CurrentCignaPolicy IP0631

Pulmonary Arterial Hypertension - Endothelian Receptor Antagonists - (IP0631)

Defines prior authorization and medical necessity criteria for ambrisentan (Letairis, generic), macitentan (Opsumit), macitentan/tadalafil combination (Opsynvi), and bosentan (Tracleer, generic) for PAH (WHO Group 1), and for bosentan in CTEPH and digital ulcers in systemic sclerosis, across Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyPulmonary Arterial Hypertension - Endothelian Receptor Antagonists - (IP0631)

Policy CodePolicy IP0631

Change TypeNew stand-alone policy; multiple additions and clarifications

Effective DateFeb 1, 2026

Next Review Date

Key ActionPrior authorization is required for coverage; ensure documentation of right heart catheterization and that prescriptions are by or in consultation with a cardiologist or pulmonologist.

SourceLink

POLICY UPDATE CHANGES

New stand-alone policy created; criteria were previously in Pulmonary Hypertension Therapy class policy.

PAH criteria clarified for 'Initial Therapy' versus 'Patient is Currently Receiving the Requested Endothelin Receptor Antagonist' and removed echocardiogram as an option for confirmation of PAH diagnosis (right heart catheterization required).

Added Opsynvi to the policy with additional preferred product criteria requirements for Individual and Family Plans.

Added new criteria supporting bosentan for CTEPH and for digital ulcers in systemic sclerosis, with preferred product criteria requirements for Tracleer.

Conditions Not Covered: Added statement that Opsynvi concurrent use with guanylate cyclase stimulators is contraindicated.

Documentation requirement for right heart catheterization added to PAH indication and noted that bosentan tablets for oral suspension are available as generic.

4Drugs addressed (ambrisentan, macitentan, macitentan/tadalafil, bosentan)

Coverage Summary

Coverage stance: covered_with_criteria. Scope: Defines prior authorization and medical necessity criteria for ambrisentan (Letairis, generic), macitentan (Opsumit), macitentan/tadalafil (Opsynvi), and bosentan (Tracleer, generic) for PAH (WHO Group 1), and for bosentan in CTEPH and digital ulcers in systemic sclerosis. Primary subject: Endothelin Receptor Antagonists for Pulmonary Arterial Hypertension (WHO Group 1). Effective date: 2026-02-01. Last review date: 2025-12-18. Other supported indications: CTEPH and digital ulcers in systemic sclerosis. Primary treated condition: PAH (WHO Group 1).

Initial Therapy Criteria — PAH (WHO Group 1)

Medically Necessary - PAH (WHO Group 1)

Approve for 1 year if the patient meets ONE of the following (A or B):

ANY of the following

A) Initial Therapy
- Patient has a diagnosis of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH)
- Right heart catheterization confirmation
  - Patient has had a right heart catheterization
  - Results of the right heart catheterization confirm the diagnosis of WHO Group 1 PAH
- Medication is prescribed by or in consultation with a cardiologist or a pulmonologist
- Preferred product criteria is met for the product(s) as listed in the product-specific tables
B) Patient is Currently Receiving the Requested ERA or Opsynvi
- Patient has a diagnosis of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH)
- Prior diagnostic documentation
  - Patient has had a right heart catheterization
  - Results of the right heart catheterization confirm the diagnosis of WHO Group 1 PAH
- Medication is prescribed by or in consultation with a cardiologist or a pulmonologist

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Continuation / Currently Receiving Therapy Criteria

Medically Necessary - PAH (WHO Group 1) — Continuation

B) Patient is Currently Receiving the Requested ERA or Opsynvi

Patient has a diagnosis of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH)
Prior diagnostic documentation
- Patient has had a right heart catheterization
- Results of the right heart catheterization confirm the diagnosis of WHO Group 1 PAH
Medication is prescribed by or in consultation with a cardiologist or a pulmonologist

Bosentan — CTEPH Criteria

Medically Necessary - Bosentan (Tracleer) for CTEPH

Approve bosentan for 1 year if the patient meets BOTH of the following (A, B, and C):

ALL of the following

A) Prior use or contraindication to Adempas
- Patient has tried Adempas
- According to the prescriber, use of Adempas is contraindicated
  Examples: patient receiving nitrates or nitric oxide donors; receiving a phosphodiesterase inhibitor such as sildenafil or tadalafil; hypotensive or at risk for hypotension
- Patient is currently receiving bosentan (Tracleer, generic)

Bosentan — Digital Ulcers in Systemic Sclerosis Criteria

Medically Necessary - Bosentan (Tracleer) for Digital Ulcers in Systemic Sclerosis

