CurrentAvMedPolicy N/A

Pulmonary Arterial Hypertension (PAH) Drugs — Pharmacy Prior Authorization and Step‑Edit

Form and clinical criteria governing prior authorization for pharmacy-covered PAH medications for AvMed members; applies to prescribers requesting PAH drugs listed on the form.

Policy Summary

PayerAvMed

PolicyPulmonary Arterial Hypertension (PAH) Drugs — Pharmacy Prior Authorization and Step‑Edit

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

Key ActionSubmit a completed, signed prior authorization form with diagnosis, NYHA functional class, right heart catheterization results, and supporting chart notes and labs.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

MultipleDrug classes listed

6 moInit. auth length

12 moContinuation auth length

mPAP≥20/PAWP≤15/PVR≥2Required hemodynamics

NYHA II–IIIFunctional class required

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Coverage Criteria for PAH Medications

Initial and continuing therapy criteria

Covered when ALL of the following are met (section A diagnosis criteria plus agent-specific and therapy-status criteria):

Overall requirements: Provider is a clinician with expertise in treating PAH; member has a diagnosis of pulmonary arterial hypertension (PAH) WHO Group 1 (or WHO Group 4 when applicable) confirmed by right heart catheterization with submitted RHC results, chart notes, labs and imaging; pregnancy excluded for females of reproductive potential and acceptable contraception used during treatment and for 1 month after discontinuation; medication-age requirements met for the selected agent.

See agent-specific nodes for additional constraints.

Medication-age requirements: Member must meet ONE: at least 18 years old (general); for bosentan requests: at least 3 years old; for sildenafil requests: at least 1 year old.

Hemodynamic confirmation: Diagnosis of PAH confirmed by right heart catheterization with mPAP ≥ 20 mmHg, PAWP ≤ 15 mmHg, and PVR ≥ 2 Wood units; submit RHC results and supporting documentation (labs, imaging, PFTs, ABGs).mPAP ≥ 20 mmHg; PAWP ≤ 15 mmHg; PVR ≥ 2 WU

Initiation vs continuation: For initiating therapy: a PAH risk assessment has been completed with documented low, intermediate, or high risk (initiation approval length 6 months). For continuing therapy: member is stable/maintained low-risk on current therapy OR follow-up shows increased risk requiring escalation (continuation approval length 12 months). IV PAH therapy requires prior authorization.

Combination therapy limited to two drugs from different therapeutic classes.

Documentation requirements: All documentation to support checked criteria must be provided: prescriber signature, diagnosis and ICD code if applicable, chart notes verifying NYHA functional class, prior therapy failures when required, baseline and monitoring labs (LFTs, hemoglobin), REMS enrollment when applicable, and diagnostics for CTEPH (V/Q or pulmonary angiography) when relevant.

Authorization may be delayed or denied if documentation is incomplete.

Sildenafil (Revatio) coverage

Sildenafil (Revatio/generic) coverage when ALL of the following are met

Sildenafil requirements: Member's symptomology is NYHA Functional Class II or III; sildenafil will NOT be used concurrently with Adempas (riociguat); member is NOT receiving organic nitrates (regularly or intermittently); if oral suspension is requested for members >18 years, medical documentation must show a clinical/medical preclusion to taking oral tablets.

Concurrent therapy and oral formulation requirements must be verified by chart notes or pharmacy paid claims.

Tadalafil (Adcirca/Alyq/Tadliq) coverage

Tadalafil (Adcirca/Alyq/Tadliq) coverage when ALL of the following are met

Tadalafil requirements: Member's symptomology is NYHA Functional Class II or III; tadalafil will NOT be used concurrently with Adempas (riociguat); member is NOT receiving organic nitrates (regularly or intermittently); if Tadliq oral suspension is requested for members >18 years, medical documentation must show a clinical/medical preclusion to taking oral tablets.

Concurrent therapy and oral formulation requirements must be verified by chart notes or pharmacy paid claims.

Riociguat (Adempas) coverage

Riociguat (Adempas) coverage when ALL of the following are met

Adempas requirements: Adempas will NOT be taken in combination with a PDE‑5 inhibitor (e.g., sildenafil or tadalafil); all provider and patient‑specific REMS requirements have been satisfied.

For CTEPH (WHO Group 4) additional CTEPH‑specific criteria apply (see CTEPH criteria).

