Pulmonary Arterial Hypertension Endothelin Receptor Antagonists
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Defines Cigna prior authorization, coverage criteria, and medical necessity for ambrisentan, macitentan, macitentan/tadalafil (Opsynvi), and bosentan for pulmonary arterial hypertension and selected other indications.
New stand-alone policy created; criteria were previously housed in the Pulmonary Hypertension Therapy class policy.
Updated PAH criteria to clarify 'Initial Therapy' versus 'Patient is Currently Receiving the Requested Endothelin Receptor Antagonist' and removed echocardiogram as an option to confirm PAH diagnosis.
Added Opsynvi (sotatercept) to the policy with additional preferred product criteria requirements for Individual and Family Plans and a condition not covered statement prohibiting concurrent use with guanylate cyclase stimulators.
Added new 'Other Uses with Supportive Evidence' criteria for bosentan (Tracleer) for chronic thromboembolic pulmonary hypertension (CTEPH) and for digital ulcers in systemic sclerosis, including preferred product criteria for the brand.
Added preferred product criteria for Tracleer (bosentan tablets for oral suspension) for Employer Plans and for Opsumit and added generic macitentan with same criteria as brand.
Coverage and Medical Necessity Criteria
Initial and continuation therapy for WHO Group 1 PAH
Approve for the duration noted if the patient meets ONE of the following (A or B):
Do not flatten — both initial and continuation pathways require documented right heart catheterization and specialist involvement.
Continuation approvals require prior diagnostic confirmation; see documentation requirements.
Bosentan — chronic thromboembolic pulmonary hypertension (CTEPH)
Approve bosentan for CTEPH for 1 year if ALL of the following are met:
For CTEPH, prior trial of Adempas is required unless contraindicated or patient is already on bosentan.
Bosentan — digital ulcers in systemic sclerosis
Approve bosentan for digital ulcers in systemic sclerosis for 1 year if ONE of the following is met:
EULAR guidance notes Tracleer may be considered to reduce new digital ulcers, particularly in patients with recurrent ulcers despite prior therapies.
Plan-specific product and Opsynvi criteria
Brand product coverage conditions for Employer and Individual/Family Plans
Applies to Letairis (ambrisentan), Opsumit (macitentan), and Tracleer (bosentan) formulations as noted in the policy tables.
These criteria apply specifically to Individual and Family Plans as described in the policy.
Coverage criteria
Covered when criteria for the specific indication and product-preference requirements are met.
Includes requirement for documented PAH diagnosis by right heart catheterization; echocardiogram removed as an acceptable diagnostic option.
Concurrent use of Opsynvi with guanylate cyclase stimulators is listed under Conditions Not Covered and may trigger denial.
Employer plan preferred product additions and effective dates are noted in the revision history.
Preferred product criteria for Opsumit were also added as described in the revision details.
The following uses of the listed endothelin receptor antagonists are considered not medically necessary: ambrisentan (Letairis, generic), macitentan (Opsumit, generic), and bosentan (Tracleer, generic) when prescribed for any indication not specified in this policy. Opsynvi (sotatercept) is also considered not medically necessary for any other use beyond indications addressed in this policy.
Concurrent use of Opsynvi with guanylate cyclase stimulators is listed under Conditions Not Covered and is considered contraindicated. The policy specifically notes an example guanylate cyclase stimulator as Adempas (riociguat).
Ambrisentan, macitentan, bosentan, and Opsynvi are considered not medically necessary for any indications that are not specified in this policy. The policy states these determinations will be updated as new published data become available.
Aside from the explicit exclusion prohibiting concurrent use of Opsynvi with guanylate cyclase stimulators, no additional specific not medically necessary conditions were added in the revision notes; other NMN language beyond this concurrent-use exclusion is not present in the cited revision details.
Product Identifiers, Diagnosis Confirmation, and Codes
Prior Authorization, Documentation, and Denial Risks
Prior authorization required for listed ERAs and Opsynvi
Prior Authorization is required for benefit coverage of ambrisentan, macitentan, Opsynvi, and bosentan. Approvals are provided for the durations noted in the policy and require that the agent be prescribed by or in consultation with a specialist in the condition being treated.
PA requirements updated to include Opsynvi and preferred-product rules
Prior authorization requirements were updated to explicitly include Opsynvi and to add preferred product criteria for specific brands/generics (e.g., Tracleer, Opsumit, generic macitentan); PAH-specific documentation requirements now apply under these authorization rules.
Step therapy: Adempas required before bosentan for CTEPH
For chronic thromboembolic pulmonary hypertension (CTEPH), approval of bosentan requires a prior trial of Adempas unless the prescriber documents that Adempas is contraindicated or the patient is already receiving bosentan.
- If Adempas is contraindicated, examples include concurrent nitrates or nitric oxide donors, concurrent phosphodiesterase inhibitors (e.g., sildenafil or tadalafil), hypotension, or risk for hypotension.
Preferred product / step edits may require brand-to-generic trials
Preferred-product and step edits were added for specific brands and generics (including Tracleer, Opsumit, and generic macitentan) which may require trial of a preferred product or impose product-preference edits per plan type.
- Employer and Individual/Family Plans have brand-to-generic trial requirements and specific preferred-product rules listed in the policy tables.
Document initiation of therapy (chart notes, cath lab reports) with patient identifiers
Documentation is required for initiation of therapy and may include chart notes and catheterization laboratory reports; all documentation must include patient‑specific identifying information. Prior right heart catheterization documentation provided for a different WHO Group 1 PAH medication is considered to meet the requirement.
- Documentation may include chart notes and catheterization laboratory reports.
- Previously supplied right heart catheterization documentation for another WHO Group 1 PAH medication satisfies the requirement.
PAH diagnostic documentation must include right heart catheterization (echocardiogram removed)
For PAH indications, documentation must include confirmation of diagnosis by right heart catheterization; echocardiogram is no longer accepted as acceptable diagnostic confirmation.
- Right heart catheterization must demonstrate mPAP >20 mmHg, PAWP ≤15 mmHg, and PVR >2 Wood units as diagnostic confirmation.
Coding/submission denial risk for missing covered codes
Claims submitted without covered diagnosis or procedure codes under the applicable Coverage Policy will be denied as not covered; providers must bill using the most appropriate covered codes.
- When billing, providers must use the most appropriate codes as of the effective date of the submission.
Concurrent-use denial risk: Opsynvi with guanylate cyclase stimulators
Concurrent use of Opsynvi with guanylate cyclase stimulators is listed under Conditions Not Covered and may trigger denial of coverage for concurrent therapy.
- The policy specifically added 'Concurrent Use with Guanylate Cyclase Stimulators' as a Conditions Not Covered statement for Opsynvi (an example guanylate cyclase stimulator is noted in a policy Note).
Background and Scope
Endothelin receptor antagonists (ambrisentan, macitentan, bosentan) and the combined product Opsynvi are oral therapies principally used to treat WHO Group 1 pulmonary arterial hypertension (PAH). These agents are intended to improve exercise capacity and reduce disease progression or hospitalization in PAH. Right heart catheterization with hemodynamic confirmation (mean pulmonary artery pressure >20 mmHg, pulmonary arterial wedge pressure ≤15 mmHg, and pulmonary vascular resistance >2 Wood units) is required for initial coverage, and the policy revision clarified diagnostic confirmation by removing echocardiogram as an acceptable alternative.
Key Definitions and Agent Notes
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