Pulmonary Arterial Hypertension (PAH) Drug Therapy Coverage Criteria
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This policy governs coverage criteria for drug therapies used to treat pulmonary arterial hypertension (WHO Group 1) and related pulmonary hypertension indications for members of Blue Cross Blue Shield - Iowa (Wellmark).
No material clinical or coverage changes in this revision.
Coverage Criteria and Approval Rules
General coverage stance
Covered when uses match FDA-approved indications and all policy approval criteria are met.
See individual drug criteria for specific diagnostic and pretreatment requirements (e.g., right heart catheterization hemodynamics).
Adempas (riociguat) PAH
Authorization of 12 months may be granted when ALL of the following are met:
Authorization duration = 12 months
Adempas (riociguat) CTEPH
Authorization of 12 months may be granted when ALL of the following are met:
Authorization duration = 12 months
PAH therapies (ambrisentan, macitentan, prostacyclins, treprostinil, bosentan, iloprost, etc.)
Authorization of 12 months may be granted for treatment of PAH when ALL of the following are met:
Applies to listed PAH therapies including endothelin receptor antagonists, prostacyclins, phosphodiesterase‑5 inhibitors, treprostinil formulations, iloprost, and related agents. Authorization duration = 12 months.
Opsynvi (macitentan;tadalafil) special criterion
Authorization of 12 months may be granted when ALL of the following are met:
Combination product requires documented failure/intolerance to separate components; authorization duration = 12 months.
Secondary Raynaud's phenomenon (tadalafil, sildenafil)
Authorization of 12 months may be granted when ALL of the following are met:
Applies to tadalafil and sildenafil when used for secondary Raynaud's; authorization duration = 12 months.
Remodulin (treprostinil) - Initial Therapy
Remodulin (treprostinil) — Authorization of 12 months when ALL of the following are met
Authorization duration = 12 months; includes IV/SC Remodulin formulations and guidance on transitions from epoprostenol when clinically indicated.
Tracleer (bosentan) - Initial Therapy
Tracleer (bosentan) — Authorization of 12 months when ALL of the following are met
Authorization duration = 12 months; also includes criteria for PH associated with interstitial lung disease (WHO Group 3) with similar hemodynamic confirmation.
Uptravi (selexipag) - Initial Therapy
Uptravi (selexipag) — Authorization of 12 months when ALL of the following are met
Authorization duration = 12 months
Winrevair (sotatercept) - Initial Therapy
Winrevair (sotatercept) — Authorization of 6 months for adults when ALL of the following are met
Authorization duration = 6 months; initial prior authorization requires documentation of current background PAH therapies and baseline labs.
Continuation and Long-Term Therapy
Continuation and long-term therapy
Applies to multiple agents; typical initial/continuation authorizations are 12 months for most agents.
Requires evidence of continued benefit (stability or improvement)
Other Indications - Continuation
Other covered indications with continuation criteria
Authorization duration = 12 months
Authorization duration = 12 months
Authorization duration = 12 months
This policy may not apply to the Federal Employee Program (FEP). Benefit determinations are based on the applicable contract language in effect at the time of service; exclusions, limitations, or exceptions may apply and individual member benefits must be verified. Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable.
For Opsynvi (macitentan; tadalafil), authorization requires documented inadequate response, intolerance, or contraindication to combined use of the individual ingredients given separately. Treatment failure due to noncompliance or unwillingness to take the two ingredients separately is not an acceptable rationale for using the fixed-dose combination. Standard PAH diagnostic and hemodynamic confirmation criteria also apply.
Drugs listed in this policy are considered not medically necessary for members who do not meet the specific criteria set forth in the policy. Members currently receiving medications as samples or through manufacturer assistance programs must still meet the initial approval criteria to ensure equitable treatment.
None explicitly listed in this section.
Use of the drugs listed in this policy for patients who do not meet the specific PAH/PH diagnostic, hemodynamic, and prior-therapy requirements described in the policy is considered not medically necessary and will not be authorized.