CTEPH (WHO Group 4) criteria

CTEPH (WHO Group 4) specific requirements when ALL of the following are met

CTEPH requirements: Diagnosis of persistent/recurrent CTEPH confirmed; member previously failed surgical treatment (such as pulmonary endarterectomy) or is inoperable; documentation of ventilation‑perfusion scan or pulmonary angiography confirming CTEPH attached with the request.

Applies to riociguat and related approvals for WHO Group 4 patients.

Endothelin receptor antagonists coverage

Endothelin receptor antagonists (ambrisentan, bosentan, macitentan) coverage when ALL of the following are met

Ambrisentan requirements: Member's symptomology is NYHA Functional Class II or III; all REMS requirements satisfied; diagnosis of idiopathic pulmonary fibrosis (WHO Group 3) is a contraindication to ambrisentan and excluded from therapy.

Bosentan requirements: For adults: NYHA Functional Class II, III, or IV; for pediatric members ≥ 3 years: PAH idiopathic or congenital etiologies; dispersible tablet limited to age 3–11 with weight 4–40 kg; baseline liver function test obtained prior to initiation and will be monitored; REMS requirements satisfied.

LFT monitoring required.

Macitentan (Opsumit) requirements: NYHA Functional Class II or III; provider has submitted medical chart notes and history documenting failure of ambrisentan or bosentan (detailed records such as decreases in 6MWD, clinical worsening, decreased exercise ability) unless member is already established on single agents; baseline hemoglobin and LFT obtained prior to initiation and will be monitored monthly; REMS requirements satisfied.

Failure documentation required for initiation of macitentan (or combination product).

Selexipag (Uptravi) coverage

Selexipag (Uptravi) coverage when ALL of the following are met

Selexipag requirements: Member's symptomology is NYHA Functional Class II or III; Uptravi is being selected as add‑on therapy due to clinical worsening and increased risk on current therapy; Uptravi will be used in combination with an endothelin receptor antagonist and/or a PDE‑5 inhibitor; Uptravi will NOT be taken in combination with a prostanoid/prostacyclin analogue.

Add‑on and combination requirements must be verified by chart notes or pharmacy paid claims.

Orenitram coverage

Orenitram (oral treprostinil) coverage when ALL of the following are met

Orenitram requirements: Member's symptomology is NYHA Functional Class II or III; a baseline liver function test has been obtained and will be monitored; Orenitram is selected as add‑on therapy due to clinical worsening and increased risk on current therapy; if transitioning from an IV prostacyclin analogue, IV therapy will be tapered while transitioning to oral treprostinil.

Monitoring and transition plan required.

Inhaled/DPI prostacyclin coverage

Inhaled or DPI prostacyclins (Tyvaso, Tyvaso DPI, Ventavis, Yutrepia) coverage when ALL of the following are met

Inhaled prostacyclin requirements: Will NOT be used as monotherapy or for treatment‑naïve PAH patients; selected as add‑on therapy due to clinical worsening and increased risk on current therapy; generally required for NYHA Functional Class III (for Ventavis, Class III or IV applies).

Add‑on use only; functional class restrictions apply.

Opsynvi (macitentan/tadalafil) coverage

Combination macitentan/tadalafil (Opsynvi) coverage when ALL of the following are met

Opsynvi requirements: Member's symptomology is NYHA Functional Class II or III; Opsynvi will NOT be used concurrently with Adempas (riociguat); member must meet ONE: provider-submitted documentation of failure with ambrisentan or bosentan (detailed clinical documentation), OR member is established on dual therapy with single‑ingredient macitentan (Opsumit) and tadalafil; member is NOT receiving organic nitrates; baseline hemoglobin and liver function tests have been obtained and will be monitored monthly; all REMS requirements satisfied.

Combination product requires prior failure on other ERAs or established single‑agent dual therapy unless otherwise documented.

Prescriptions for phosphodiesterase type‑5 inhibitors (sildenafil or tadalafil) must not be used concurrently with a soluble guanylate cyclase stimulator (riociguat / Adempas). Requests must document verification (chart notes or pharmacy paid claims) that the member is not taking riociguat. Additionally, organic nitrates (e.g., isosorbide mononitrate, isosorbide dinitrate) must not be used either regularly or intermittently with PDE‑5 inhibitors due to potentiation of hypotensive effects; verification via chart notes or pharmacy claims is required.

