Pulmonary Arterial Hypertension (PAH) Drug Therapy — Coverage Criteria
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Covers selection and coverage criteria for FDA‑approved and compendial PAH drug therapies for Wellmark members, governing medical necessity determinations and benefit applicability.
No material clinical or coverage changes in this revision.
Coverage Criteria for PAH/PH Drug Therapies
General coverage for PAH drug indications
Covered when ALL of the following are met
From policy description
Drug-specific covered indications (informational)
Drugs listed with their FDA‑approved PAH indications and study populations
See chunks 4 and 12
See chunk 5
See chunks 7, 8, 9, 13
See chunks 6, 10, 11, 14, 15, 17
See chunk 16
See chunk 18
Winrevair (sotatercept) — Pulmonary Arterial Hypertension (PAH)
Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:
Authorization duration: 12 months
Winrevair (sotatercept) — Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Authorization of 12 months may be granted for treatment of CTEPH when ALL of the following criteria are met:
Authorization duration: 12 months
Tadalafil products (Adcirca, Alyq, Tadliq) — Pulmonary Arterial Hypertension (PAH)
Authorization of 12 months may be granted for treatment of PAH when ALL of the following criteria are met:
Authorization duration: 12 months
Tadalafil/Sildenafil — Secondary Raynaud's Phenomenon
Authorization of 12 months may be granted for treatment of secondary Raynaud's phenomenon when the following are met:
Authorization duration: 12 months
Other PAH therapies (epoprostenol, iloprost, ambrisentan, macitentan, treprostinil, bosentan, treprostinil inhaled) — PAH/PH
Authorization of 12 months may be granted for treatment when ALL of the following criteria are met:
Authorization duration: 12 months
Opsynvi (macitentan; tadalafil) — Combination product
Authorization of 12 months may be granted for treatment of PAH when ALL of the following are met:
Authorization duration: 12 months
Tracleer (bosentan) — Initial Authorization
Authorization of 12 months may be granted when ALL of the following are met:
Authorization duration = 12 months
Tyvaso, Tyvaso DPI, Yutrepia (treprostinil) — Initial Authorization
Authorization of 12 months may be granted when BOTH of the following are met:
Authorization duration = 12 months
Uptravi (selexipag) — Initial Authorization
Authorization of 12 months may be granted when ALL of the following are met:
Authorization duration = 12 months
Winrevair (sotatercept) — Initial Authorization
Authorization of 6 months may be granted for members ≥18 years when ALL of the following are met:
Authorization duration = 6 months; age ≥18 required
Continuation / Long-term Authorization and Indications
Authorizations may be extended when members are receiving therapy and are experiencing benefit as evidenced by disease stability or improvement:
See chunk for drug-specific lists and durations
Drug quantity limits and dosing adherence
Covered when drug dispensing is within the listed standard quantity limits and consistent with FDA‑recommended dosing
This section provides quantity limits and FDA dosing; full medical necessity criteria may be located in other policy parts.
This policy may not apply to Federal Employee Program (FEP) members. Benefit determinations for FEP members are governed by the Federal Employee Program and are made according to the applicable FEP contract language; verify benefits under the member's FEP plan prior to prior authorization review.
For the combination product Opsynvi (macitentan; tadalafil), a member must have experienced an inadequate response, adverse event, intolerance, or a contraindication to the combined use of the individual ingredients given separately. Treatment failure cannot be due to noncompliance or unwillingness to take the two ingredients separately; such reasons do not satisfy the requirement for prior treatment failure when considering authorization.
Drugs addressed in this policy are considered not medically necessary for patients who do not meet the specific criteria set forth in the applicable sections above. Requests that lack required diagnostic confirmation, documentation of prior therapy where applicable, or other stated criteria may be denied as not medically necessary.
Members who are currently receiving Opsynvi as samples or through the manufacturer's patient assistance program must meet the policy's initial approval criteria to qualify for coverage. Enrollment in a manufacturer assistance or receipt of samples does not waive the requirement to satisfy initial authorization criteria.
