CurrentNeighborhood Health Plan of Rhode IslandPolicy 1645-A

Selexipag (Uptravi) — Coverage Criteria for Pulmonary Arterial Hypertension

Covers clinical authorization criteria and requirements for selexipag (Uptravi) for treatment of pulmonary arterial hypertension (WHO Group 1) for members of Neighborhood Health Plan of Rhode Island when approval criteria are met.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicySelexipag (Uptravi) — Coverage Criteria for Pulmonary Arterial Hypertension

Policy CodePolicy 1645-A

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization for up to 12 months when all coverage criteria are met; ensure documentation of diagnostic right heart catheterization and specialist prescriber involvement.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 moauthorization duration

WHO Group 1disease group

mPAP >20; PCWP ≤15; PVR >2hemodynamic criteria

PVRI >3pediatric threshold

Coverage Criteria for Selexipag (Uptravi)

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Initial therapy (PAH)

Covered when ALL of the following are met:

PAH indication: Member has pulmonary arterial hypertension defined as WHO Group 1 (PAH).WHO Group 1

See WHO classification appendix

Diagnostic confirmation: PAH confirmed by pretreatment right heart catheterization showing mean pulmonary arterial pressure (mPAP) > 20 mmHg, pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units (for pediatric members PVRI > 3 WU·m2 acceptable). For infants <1 year, Doppler echocardiogram acceptable if catheterization cannot be performed.see diagnostic thresholds

Hemodynamic criteria required

Prescriber specialty: Medication prescribed by or in consultation with a pulmonologist or cardiologist.specialist prescriber

Continuation therapy

Covered when ALL of the following are met:

Current therapy: Member is currently receiving the requested medication through a paid pharmacy or medical benefit.current therapy

12-month authorization may be granted when criteria met

Clinical benefit: Member is experiencing benefit from therapy as evidenced by disease stability or disease improvement.clinical benefit

Hemodynamic and Definition Details

Hemodynamic thresholds

Mean pulmonary arterial pressure (mPAP)> 20 mmHg

Pulmonary capillary wedge pressure (PCWP)≤ 15 mmHg

Pulmonary vascular resistance (PVR)> 2 Wood units

Pediatric alternative (PVRI)PVRI > 3 Wood units·m2 acceptable for pediatric members

Infants <1 year exceptionDoppler echocardiogram acceptable if right heart catheterization cannot be performed

Policy Definitions

PAH (WHO Group 1) — definition and included subtypes

DefinitionPulmonary arterial hypertension (PAH) — WHO Group 1 class of pulmonary hypertension

Included subtypesIdiopathic; long‑term responders to calcium channel blockers; heritable; drug- and toxin-associated

Associated conditionsConnective tissue disease, HIV infection, portal hypertension, congenital heart disease, schistosomiasis

Other variantsPAH with venous/capillary involvement (PVOD/PCH); persistent pulmonary hypertension of the newborn

Hemodynamic confirmation — pretreatment right heart catheterization

Required diagnostic procedurePretreatment right heart catheterization

mPAP thresholdMean pulmonary arterial pressure (mPAP) > 20 mmHg

PCWP thresholdPulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg

PVR thresholdPulmonary vascular resistance (PVR) > 2 Wood units (PVRI > 3 WU·m2 for pediatrics)

Infant exceptionFor infants <1 year, Doppler echocardiogram acceptable if catheterization cannot be performed

Provider Requirements and Authorization

Prior Authorization

Prior Authorization: 12‑month approval

Authorization of 12 months may be granted when all coverage criteria are met.

Authorization may be granted for up to 12 months when all coverage criteria are met.
Applies to initial and continuation authorizations when criteria are satisfied.

Step Therapy

Step therapy

No step therapy requirements for this medication. Prescriber specialty requirement still applies (prescription must be by or in consultation with a pulmonologist or cardiologist).

No step therapy required.
Prescriber specialty requirement: pulmonologist or cardiologist (or in consultation with).

Documentation Required

Diagnostic documentation required

Documentation of pulmonary arterial hypertension (PAH) diagnosis is required. Pretreatment right heart catheterization must show mean pulmonary arterial pressure (mPAP) > 20 mmHg, pulmonary capillary wedge pressure (PCWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units. For pediatric members, pulmonary vascular resistance index (PVRI) > 3 Wood units·m2 is acceptable. For infants under one year, Doppler echocardiogram confirmation is acceptable if right heart catheterization cannot be performed.

Right heart catheterization results documenting mPAP > 20 mmHg, PCWP ≤ 15 mmHg, and PVR > 2 Wood units.
PVRI > 3 Wood units·m2 acceptable for pediatric members.
Doppler echocardiogram acceptable for infants <1 year if catheterization not possible.

Note

Prescriber specialty

Prescription must be by or in consultation with a pulmonologist or cardiologist.

Prescriber specialty: pulmonologist or cardiologist (or in consultation with).

Denial Risk

Non‑FDA indications

Use for indications other than FDA‑approved PAH (WHO Group I) is considered experimental/investigational and not medically necessary.

FDA‑approved indication: treatment of PAH (WHO Group I) to delay disease progression and reduce risk of hospitalization.
All other indications are experimental/investigational and not medically necessary.

Background

Selexipag (Uptravi) is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression and reduce the risk of hospitalization for PAH. Effectiveness was established in a long‑term study in patients with WHO Functional Class II–III symptoms, including idiopathic, heritable, PAH associated with connective tissue disease, and PAH associated with repaired congenital heart disease shunts.

Revision History

2025document_versionLatest

2022-07clinical_reference_update

Package insert reference updated to Uptravi [package insert], Actelion Pharmaceuticals US, Inc.; July 2022.