Approve bosentan for 1 year if the patient meets ONE of the following (A or B) AND C:

ALL of the following

ANY of the following
- Patient has tried one calcium channel blocker (examples: amlodipine, felodipine, nifedipine)
- Patient has tried one phosphodiesterase type 5 (PDE5) inhibitor (examples: sildenafil, tadalafil, vardenafil)
C: Preferred product criteria is met for the product(s) as listed in the product-specific tables

Not Medically Necessary / Exclusions and Contraindications

Not Medically Necessary / Exclusions

Not medically necessary for any other use unless criteria updated with new evidence. Specific contraindication:

Use of Opsynvi with guanylate cyclase stimulators is contraindicated (example: Adempas/riociguat)

A specific contraindication was added stating that concurrent use of Opsynvi with guanylate cyclase stimulators (e.g., riociguat/Adempas) is contraindicated; this change was introduced when the new stand‑alone policy was created and Opsynvi was added (revision notes) and is listed under Conditions Not Covered. Per the policy provider actions and policy language, this concurrent use is not covered.

Applicable Drugs / Codes

Covered Drugs (HCPCS/NDC not specified in document)mixedCovered

ambrisentan	Letairis and generic - endothelin receptor antagonist
macitentan	Opsumit - endothelin receptor antagonist
macitentan/tadalafil	Opsynvi - macitentan/tadalafil combination
bosentan	Tracleer and generic - endothelin receptor antagonist (tablets and oral suspension)

Covered Drugs (HCPCS/NDC not specified in document)mixedCovered

ambrisentan	Letairis and generic - endothelin receptor antagonist
macitentan	Opsumit - endothelin receptor antagonist
macitentan/tadalafil	Opsynvi - macitentan/tadalafil combination
bosentan	Tracleer and generic - endothelin receptor antagonist (tablets and oral suspension)

Provider Actions & Authorization Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for coverage of ambrisentan, Opsumit, Opsynvi, and bosentan; approvals provided for specified durations (typically 1 year).

Typical approval duration: 1 year
Affected products: ambrisentan, Opsumit, Opsynvi, bosentan

Documentation Required

Right Heart Catheterization Documentation

Documentation of right heart catheterization and results confirming WHO Group 1 PAH is required for initiation and is considered met if previously provided for a different WHO Group 1 PAH medication prior authorization review.

Right heart catheterization required with results confirming WHO Group 1 PAH

Clinical Evidence

Randomized placebo-controlled trial n=156BENEFiT trial (bosentan in CTEPH) showed improvements in some hemodynamic parameters

Trials showed reduction in new digital ulcers; primary endpoint met in RAPIDS-1RAPIDS-1/2 (bosentan in digital ulcers) showed reduction in new digital ulcers; RAPIDS-1 primary endpoint met

Evidence entries: The brief lists the BENEFiT randomized placebo‑controlled trial (n=156) of bosentan in CTEPH showing improvements in some hemodynamic parameters, and the RAPIDS‑1/2 trials of bosentan in systemic sclerosis with digital ulcers where trials showed a reduction in new digital ulcers (RAPIDS‑1 primary endpoint met; RAPIDS‑2 also showed reduction over 24 weeks).

Background & Definitions

Background: Endothelin receptor antagonists (ambrisentan/Letairis, macitentan/Opsumit, macitentan/tadalafil/Opsynvi, bosentan/Tracleer) are indicated for pulmonary arterial hypertension (WHO Group 1). Bosentan has evidence in CTEPH and in reducing new digital ulcers in systemic sclerosis. PAH is defined as mPAP > 20 mmHg, PAWP ≤ 15 mmHg, and PVR > 2 Wood units measured by cardiac catheterization.

Revision History

2024-07-18added

New stand-alone policy created; criteria were previously housed in the Pulmonary Hypertension Therapy class policy. Pulmonary Arterial Hypertension (PAH) criteria updated to clarify 'Initial Therapy' versus 'Patient is Currently Receiving the Requested Endothelin Receptor Antagonist' and removed echocardiogram as an option for confirmation of PAH diagnosis (right heart catheterization required). Opsynvi added to the policy with additional preferred product criteria requirements for Individual and Family Plans. New criteria added supporting bosentan for CTEPH and for digital ulcers in systemic sclerosis, with preferred product criteria requirements for Tracleer.

2024-08-15added

Conditions Not Covered updated: Added statement that Opsynvi concurrent use with guanylate cyclase stimulators is contraindicated. Effective Date listed for this addition: 8/15/2024.

2024-10-17