No additional explicit exclusions are listed in the provided excerpt of this policy beyond those noted elsewhere (for example, FEP applicability). Where specific exclusions apply they will be determined by contract language and the member's benefits.
Wellmark will determine medical necessity only if an applicable benefit exists and no contract exclusions apply. Individual member benefits and contract exclusions must be verified prior to coverage determination; services lacking an applicable benefit may be denied regardless of clinical criteria.
For certain agents (for example, Winrevair/sotatercept), absence of documentation supporting the member's current PAH therapy may lead to denial. The policy requires submission of chart notes, medical record documentation, or claims history to initiate prior authorization review for initial requests and to confirm that required background PAH therapies are being used where specified.
Use of the PAH/PH drugs listed in this policy for indications not supported by the stated diagnostic or hemodynamic criteria, or for requests that do not meet the required pretreatment confirmation (e.g., right heart catheterization demonstrating mPAP > 20 mmHg, PCWP ≤ 15 mmHg, and PVR > 2 Wood units), is considered not medically necessary and may be denied.
Requests that exceed the listed standard quantity limits for a given product or strength may be subject to limits or denial. Examples include the standard limit for Tracleer (bosentan) 125 mg = 60 tablets per 30 days, and various per‑28/30‑day cartridge or vial limits for inhaled and injectable formulations; quantities above the stated standard limit will trigger coverage review.
Procedure and Billing Codes / Hemodynamic Thresholds
Provider Actions — Prior Authorization, Documentation, and Prescribing
Policy is clinical in nature; Prior Authorization Required
PAH drug therapies are clinical in nature and considered covered benefits only when all approval criteria are met and no member contract exclusions apply. Providers should verify member benefits and contract language prior to submission. Prior authorization is required for specialty pulmonary hypertension agents listed in this policy; approvals are contingent on meeting the specific criteria below.
- Prior authorization required for listed PAH agents
- Coverage contingent on meeting approval criteria and absence of exclusions
- Verify member benefits and contract language
Hemodynamic confirmation and diagnosis required
Authorization generally requires a documented diagnosis and hemodynamic confirmation. For PAH (WHO Group 1) and many other indications in this policy, right heart catheterization pretreatment results must demonstrate: mean pulmonary arterial pressure (mPAP) > 20 mmHg, pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units (PVRI thresholds noted for pediatric patients where specified). For infants <1 year, Doppler echocardiogram is acceptable when catheterization cannot be performed.
- Right heart catheterization data (mPAP, PCWP, PVR) required unless infant <1 year
- Pediatric PVRI thresholds where specified
- Doppler echocardiogram acceptable in infants when cath not feasible
Prior authorization duration and timing
Prior authorization may be authorized for up to 12 months for many PAH therapies when the listed diagnostic and clinical criteria are met. Specific drugs (for example Winrevair) may have shorter authorization durations (e.g., Winrevair authorization of 6 months) and additional entry requirements.
- Typical authorization = 12 months when criteria met
- Winrevair authorization = 6 months for adults ≥18 years with additional criteria
Prior authorization not specified in this portion of the policy
Some parts of this section list clinical information, references, or package insert citations but do not themselves impose prior authorization requirements. Providers should refer to the specific drug criterion blocks for authorization requirements and to other policy sections as needed.
- References and package inserts may inform clinical decisions but are not a substitute for criteria
- See specific drug sections for authorization/denial rules
Drugs considered not medically necessary when criteria unmet
Drugs referenced in this section are considered not medically necessary if the member does not meet the criteria set forth in the applicable drug-specific authorization criteria. Lack of required documentation (diagnostic testing, chart notes, or evidence of prior therapy where required) may lead to denial.
- Drugs not medically necessary when criteria unmet
- Denial risk if required documentation or diagnostic criteria are absent
Quantity limit exceedance — check standard limits and dosing
Quantity limits apply to many products. Prescriptions that exceed standard quantity limits or FDA-recommended dosing may trigger coverage limits or denials. Examples include Tracleer tablet limits and Tyvaso/DPI cartridge limits. Providers should ensure dose and quantity requests align with the policy’s quantity limits and FDA-recommended dosing.
