Prior authorization required for listed PAH therapies

Prior authorization is required for the listed PAH medications (examples include Alyq/tadalafil, bosentan, Flolan/Veletri (epoprostenol), macitentan, Orenitram (treprostinil), sildenafil (generic Revatio), Tadliq, treprostinil formulations, Uptravi (selexipag)). Requests will be approved only when the medical necessity criteria in the policy are met.

• Applies to oral, inhaled, subcutaneous and intravenous formulations listed in the policy.

PAH diagnosis and combination-therapy required in prior auth

Prior authorization requests must document that the patient has pulmonary arterial hypertension (PAH, WHO Group 1) and, when combination therapy is requested, must show that the patient failed to demonstrate an adequate response to a single medication and that the medications in the combination are from different therapeutic classes.

• Combination-therapy requests require evidence of prior inadequate response to monotherapy and that agents are from different classes.

Prior authorization implied by coverage criteria

Coverage criteria in the policy imply prior authorization is required for the listed agents; providers must submit requests showing the patient meets the policy's medical necessity conditions (diagnosis of PAH, WHO Group 1, and combination-therapy conditions when applicable).

• Policy lists specific agents and states coverage applies only when all criteria are met.

Prior authorization requires clinical justification

Prior authorization requests for advanced PAH therapies must include clinical justification documenting the PAH (WHO Group 1) diagnosis and, if combination therapy is requested, documentation of prior inadequate response to single-agent therapy and that agents are from different therapeutic classes.

• Requests without this documentation risk denial.

Prior authorization contingent on documented PAH and combo conditions

Prior authorization will be approved only when the individual has a documented diagnosis of PAH (WHO Group 1) and, for combination therapy, documentation that the patient failed to demonstrate an adequate response to a single medication and that combination agents are from different therapeutic classes.

• Approval is contingent on meeting all policy criteria for the requested agent or regimen.

Prior authorization for PAH advanced therapies — include diagnosis and prior trials

For PAH advanced therapies, prior authorization must include documentation of the PAH (WHO Group 1) diagnosis and, when applicable, evidence that prior single-agent therapy failed before starting combination therapy.

• Drug-specific criteria (e.g., trials of generic equivalents or prior agents) must also be supplied when required by the product section.

Prior authorization requirements — document diagnosis, prior inadequate response, and prior trials when specified

Prior authorization requests must document the PAH (WHO Group 1) diagnosis and, for combination therapy, show prior inadequate response to a single medication and that agents are from different therapeutic classes; branded product requests may also require prior trial/intolerance to generic equivalents per drug-specific rules.

• Examples: Letairis/Remodulin/Revatio/Tracleer sections list required prior trials of generics or prior inadequate response.

Document failed adequate response to single medication for combo therapy

For combination-therapy prior authorization, provide documentation that the patient failed to demonstrate an adequate response to a single medication before escalation to combination regimens; the policy requires this step prior to approving combination use.

• Policy repeats that combination therapy is permitted only after single-agent failure and that agents must be from different therapeutic classes.

Required documentation: diagnosis and prior inadequate response for combination therapy

Reiterate required supporting documentation: the PAH (WHO Group 1) diagnosis and, when combination therapy is sought, evidence of prior inadequate response to monotherapy and that combination agents are from different classes.

• Include medication history showing trials, dates, and reasons for discontinuation or inadequate response.

PAH diagnosis and prior inadequate response required for listed agents

When requesting prior authorization for listed PAH agents, demonstrate the diagnosis of PAH (WHO Group 1) and, if combination therapy is requested, that the patient failed to demonstrate adequate response to a single medication and that agents are from different therapeutic classes.

• Provider should reference the drug-specific coverage language where applicable.

Prior authorization contingent on documented criteria

Prior authorization approval is contingent on documented diagnosis of PAH (WHO Group 1) and, where applicable, evidence of inadequate response to single-agent therapy plus use of agents from different therapeutic classes.

• Ensure records clearly connect the clinical rationale to the policy criteria.

