CurrentCignaPolicy N/A

Pulmonary Arterial Hypertension - Endothelin Receptor Antagonists

Prior authorization policy for coverage of endothelin receptor antagonists (ambrisentan, macitentan, macitentan/tadalafil, bosentan) for treatment of pulmonary arterial hypertension and specified other indications; applies to Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicyPulmonary Arterial Hypertension - Endothelin Receptor Antagonists

Policy CodePolicy N/A

Change TypeAdded Opsynvibosentan clarifications

Effective DateN/A

Next Review DateOct 2, 2024

Key ActionObtain prior authorization and document right heart catheterization confirming WHO Group 1 PAH; prescriptions should be by or in consultation with a cardiologist or pulmonologist.

SourceLink

POLICY UPDATE CHANGES

Added Opsynvi (macitentan/tadalafil) to the Policy.

Clarified that chronic thromboembolic pulmonary hypertension and digital ulcer coverage applies to bosentan products (Tracleer or generic), not just the brand.

Bosentan tablets for oral suspension are now available as generic; no criteria changes.

4products listed

1 yrtypical approval duration

WHO Group 1primary covered indication

<20k

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Coverage Criteria for Endothelin Receptor Antagonists

COVERAGE CRITERIA

Approve for the duration noted if the patient meets ONE of the following (A or B):

A or B

A Initial Therapy: i. Patient has a diagnosis of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH); AND ii. Patient meets BOTH of the following (a and b): a) Patient has had a right heart catheterization [documentation required]; AND b) Results of the right heart catheterization confirm the diagnosis of WHO Group 1 PAH; AND iii. The medication is prescribed by or in consultation with a cardiologist or a pulmonologist
B Currently Receiving: i. Patient has a diagnosis of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH); AND ii. Patient meets BOTH of the following (a and b): a) Patient has had a right heart catheterization (prior to starting therapy); AND b) Results of the right heart catheterization confirm the diagnosis of WHO Group 1 PAH; AND iii. The medication is prescribed by or in consultation with a cardiologist or a pulmonologist

Opsynvi (macitentan/tadalafil) must not be used concurrently with guanylate cyclase stimulators. Concurrent use with a guanylate cyclase stimulator (for example, Adempas/riociguat) is contraindicated, and coverage is not recommended for that combination. When evaluating coverage for Opsynvi, ensure documentation shows the patient is not receiving a guanylate cyclase stimulator concurrently.

Endothelin receptor antagonists listed in this policy (Letairis/ambrisentan, Opsumit/macitentan, Opsynvi/macitentan‑tadalafil, and Tracleer/bosentan) are considered not medically necessary for any uses other than those specifically listed in the Recommended Authorization Criteria. Coverage is not recommended for circumstances outside the criteria set forth in this policy; the list of noncovered uses may be updated as new data become available.

Coding, Diagnostic Criteria, and Hemodynamics

Coding guidancemixed

affected codes

Most appropriate codes as of submission date must be used; claims without covered code(s) will be denied.

Right heart catheterization confirmation — Hemodynamic criteria

Mean pulmonary artery pressure (mPAP)Greater than 20 mmHg (at rest)

Pulmonary arterial wedge pressure (PAWP)Less than or equal to 15 mmHg

Pulmonary vascular resistance (PVR)Greater than 2 Wood units

Prior Authorization, Documentation, and Step Therapy

Prior Authorization

Prior Authorization Recommended

Prior authorization is recommended for ambrisentan, macitentan, macitentan/tadalafil, and bosentan. Approvals are provided for the duration noted in the policy. Because of the specialized skills required for evaluation and diagnosis and the monitoring required for adverse events and long‑term efficacy, these agents must be prescribed by or in consultation with a specialist (cardiologist or pulmonologist).

Applies to: ambrisentan (Letairis, generic), Opsumit, Opsynvi, macitentan, macitentan/tadalafil, bosentan (Tracleer, generic).
Approvals are provided for the duration specified in the policy (typically 1 year for initial or continuation therapy when criteria are met).

Documentation Required

For initiation of therapy, documentation is required as specified in the criteria ("documentation required"). Documentation may include, but is not limited to, chart notes and catheterization laboratory reports and must contain patient‑specific identifying information. If right heart catheterization documentation has already been provided for prior authorization review for another WHO Group 1 PAH medication, that documentation is considered to meet the requirement for these agents.

Background

Pulmonary arterial hypertension (PAH, WHO Group 1) is a rare, progressive disorder characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance that can lead to right heart failure. Endothelin receptor antagonists are therapies used to improve exercise capacity and reduce clinical worsening in adults with PAH. For diagnostic confirmation, right heart catheterization is required to document hemodynamics consistent with PAH.

Definitions and Diagnostic Thresholds

PAH diagnostic hemodynamic definition

Mean pulmonary artery pressure (mPAP)> 20 mmHg at rest

Pulmonary arterial wedge pressure (PAWP)≤ 15 mmHg

Pulmonary vascular resistance (PVR)> 2 Wood units (measured by cardiac catheterization)