Certain diagnoses and agent‑specific contraindications exclude members from coverage. A diagnosis of idiopathic pulmonary fibrosis (WHO Group 3) is a contraindication to ambrisentan and such members are excluded from ambrisentan therapy. For endothelin receptor antagonists (ambrisentan, bosentan, macitentan), REMS requirements and baseline/ongoing laboratory monitoring (for example, liver function tests or hemoglobin where specified) must be satisfied as detailed in the agent‑specific criteria.

Authorization requests lacking required supporting documentation risk denial or delay. To support any checked criteria, submit complete medical chart notes, right heart catheterization results (hemodynamics), baseline and monitoring labs, imaging or diagnostic studies (e.g., V/Q scan or pulmonary angiography when applicable), and any REMS enrollment documentation; incomplete, incorrect, or illegible submissions may result in denial.

Inhaled or dry‑powder prostacyclins (Tyvaso, Tyvaso DPI, Ventavis, Yutrepia) are not supported as monotherapy or for treatment‑naïve PAH patients. Coverage is limited to add‑on use for members experiencing clinical worsening and increased risk on current therapy, with most requests limited to NYHA Functional Class III (Ventavis may be used for Class III or IV).

Coding and Hemodynamic Criteria

Diagnosis codes (form field)ICD-10

ICD code

ICD Code, if applicable (field on form)

Hemodynamic thresholds — PAH diagnosis

Mean pulmonary arterial pressure (mPAP)mPAP ≥ 20 mmHg (measured at rest by right heart catheterization)

Pulmonary artery wedge pressure (PAWP)PAWP ≤ 15 mmHg (measured by right heart catheterization)

Pulmonary vascular resistance (PVR)PVR ≥ 2 Wood units (measured by right heart catheterization)

Initial Therapy Requirements and Approval Length

Initial therapy

Initiation requirements and approval length

Initiation prerequisites: A PAH risk assessment has been completed with documented risk status (low, intermediate, high); combination therapy limited to two drugs from different therapeutic classes; initiation approval length is 6 months; IV PAH therapy requires prior authorization.

Initial therapy requirements

Initial authorization requirements vary by agent and generally require NYHA functional class documentation, absence of contraindicated combinations, and drug‑specific monitoring and prior therapy failure evidence where applicable.

General initial requirements: Document NYHA functional class (typically II or III for many agents), verify no contraindicated concurrent medications (e.g., PDE‑5 inhibitors with riociguat; organic nitrates with PDE‑5 inhibitors), provide baseline labs when required (LFTs, hemoglobin), and satisfy REMS when applicable.

Refer to agent‑specific criteria for details.

Agent-specific initial constraints: Agent-specific criteria (age, formulation constraints, monitoring, prior failure documentation, and combination restrictions) must be met as detailed in individual product nodes (sildenafil, tadalafil, riociguat, ERAs, selexipag, orenitram, inhaled/DPI prostacyclins, Opsynvi).

Concurrent therapy conflicts will be verified by chart notes or pharmacy claims.

Continuation Therapy Requirements and Renewal

Continuation therapy

Continuation therapy approval length and conditions

Continuation conditions: Member's condition is stable (not clinically worsening) or has maintained low‑risk status on current therapy and the current regimen will continue; OR follow-up shows increased risk requiring escalation (combination/addition of agents).

Approval length 12 months; IV PAH therapy requires prior authorization.

Opsynvi continuation

Continuation on combination product

Opsynvi continuation: Member is established on dual therapy with single‑ingredient macitentan (Opsumit) AND tadalafil (Adcirca/Alyq), allowing transition to the fixed‑dose combination Opsynvi; documentation of current use of both single agents should be provided.

Step Therapy and Prior Failures

Limit	Details
Combination therapy limited to two drugs	Combination therapy is limited to a two‑drug regimen drawn from two different therapeutic classes listed in Section C. Applies to initiating and continuing PAH therapy; continuation approvals consider stability or need for escalation based on risk status.
Escalation exception	Escalation to add a second agent is permitted when follow‑up shows increased risk (change to intermediate or high risk) requiring escalation of therapy; IV PAH therapy requires prior authorization.
Continuation exceptions	Continuation approvals may maintain an existing two‑drug regimen if the member is stable (not clinically worsening) or has maintained low‑risk status; prior therapies will be verified via pharmacy claims or chart notes.

