Pulmonary Arterial Hypertension (PAH) Drug Therapy — Coverage Criteria
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Criteria and coverage guidance for pharmacologic treatments of pulmonary arterial hypertension (WHO Group 1) and selected related pulmonary hypertension indications for Wellmark Blue Cross and Blue Shield members.
No material clinical or coverage changes in this revision.
Coverage Criteria and Product-specific Authorization Conditions
General coverage conditions for PAH drugs
Covered when ALL of the following are met
Supported by drug-specific indication statements in the document.
See individual drug entries for specific functional class or age details.
Hemodynamic thresholds cited across multiple agent sections.
Required prescriber specialty for PH indications.
Benefit determinations vary by contract and exclusions may apply.
Failure to submit required documentation may trigger denial.
Adempas (riociguat) — PAH
Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:
Authorization period: 12 months.
Adempas (riociguat) — CTEPH
Authorization of 12 months may be granted for treatment of CTEPH when ALL of the following criteria are met:
Authorization period: 12 months.
Tadalafil agents (Adcirca, Alyq, Tadliq) — PAH
Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:
Authorization period: 12 months.
Tadalafil agents (Adcirca, Alyq, Tadliq) — Secondary Raynaud's phenomenon
Authorization of 12 months may be granted for treatment of secondary Raynaud's phenomenon when ALL of the following are met:
Authorization period: 12 months when benefit is demonstrated.
Multiple PAH agents (Revatio, Ventavis, Epoprostenol, Letairis, Opsumit, Orenitram, Remodulin, Tracleer) — PAH
Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:
Applies to Revatio, Ventavis, epoprostenol products, Letairis, Opsumit, Orenitram, Remodulin, and Tracleer per document sections.
Opsynvi (macitentan; tadalafil) — PAH
Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:
Authorization period: 12 months.
Tyvaso, Tyvaso DPI, Yutrepia (treprostinil) — PH
Authorization of 12 months may be granted for treatment of pulmonary hypertension (PH) when both of the following criteria are met:
Tyvaso/Tyvaso DPI/Yutrepia have indications including PH-ILD; authorization period: 12 months.
Tracleer (bosentan) — Initial approval
Tracleer (bosentan) — Authorization of 12 months may be granted when ALL of the following are met
Authorization period: 12 months; pediatric dosing and limits referenced in quantity limits section.
Tyvaso / Tyvaso DPI / Yutrepia — Initial approval
Tyvaso, Tyvaso DPI, Yutrepia (treprostinil) — Authorization of 12 months may be granted when BOTH of the following are met
Authorization period: 12 months; product-specific dosing and cartridge limits are specified in quantity limits sections.
Uptravi (selexipag) — Initial approval
Uptravi (selexipag) — Authorization of 12 months may be granted when ALL of the following are met
Authorization period: 12 months; Uptravi titration and standard tablet quantity limits provided in quantity limits section.
Winrevair (sotatercept) — Initial & add-on therapy criteria
Winrevair (sotatercept) — Authorization of 6 months may be granted for treatment of PAH in members ≥18 years when ALL criteria are met
Authorization period: 6 months; age ≥18 years required; weight‑based dosing and billing guidance in quantity limits and coding sections.
Continuation therapy and condition-specific coverage
Continuation / specific-condition authorizations
Remodulin, treprostinil IV/SC, Flolan, Veletri, IV epoprostenol, and Ventavis may qualify for 36‑month authorization when benefit is demonstrated.
Covered when consistent with FDA dosing and quantity limits
Coverage aligns with FDA-recommended dosing and specified quantity limits for each product.
Providers requesting quantities above standard limits should provide clinical justification; prescriptions exceeding limits may be reviewed or denied.
This policy may not apply to the Federal Employee Program (FEP). Benefit determinations for FEP members are made by the Federal Employee Program and may supersede the coverage guidance in this document. Individual member benefits and contract terms must be verified prior to authorization because exclusions, limitations, or exceptions may apply.