- Confirm standard limit and dosing before submitting prior authorization
- Exceeding listed quantity limits (e.g., Tracleer, Tyvaso, Winrevair kit limits) may be denied or require additional justification
Package inserts and clinical literature may support documentation
This section includes references, package inserts, and clinical literature that may be used to support documentation for prior authorization requests. Providers may submit relevant package insert information, published guidelines, or compendia citations as supporting evidence.
- Package inserts and clinical literature listed in References may support requests
- Compendia (AHFS, Micromedex) and guideline citations acceptable
Required documentation and Non‑Formulary exception documentation
Required documentation for specific products must be submitted with the prior authorization request. For Winrevair (sotatercept) initial requests, submit chart notes, medical record documentation, or claims history supporting current PAH therapy. For non-formulary exception requests, include chart notes documenting prior treatment failure, severity of adverse events, dosage and duration of prior therapies, or contraindications to formulary alternatives.
- Winrevair initial requests: chart notes, medical records, or claims history supporting current PAH therapy
- Non-formulary exception: documentation of prior treatment failure, severity, dosage, duration, or contraindication to alternatives
Weight-based dosing documentation required for injectable agents
For weight-based and injectable biologics (e.g., Winrevair), include documentation of weight and dosing calculations. Winrevair dosing is weight-based: starting dose 0.3 mg/kg subcutaneously every 3 weeks with a target dose of 0.7 mg/kg every 3 weeks; standard kit limits are noted in the policy.
- Provide patient weight and dose calculation for weight-based agents
- Winrevair dosing: start 0.3 mg/kg q3w; target 0.7 mg/kg q3w; standard kit limits apply
Clinical decision and documentation when transitioning between prostanoids
Clinical decisions about transitioning between prostanoid products (for example between Remodulin and Flolan) should weigh risks and benefits. The policy notes that transitions are indicated in some situations (e.g., to diminish clinical deterioration) and that such transitions should be clinically justified in the medical record.
- Document clinical rationale and risks/benefits when transitioning prostanoids
- Transitions should be supported by chart notes and clinical justification
Combination product and step requirements (Opsynvi, secondary Raynaud's)
Certain product-specific step requirements apply. For combination products like Opsynvi (macitentan/tadalafil), the member must have inadequate response, adverse event, intolerance, or contraindication to the combined use of the individual components (macitentan and tadalafil) before approving the combination product. For tadalafil/sildenafil when used for secondary Raynaud's phenomenon, authorization requires prior inadequate response to at least one alternate therapy listed in the policy.
- Opsynvi requires failure/intolerance/contraindication to using macitentan and tadalafil separately
- Secondary Raynaud's: prior inadequate response to specified alternate therapies required
Step therapy not specified across the section
No general step therapy sequencing is specified across the entire section beyond the drug-specific requirements above. Clinical guideline citations are provided to inform therapy sequencing, but the policy does not impose additional universal step-therapy rules beyond those described in individual drug sections.
- No overarching step therapy beyond drug-specific requirements
- Use guideline references for clinical sequencing when applicable
Background and Scope
Pulmonary arterial hypertension (PAH, WHO Group 1) therapies are prescribed with the goals of improving exercise capacity, delaying clinical worsening, and reducing hospitalization risk. Targeted drug classes include phosphodiesterase‑5 inhibitors, endothelin receptor antagonists, prostanoids, soluble guanylate cyclase stimulators, prostacyclin receptor agonists, and newer agents such as activin signaling inhibitors; clinical trials primarily enrolled patients with WHO/NYHA functional class II–III (and some III–IV) across idiopathic, heritable, connective tissue disease–associated, and other PAH etiologies.
Definitions and WHO PH Group Classifications
Policy History and References
This section of the document does not itself specify prior authorization mechanics; refer to other parts of the policy for detailed prior authorization requirements and process. The policy includes creation and revision metadata (policy history and effective dates) that are relevant to administration of prior authorization rules.
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