Requirement	Evidence needed / Notes
Prior failure of ambrisentan or bosentan required	Provider must submit medical chart notes and history detailing failure on ambrisentan or bosentan (documentation may include decreases in 6‑minute walk distance, clinical worsening, decreased exercise ability) prior to initiating macitentan (Opsumit).
Alternate: established dual therapy	For the macitentan/tadalafil combination (Opsynvi), member may alternatively be approved if established on single‑ingredient macitentan (Opsumit) plus tadalafil (Adcirca/Alyq); documentation of concurrent use should be provided.
Concurrent use exclusions	Opsynvi will NOT be used concurrently with riociguat (Adempas). Member must not be receiving organic nitrates due to potentiation of hypotensive effects. Baseline hemoglobin and LFTs required and must be monitored monthly; REMS requirements must be satisfied.

Quantity Limits by Product

Quantity limit — sildenafil 20 mg tablet

Productsildenafil 20 mg tablet

Quantity limit90 units per 30 days

Day supply / Units per dayDay supply: 30 days; Units/day: 3

Quantity limit — sildenafil 10 mg/mL oral suspension

Productsildenafil 10 mg/mL oral suspension

Quantity limit180 mL per 30 days

Day supply / Units per dayDay supply: 30 days; Units/day: 6 (mL/day)

Quantity limit — tadalafil 20 mg tablet

Producttadalafil 20 mg tablet

Quantity limit60 units per 30 days

Day supply / Units per dayDay supply: 30 days; Units/day: 2

Quantity limit — Adempas (riociguat)

ProductAdempas (riociguat) — all strengths

Quantity limit90 units per 30 days

Day supply / Units per dayDay supply: 30 days; Units/day: 3

Quantity limit — Uptravi (selexipag)

ProductUptravi (selexipag) — all strengths

Quantity limit60 units per 30 days

Day supply / Units per dayDay supply: 30 days; Units/day: 2

Quantity limit — Yutrepia

ProductYutrepia inhalation capsules

Quantity limit112 units per 28 days

Day supply / Units per dayDay supply: 28 days; Units/day: 4

Quantity limit — Ventavis ampules

ProductVentavis ampules (10 mcg/mL & 20 mcg/mL)

Quantity limit270 one‑mL ampules per 30 days

Day supply / Units per dayDay supply: 30 days; Units/day: 9 ampules

Provider Actions, Documentation, and Denial Risks

Prior Authorization

Prior Authorization Required

Prior authorization is required for all listed PAH pharmacy agents. Initiation approvals are issued for 6 months; continuation approvals are issued for 12 months. Incomplete or missing information may delay or result in denial of the request.

Approval lengths: Initiation = 6 months; Continuation = 12 months
Pharmacy prior authorization fax: 1-305-671-0200

Documentation Required

Prescriber Signature and Form Completion

Prescriber signature is required on the prior authorization request (preprinted stamps are not valid). The prescriber must clearly print their name. All other form fields must be complete and legible; missing or incorrect fields (including phone/fax) can delay processing.

Prescriber signature and printed name required (no preprinted stamps)
Ensure phone and fax numbers on form are accurate

Documentation Required

Required Drug and Patient Details

Provide complete drug information and clinical details: drug name, form/strength, dosing schedule, length of therapy, diagnosis and ICD code, weight (if applicable) and date weight obtained. Authorization may be delayed if this information is incomplete.

Drug name/form/strength, dosing schedule, length of therapy
Diagnosis and ICD-10 code; weight and date weight obtained (if applicable)

Documentation Required

Documentation to Attach

Attach supporting documentation with the request: chart notes confirming NYHA (WHO) functional class, right heart catheterization results and hemodynamics for PAH diagnosis, ventilation-perfusion scan or pulmonary angiography for CTEPH, baseline and monitoring labs (LFTs, hemoglobin) as specified, and any REMS documentation when applicable.

Right heart catheterization data: mPAP ≥ 20 mmHg, PAWP ≤ 15 mmHg, PVR ≥ 2 Woods units
Ventilation-perfusion scan or pulmonary angiography for CTEPH
Baseline LFTs and hemoglobin where required; monitoring schedule (monthly where specified)
REMS documentation when applicable

Step Therapy

Combination Therapy and Step Limits

Combination therapy is limited to a two-drug regimen from different therapeutic classes. Combination/add-on therapy for worsening disease requires documentation of clinical worsening or increased risk status. IV PAH therapy requires separate prior authorization.