No additional explicit 'not medically necessary' conditions are stated beyond the requirement that patients meet the policy criteria; treatments for members who do not satisfy the listed PAH/PH criteria are considered not medically necessary.
Medications, Codes, and Limits
| Winrevair | sotatercept |
| Adcirca/Alyq/Tadliq | tadalafil |
| Revatio/Liqrev | sildenafil |
| Adempas | riociguat |
| Flolan/Veletri/Epoprostenol | epoprostenol |
| Ventavis | iloprost |
| Letairis | ambrisentan |
| Opsumit | macitentan |
| Opsynvi | macitentan; tadalafil (combination) |
| Orenitram/Remodulin | treprostinil (oral/parenteral formulations) |
Provider Responsibilities, Prior Authorization, and Billing
Coverage contingent on approval criteria
Coverage of the PAH and related indications listed in this policy is contingent on meeting the policy's specific approval criteria and the member having benefits that allow coverage for the prescribed therapy. Providers must verify member benefits and plan exclusions/limitations prior to submission.
- Benefit determinations are based on the applicable contract language in effect at the time of service.
- Members currently receiving samples or manufacturer assistance must meet the same criteria as other members.
Adempas (riociguat) — PAH prior authorization
Prior authorization for Adempas (riociguat) for pulmonary arterial hypertension (PAH) may be approved for up to 12 months when PAH is confirmed and pretreatment right heart catheterization demonstrates mPAP > 20 mmHg, PCWP < 15 mmHg, and PVR > 2 Wood units, and the request otherwise meets the policy criteria.
- Authorization duration: 12 months for PAH.
- PAH must be WHO Group 1 per policy appendix and confirmed by right heart catheterization.
Adempas (riociguat) — CTEPH prior authorization
Adempas (riociguat) for chronic thromboembolic pulmonary hypertension (CTEPH) may be authorized for up to 12 months when the member has CTEPH (WHO Group 4) and meets either (a) recurrent or persistent CTEPH after pulmonary endarterectomy (PEA) confirmed by imaging, or (b) inoperable CTEPH confirmed by imaging, with pretreatment right heart catheterization showing mPAP > 20 mmHg, PCWP < 15 mmHg, and PVR > 2 Wood units.
- Authorization duration: 12 months for CTEPH.
- Imaging required: CT/MR angiography or pulmonary angiography when applicable.
- Right heart catheterization pretreatment values required as specified.
PAH therapies — prior authorization and general requirements
Prior authorization of 12 months (typical initial authorization) may be granted for multiple PAH therapies when the member meets the specific drug criteria in this policy (e.g., tadalafil products, sildenafil products, epoprostenol products, prostacyclin analogs and receptor agonists, endothelin receptor antagonists, soluble guanylate cyclase stimulators, macitentan, treprostinil, bosentan, selexipag, etc.). Continuation and longer authorizations are described elsewhere in the policy.
- Typical initial authorizations: 12 months for most agents; Winrevair (sotatercept) initial authorization = 6 months.
- Continuation authorizations: see policy sections for durations (some agents eligible for longer continuation approvals).
- Prescriptions for pulmonary hypertension indications should be prescribed by or in consultation with a pulmonologist.
- Refer to individual drug criteria (right heart catheterization, WHO Group, pediatric exceptions) in policy.
Combination product failure requirement
Combination-product requests (e.g., combination macitentan/tadalafil) will be denied unless the member has demonstrated inadequate response, intolerance, or contraindication to separate use of the individual components. Treatment failure due to noncompliance or unwillingness to take ingredients separately does not meet this requirement.
- Documentation must show trial and failure, intolerance, or contraindication to individual ingredients.
- Failure cannot be attributed to lack of compliance or refusal to take agents separately.
Drugs considered not medically necessary when criteria not met
Drugs listed in this policy are considered not medically necessary for patients who do not meet the criteria set forth in the relevant drug-specific sections. Members receiving samples or in manufacturer assistance programs must still meet the criteria for initial approval.
- Requests not meeting the policy criteria may be denied as not medically necessary.
- Members receiving samples or patient assistance must meet the same initial criteria as other members.
