsatfda_docs/appletter/2009/022332s000ltr.pdf

12. Adempas Package Insert. Bayer. October 2013.

13. Opsumit Package Insert. Actelion Pharmaceuticals.  October 2013.

14. Clinical Pharmacology. Accessed online September 2016.

15. Evidence-based Practice Center Systematic Review Protocol; Project Title: Pulmonary Arterial Hypertension: Screening, Management, and Treatment.  Available at www.effectivehealthcare.ahrq.gov and accessed on May 27, 2015.

16. Uptravi Package Insert. Actelion Pharmaceuticals. December 2015.

 

Addendum:

Effective 01/01/2017: Added new drug (Uptravi) to criteria

Application to Products

This policy applies to all health plans and products administered by QualChoice, both those insured by QualChoice and those that are self-funded by the sponsoring employer, unless there is indication in this policy otherwise or a stated exclusion in your medical plan booklet.  Consult the individual plan sponsor Summary Plan Description (SPD) for self-insured plans or the specific Evidence of Coverage (EOC) or Certificate of Coverage (COC) for those plans or products insured by QualChoice.  In the event of a discrepancy between this policy and a self-insured customer’s SPD or the specific QualChoice EOC or COC, the SPD, EOC, or COC, as applicable, will prevail.  State and federal mandates will be followed as they apply.


Changes: QualChoice reserves the right to alter, amend, change or supplement benefit interpretations as needed.