Combination therapy limited to two agents from different classes (Section C)
Add-on agents (e.g., Uptravi, Orenitram, inhaled/iloprost/treprostinil products) require documentation of clinical worsening
IV prostacyclin and IV PAH therapies require prior authorization

Step Therapy

Endothelin Receptor Antagonist Step Requirement

Prior failure documentation is required before approving macitentan (Opsumit) or Opsynvi (macitentan/tadalafil). For Opsynvi, alternative is established dual therapy with single-ingredient macitentan plus tadalafil. For Opsumit or Opsynvi approvals include baseline hemoglobin and liver function tests and REMS compliance where applicable.

Documented failure of ambrisentan or bosentan (include 6MWD changes, clinical worsening) required before macitentan/Opsynvi
Opsynvi alternative: established on macitentan + tadalafil
Baseline hemoglobin and LFTs required and monthly monitoring as specified
REMS requirements must be satisfied

Denial Risk

Concurrent Therapy Conflicts

Certain concurrent therapies are contraindicated and will be denied if used together (verify via chart notes and pharmacy paid claims). Examples include PDE-5 inhibitors or tadalafil/sildenafil with Adempas (riociguat), and Adempas with opsynvi/macitentan-tadalafil. Avoid concurrent organic nitrates with PDE-5 inhibitors or tadalafil/sildenafil.

Do not use sildenafil or tadalafil concurrently with riociguat (Adempas)
Do not use Opsynvi concurrently with riociguat
Avoid concurrent use of organic nitrates with sildenafil/tadalafil

Denial Risk

Delay or Denial Risk

Incomplete forms or missing supporting documentation (prescriber signature, chart notes, diagnostic confirmation, lab results, REMS) may delay or result in denial of the authorization. Use pharmacy paid claims to verify prior therapies when available. Note: use of samples to initiate therapy does not meet step-edit/preauthorization criteria.

Missing prescriber signature or illegible form may delay/deny request
Attach chart notes and diagnostics proving diagnosis and prior therapy failures
Samples do not satisfy step-edit/prior authorization requirements

Key Definitions and Clinical Thresholds

Hemodynamic criteria for PAH

mPAP thresholdmPAP ≥ 20 mmHg

PAWP thresholdPAWP ≤ 15 mmHg

PVR thresholdPVR ≥ 2 Wood units

CTEPH — definition & riociguat relevance

DefinitionChronic Thromboembolic Pulmonary Hypertension (CTEPH): persistent/recurrent Group 4 PH confirmed by V/Q scan or pulmonary angiography

Surgical eligibilityMember previously failed pulmonary endarterectomy or is inoperable — documentation required

Relevance to riociguatRiociguat (Adempas) may be covered for CTEPH when CTEPH criteria and documentation are met

NYHA Functional Classification — use for coverage eligibility

PurposeNYHA Functional Classification used to document symptom severity for coverage eligibility

Common classes for coverageMost agents require NYHA Class II or III; some agents (e.g., bosentan for adults) allow II–IV; inhaled prostacyclins generally for Class III (Ventavis allowed for III–IV)

DocumentationProvider medical chart notes must verify NYHA functional class for prior authorization

Background

Pulmonary arterial hypertension (PAH, WHO Group 1) is a hemodynamic disorder confirmed by right heart catheterization and managed with therapies targeting the PDE‑5, endothelin, sGC, and prostacyclin pathways. Diagnosis for coverage requires documented hemodynamics (mean pulmonary arterial pressure, wedge pressure, and pulmonary vascular resistance) and a PAH risk assessment; treatment selection and authorization lengths depend on whether the request is for initiation (approval length: 6 months) or continuation (approval length: 12 months) of therapy.

Site of Care and Specialty Pharmacy

Billing Rule

Specialty Pharmacy supply and plan coverage may vary

Medication is provided by the designated Specialty Pharmacy (Proprium Rx); coverage and whether the drug is on‑formulary may vary by plan — if non‑formulary, submit documentation of medical necessity.

Medication supplied through Specialty Pharmacy – Proprium Rx
Not all drugs may be covered under every plan; non‑formulary drugs require medical necessity documentation

Policy Summary

PayerAvMed

PolicyPulmonary Arterial Hypertension (PAH) Drugs — Pharmacy Prior Authorization and Step‑Edit

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

Key ActionSubmit a completed, signed prior authorization form with diagnosis, NYHA functional class, right heart catheterization results, and supporting chart notes and labs.

SourceLink

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