Quantity limit exceedance — prior authorization required
Requests that exceed the specified standard quantity limits for PAH medications are subject to denial or require additional prior authorization documentation. Providers should adhere to the policy's listed quantity limits for each product and submit justification if higher amounts are requested.
- Examples of standard quantity limits: Tracleer tablets limits per 28/30 days; Tyvaso and Tyvaso DPI cartridge limits per 28 days; Uptravi tablet limits per 28/30 days; Winrevair (sotatercept) standard limit = kit per 21 days, etc.
- Requests exceeding these limits require prior authorization and clinical justification.
Provider responsibility and documentation
Providers are responsible for verifying member benefits and may be asked to provide clinical documentation to support medical necessity. Required documentation may include chart notes, medical records, prior treatment history, imaging and diagnostic reports, and claims history as applicable.
- Benefit verification is required prior to service; exclusions/limitations may apply.
- Non-formulary exception requests must include documentation that formulary alternatives were ineffective, not tolerated, or contraindicated, with dosage and duration details.
- Diagnostic testing/lab results required when applicable.
Winrevair (sotatercept) — documentation for initial authorization
For initial prior authorization requests for Winrevair (sotatercept), submit chart notes, supporting medical record documentation, or claims history that demonstrates the member is currently receiving PAH therapy and meets the sotatercept-specific criteria (including platelet count and current use of medications from at least two other PAH drug classes).
- Required for Winrevair initial authorization: documentation of current PAH therapy and prior treatments from at least two of the listed drug classes.
- Include platelet count documentation (must be >= 50,000/mm3) and evidence that sotatercept will be used as add-on therapy.
- Winrevair initial authorization duration: 6 months; standard limit: kit per 21 days.
Diagnostic testing / laboratory results required
Diagnostic testing and laboratory results are required when applicable to support medical necessity (for example, right heart catheterization results, platelet counts for sotatercept, pregnancy testing when indicated, and imaging for CTEPH). Include pretreatment hemodynamic measurements and other test results in the medical record submitted for review.
- Right heart catheterization pretreatment values commonly required: mPAP, PCWP, and PVR (or PVRI for pediatric patients).
- Platelet count requirement for sotatercept: >= 50,000/mm3.
- Imaging required for CTEPH: CT/MR angiography or pulmonary angiography when applicable.
Add-on therapy requirement for Winrevair (sotatercept)
Winrevair (sotatercept) may be approved only as add-on therapy to existing PAH treatment. The member must be currently receiving PAH therapy with medications from at least two of the specified PAH drug classes and meet all sotatercept-specific criteria (including platelet count, pregnancy status, and age requirements).
- Add-on requirement: sotatercept will be used in addition to current PAH therapy.
- Member must be >= 18 years old for sotatercept per policy and not pregnant or planning pregnancy.
- Initial authorization for sotatercept: 6 months; continuation criteria detailed in policy.
Clinical Background and Scope
Pulmonary arterial hypertension (PAH, WHO Group 1) is managed with multiple drug classes including endothelin receptor antagonists, phosphodiesterase‑5 inhibitors, soluble guanylate cyclase stimulators, prostanoids, and newer agents such as activin signaling inhibitors. These therapies are indicated to improve exercise capacity, WHO/NYHA functional class, and/or reduce clinical worsening in patients with PAH. Clinical trials informing approvals predominantly enrolled patients with WHO/NYHA functional class II–III symptoms and etiologies such as idiopathic, heritable, or connective tissue disease–associated PAH.
Definitions and Classification
Policy Revision History
Policy effective date updated to 2025-08-09.
Clinical review completed with last_review recorded as 2025-06-01.
Winrevair (sotatercept) initial authorization length specified as 6 months for adults and add-on therapy requirement clarified.
Adempas (riociguat) authorization criteria reaffirmed with hemodynamic thresholds (mPAP >20 mmHg; PCWP <15 mmHg; PVR >2 Wood units) for PAH and CTEPH indications.
Continuation and long-term therapy rules clarified, including typical 12-month initial authorizations and 36-month authorization for specified long-term agents.
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