Prior authorization length and reauthorization documentation

Initial authorizations and reauthorizations for drugs listed in this policy require prior authorization; approvals may be issued for up to 12 months, and reauthorization requires documentation of continued positive clinical response.

• Chart notes should demonstrate ongoing benefit for reauthorization.

Use listed HCPCS/J-codes and bill to correct benefit

When submitting claims for advanced PAH therapies, use the listed HCPCS/J-codes (e.g., J1325, J3285, J3490, J3590, J7686, K0455, Q4074) and bill under the correct benefit (medical vs pharmacy) indicated for each drug.

• Billing under the incorrect benefit may cause processing issues; check the Benefit Application table to confirm medical vs pharmacy management.

Follow benefit-specific management (medical vs pharmacy) and prior auth processes

Many PAH therapies are managed under either the medical or pharmacy benefit (drug-specific); providers must follow the applicable benefit management and prior authorization processes for each drug as indicated in the policy.

• Examples: Flolan, Remodulin, Uptravi, and Veletri are medical-benefit managed; numerous others (Adempas, Alyq, macitentan, Tyvaso, Yutrepia, etc.) are pharmacy-managed; Winrevair is dual-managed.

Prior auth for initiation of oral treprostinil — include trial eligibility and background therapy

Prior authorization for initiation of oral treprostinil and other advanced PAH therapies should reference trial eligibility criteria (e.g., baseline 6MWD when applicable) and background PAH therapy status as specified in the product sections.

• FREEDOM‑M trial eligibility (example) required minimum baseline 6MWD of 100 meters — referenced for context in trial-based coverage language.

Step therapy: combination allowed only after single-agent failure

Combination therapy is allowed only after the patient has failed to demonstrate an adequate response to a single medication; medications used concurrently must be from different therapeutic classes.

• The policy repeatedly states both subconditions (failed single-agent response and different therapeutic classes) are required for combination coverage.

Document prior single-agent failure before combination regimens

When used as combination therapy, the policy requires documented failure (inadequate response) to a single medication before adding another class of medication; provide prior treatment dates and response details in the request.

• Requests without clear documentation of prior single-agent failure and class distinction risk denial.

Step therapy requirement — document monotherapy failure

Consistent policy language: combination regimens require prior inadequate response to a single medication before adding additional agents — the provider must document the monotherapy trial and outcome.

• Specify reasons for considering combination therapy and how prior monotherapy was insufficient.

Step therapy: record failure and class distinction for combo approval

Before approving combination therapy, providers must record that the patient failed to demonstrate an adequate response to a single medication and that proposed combination agents are from different therapeutic classes.

• Include objective or clinical evidence of inadequate response (symptoms, functional class, test results) when available.

Branded products often require prior trial of generic equivalent

Many branded products require a prior trial and inadequate response or intolerance to the generic equivalent before branded product coverage (examples: Letairis, Revatio/Revatio generics, Remodulin/generic treprostinil).

• Follow the drug-specific sections which list required prior trials or intolerance to generic equivalents.

Therapy selection guidance — specialty drug management

Advanced pharmacologic therapies are specialty drugs intended to impact PAH/CTEPH natural history and may be used as single agents or in combination per guidelines and approval status; selection should follow product-specific and policy requirements.

• Therapy choice should align with policy coverage criteria and any drug-specific step or trial requirements.

Do not combine riociguat with PDE5 inhibitors (contraindicated)

Do not combine riociguat (Adempas) with a phosphodiesterase‑5 inhibitor (sildenafil, tadalafil, or vardenafil); the policy explicitly states this combination is contraindicated and should not be requested.

• Requests that include riociguat plus a PDE5 inhibitor will conflict with the policy contraindication and risk denial.

Required documentation to support diagnosis and rationale

Provider documentation must support the diagnosis of PAH (WHO Group 1) and the clinical rationale for therapy; the Introduction and Documentation Requirements sections direct providers to include supporting clinical records.

• Include office visit notes with diagnosis, relevant history, physical exam, and medication history.

Required documentation: PAH diagnosis and prior inadequate response for combos

Documentation must show the diagnosis of pulmonary arterial hypertension (PAH, WHO Group 1) and, for combination therapy, evidence of prior inadequate response to a single medication and that agents are from different therapeutic classes.