Use of Opsynvi (macitentan; tadalafil) is permitted only when the member has had an inadequate response, adverse event, intolerance, or contraindication to the combined use of the individual ingredients macitentan and tadalafil. The policy explicitly states that treatment failure cannot be due to noncompliance or unwillingness to take the two ingredients separately; documentation of the clinical reason for not using the separate agents is required.
Medications listed in this policy are considered not medically necessary for members who do not meet the specific diagnostic, laboratory, or treatment‑trial criteria described in the policy. Requests that lack required supporting documentation or do not satisfy the stated criteria may be denied as not medically necessary.
Members who are currently receiving Opsynvi as samples or through a manufacturer's patient assistance program must satisfy the policy's initial approval criteria to continue coverage. This requirement ensures equal treatment of members regardless of prior access to free or sample medication and requires submission of the same clinical documentation used for initial approvals.
Prescriptions that exceed the policy's stated standard quantity limits or doses not supported by the FDA‑recommended dosing may be reviewed or denied. Examples include oral tablet limits (standard limits such as 60 tablets per 30 days for some agents) and inhaled cartridge use where doses >80 mcg per treatment session would require multiple cartridges; higher quantities require supporting justification.
FDA communications and published guidance regarding pediatric use of sildenafil/Revatio are referenced in the policy. These sources include an FDA Drug Safety Communication clarifying pediatric warnings and peer‑reviewed literature that inform safety considerations for pediatric sildenafil use and related coverage decisions.
Wellmark determines medical necessity only when the requested service or medication is included in the member's benefit plan and no contract exclusions apply. Benefit determinations are based on the applicable contract language in effect at the time of service; therefore, coverage is contingent on the existence of the benefit and the member's specific contract terms.
Billing Codes, Hemodynamic Thresholds, and Dosing Limits
| Adcirca, Alyq, Tadliq (tadalafil) — oral PDE5 inhibitors indicated for PAH |
| Adempas (riociguat) — sGC stimulator for PAH and CTEPH |
| Flolan/Veletri (epoprostenol) — intravenous prostanoid |
| Letairis (ambrisentan) — endothelin receptor antagonist |
| Opsumit (macitentan) — endothelin receptor antagonist | |
| Opsynvi (macitentan; tadalafil) — macitentan + tadalafil combination for PAH |
| Orenitram (treprostinil) — oral prostanoid | |
| Remodulin (treprostinil) — IV/SC prostanoid | |
| Tyvaso (treprostinil) — inhaled prostanoid | |
| Yutrepia (treprostinil) — inhaled prostanoid | |
| Uptravi (selexipag) — oral prostacyclin receptor agonist | |
| Ventavis (iloprost) — inhaled prostanoid | |
| Winrevair (sotatercept) — subcutaneous activin signaling inhibitor |
| Revatio/Liqrev (sildenafil) — oral/IV PDE5 inhibitor | |
| Tracleer (bosentan) — endothelin receptor antagonist |
Prior Authorization, Documentation, and Provider Responsibilities
Prior authorization contingent on meeting approval criteria
Coverage of the listed PAH and related pulmonary hypertension therapies is contingent on meeting the policy's approval criteria and the member having no exclusions to the prescribed therapy.
Prior authorization required; typical 12-month approvals
Prior authorization is required for the therapies described; when criteria are met, typical authorizations are granted for 12 months (drug- and indication-specific durations vary).
Authorization durations (12 months typical; Winrevair 6 months)
Authorization durations vary by product and indication: most PAH/PH therapies may be authorized for 12 months; Winrevair (sotatercept) is authorized for 6 months when criteria are met; select prostacyclin/epoprostenol therapies may receive 36-month authorization when benefit is demonstrated.
Prior authorization — not otherwise specified here
No additional or alternative prior authorization requirement is specified in this excerpt beyond the policy's documented prior authorization framework.
Consider risks/benefits when transitioning therapies
When transitioning prostacyclin therapies (for example, from Flolan/epoprostenol to Remodulin/treprostinil), the risks and benefits should be carefully considered prior to transition.