• Provide medication trial dates, dosing, objective response measures, and rationale for switching or adding agents.

Clinical documentation must show diagnosis and monotherapy failure when applicable

Clinical documentation supporting authorization requests must demonstrate the PAH (WHO Group 1) diagnosis and, when combination therapy is requested, that single-agent therapy failed and agents used in combination are from different therapeutic classes.

• Include prior treatment history, intolerance or inadequate response notes, and class of prior agent.

Documentation required to demonstrate coverage criteria

To demonstrate policy criteria, provide documentation of PAH (WHO Group 1) diagnosis and evidence of prior inadequate response to single-agent therapy for combination requests; the medical record should include office notes and medication history.

• Objective data (hemodynamics, functional class, 6MWD) should be included when available.

Medical record documentation: office notes and med history required

Medical records submitted must document that medical necessity criteria are met and include office visit notes with diagnosis, relevant history, physical evaluation, and medication history.

• Ensure records explicitly reference PAH WHO Group 1 and prior therapy trials with outcomes.

Coding and benefit documentation — use listed codes and bill to correct benefit

Use the HCPCS/J-codes and HCPCS DME code listed in the policy when submitting claims; verify whether the therapy is managed under the medical or pharmacy benefit and bill accordingly.

• Codes include J1325, J3285, J3490, J3590, J7686, K0455, Q4074 as referenced in the Coding section.

Infusion pump and back‑up pump documentation for infused prostanoids

For patients receiving continuous IV or SC prostanoids (e.g., epoprostenol, treprostinil), document infusion pump use and back‑up pump plan; cost of a back‑up pump may be included in home infusion charges or the HCPCS code.

• Include documentation of pump type, back‑up arrangements, and where costs are billed.

Required procedural documentation for IV prostanoids

When requesting coverage for IV prostanoids, include procedural documentation such as evidence of initial dose‑ranging (if performed), central venous catheter insertion, and a plan for a portable infusion pump with backup and maintenance.

• Document central line placement, inpatient dose‑ranging notes, and pump management plans to address rebound risk and complications.

Denial triggers — missing PAH diagnosis or combo-therapy documentation

Requests lacking a documented diagnosis of PAH (WHO Group 1) or lacking evidence that combination therapy meets the policy conditions (failed response to single medication and agents from different therapeutic classes) may be denied.

• Combination therapy without documented monotherapy failure or using agents from the same class is specifically cited as a denial trigger.

Denial risk for unmet diagnostic or combination-therapy requirements

Coverage may be denied if the individual does not have a documented diagnosis of PAH (WHO Group 1) or does not meet the combination‑therapy prerequisites (failed adequate response to a single medication and use of agents from different therapeutic classes).

• Requests for listed agents that do not meet those documented criteria risk denial.

Do not request PAH therapies for non‑PAH PH subtypes (investigational)

Requests for listed PAH therapies for pulmonary hypertension subtypes outside PAH (WHO Groups 2–5) are considered investigational and may be denied; do not submit requests for non‑PAH indications under these PAH-specific criteria.

• Policy explicitly excludes treatment of non‑PAH PH subtypes with these agents as investigational.

Benefit assignment mismatch may cause processing issues

Failure to bill under the correct benefit (medical vs pharmacy) for listed therapies may lead to claim processing issues or denials; confirm benefit assignment in the policy's Benefit Application table before submitting claims.

• Examples: Flolan, Remodulin, Uptravi, and Veletri are medical‑managed; many others are pharmacy‑managed; Winrevair is dual-managed.

Denial risk: insufficient administration documentation for prostanoids

Lack of appropriate administration documentation for prostanoid therapies (e.g., no inpatient initial dose‑ranging for epoprostenol, missing central venous catheter placement details, or absent infusion pump/back‑up plan) may lead to coverage denial for infused prostanoid therapy.

• Include inpatient dose‑ranging notes (if performed), central line insertion records, pump/back‑up plans, and ongoing maintenance/complication management documentation.