Step requirement before Opsynvi: try macitentan + tadalafil
For Opsynvi, the member must have an inadequate response, adverse event, intolerance, or contraindication to the combined use of macitentan and tadalafil (failure must not be due to noncompliance or unwillingness to take the ingredients separately).
Winrevair authorized only as add-on to at least two PAH drug classes
Winrevair (sotatercept) is covered only as add-on therapy; the member must be receiving PAH therapy from at least two specified drug classes prior to approval.
- Required concomitant classes: endothelin receptor antagonist, PDE-5 inhibitor, soluble guanylate cyclase stimulator, prostacyclin analog, or prostacyclin receptor agonist.
Calcium channel blocker responsiveness noted for select WHO Group 1 patients
A subset of WHO Group 1 patients (for example, heritable PAH or drug/toxin-associated PAH) may be long-term responders to calcium channel blockers; this clinical note may affect consideration of step or trial therapies.
Step therapy — no additional requirements specified
No comprehensive step therapy protocol beyond the product-specific requirements (e.g., Opsynvi, Winrevair) is detailed in these references.
Verify member benefits and provider responsibility
Providers are responsible for medical advice and treatment decisions and must verify individual member benefits, limitations, and contract applicability prior to requesting authorization.
Submit chart notes or medical records for initial Winrevair requests
For initial prior authorization of Winrevair, submit chart notes, medical record documentation, or claims history supporting current PAH therapy to initiate review.
Prescriber specialty requirement: pulmonologist or cardiologist
For pulmonary hypertension indications, the requested medication must be prescribed by or in consultation with a pulmonologist or cardiologist.
Required clinical documentation: WHO group and confirmatory hemodynamics
Documentation must support the WHO grouping diagnosis and confirmatory testing—typically pretreatment right heart catheterization results (mPAP > 20 mmHg; PCWP ≤ 15 mmHg; PVR > 2 Wood units) or, for infants <1 year when catheterization is not feasible, Doppler echocardiogram.
Billing guidance for sotatercept (Winrevair): J3590/C9399 and dosing documentation
When billing Winrevair (sotatercept) as an unclassified biologic/drug, identify the product and use J3590 or C9399 per guidance; include documentation of weight-based starting and target mg/kg dosing and the dosing interval.
References available to support clinical documentation
The policy provides bibliographic references (package inserts and peer‑reviewed literature) that may be used to support documentation of indications, dosing, and safety for PAH therapies.
Benefits vary by contract; medical necessity determined only if benefit exists
Benefits and coverage may vary by contract; Wellmark will determine medical necessity only when the benefit exists and no contract exclusions apply.
Missing documentation may cause denial
Failure to provide required supporting documentation (chart notes, medical records, or claims history) may result in denial of the prior authorization request.
Not medically necessary if criteria are not met
Use of the listed medications is considered not medically necessary when the stated diagnostic, laboratory, or treatment-trial criteria in the policy are not satisfied.
Quantity limits — overages may trigger denial or review
Prescriptions exceeding the policy's standard quantity limits (for example, >60 tablets per 30 days for many oral agents or cartridge/kit limits for inhaled products and 1 kit per 21 days for Winrevair vial kits) may be subject to review or denial.
- Example limits: Tracleer 60 tablets/30 days; Uptravi 60 tablets/30 days; Tyvaso DPI maintenance kits 112 cartridges/28 days; Winrevair 1 kit per 21 days.
Background and Clinical Context
Pulmonary arterial hypertension (PAH, WHO Group 1) is a progressive disorder managed with targeted therapies from several drug classes, including prostanoids (intravenous, subcutaneous, oral, and inhaled), endothelin receptor antagonists (ERAs), phosphodiesterase‑5 (PDE5) inhibitors, soluble guanylate cyclase stimulators, and newer agents such as activin signaling inhibitors. Drug treatment aims to improve exercise capacity, delay clinical worsening, and reduce morbidity and hospitalization; clinical trials supporting these therapies predominantly enrolled patients with WHO/NYHA functional class II–III (and some III–IV) and etiologies including idiopathic, heritable, connective tissue disease‑associated, and congenital heart disease‑associated PAH.
Definitions and WHO Group Classifications
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