CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Uptravi (selexipag) — Coverage Criteria for Pulmonary Arterial Hypertension (WHO Group 1)

Covers clinical criteria and authorization requirements for Uptravi (selexipag) use in pulmonary arterial hypertension (WHO Group 1) for members of Neighborhood Health Plan of Rhode Island following payer criteria.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyUptravi (selexipag) — Coverage Criteria for Pulmonary Arterial Hypertension (WHO Group 1)

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization with documentation of right heart catheterization showing PCWP < 15 mmHg, mPAP > 20 mmHg, and PVR ≥ 3 Wood units and prescriber specialty (pulmonologist or cardiologist).

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsauthorization period

WHO Group 1disease group

<15 mmHgPCWP threshold

>20 mmHgmPAP threshold

≥3 WUPVR threshold

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Coverage and Medical Necessity Criteria

Initial therapy (PAH)

Covered when ALL of the following are met:

FDA indication: Uptravi is used for treatment of pulmonary arterial hypertension (WHO Group 1) to delay disease progression and reduce risk of hospitalization; effectiveness established in PAH patients with WHO FC II-III

supports covered indication

Prescriber specialty: Must be prescribed by or in consultation with a pulmonologist or cardiologist

see prescriber requirement

Hemodynamic confirmation: PAH confirmed by right heart catheterization with pulmonary capillary wedge pressure (PCWP) < 15 mmHg, mean pulmonary arterial pressure (mPAP) > 20 mmHg, and pulmonary vascular resistance (PVR) ≥ 3 Wood units in adults (PVRI ≥ 3 Wood units·m2 in pediatrics); for infants < 1 year, Doppler echocardiogram acceptable if catheterization cannot be performedPCWP <15; mPAP >20; PVR ≥3 WU

required diagnostic tests

Continuation therapy

Continuation of 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

continuation eligibility

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

documentation of stability or improvement required

All indications for Uptravi (selexipag) outside the FDA‑approved and compendial uses specified in this policy are considered experimental/investigational and not medically necessary.

Use of Uptravi for non‑FDA approved indications is not medically necessary.

Diagnostic & Hemodynamic Thresholds

Pulmonary hemodynamic thresholds

Pulmonary capillary wedge pressure (PCWP)< 15 mmHg

Mean pulmonary arterial pressure (mPAP)> 20 mmHg

Pulmonary vascular resistance (PVR) — adults≥ 3 Wood units

Pulmonary vascular resistance index (PVRI) — pediatrics≥ 3 Wood units·m2

Hemodynamic PAH criteria (RHC)

Required diagnostic testRight heart catheterization confirming hemodynamics

PCWP threshold

Prescriber Requirements, Authorization, and Documentation

Prior Authorization

Prior Authorization: 12‑Month Approvals

Prior authorization may be granted for up to 12 months when pulmonary arterial hypertension (PAH, WHO Group 1) is confirmed with required hemodynamic criteria and the prescriber specialty is documented. Continuation requests may also be approved for 12 months when the member is currently receiving the medication through a paid benefit and is demonstrating benefit or stability.

12‑month approvals when PAH (WHO Group 1) is confirmed with required hemodynamic criteria and prescriber specialty documented.
Continuation authorization: 12 months for members already receiving therapy with documented clinical benefit or stability.

Documentation Required

Prescriber and Continuation Documentation

This medication must be prescribed by or in consultation with a pulmonologist or cardiologist. For continuation requests, documentation from the prescriber must indicate ongoing benefit or disease stability (e.g., clinical notes, functional class, exercise tolerance, hospitalizations) to support continued coverage.

Clinical Background

Pulmonary arterial hypertension (PAH; WHO Group 1) is a progressive condition characterized by elevated pulmonary arterial pressures leading to increased pulmonary vascular resistance and right heart strain. Uptravi (selexipag) is indicated for treatment of PAH to delay disease progression and reduce the risk of hospitalization, with effectiveness demonstrated in patients with WHO Functional Class II–III symptoms, including idiopathic, heritable, and certain associated forms of PAH.

Definitions and Disease Grouping

WHO Group 1 (Pulmonary Arterial Hypertension)

WHO Group 1 (PAH) definitionPulmonary arterial hypertension (PAH) group including idiopathic, heritable, drug- and toxin-induced, and PAH associated with specified conditions

Examples — idiopathic and heritableIdiopathic PAH; Heritable PAH

Examples — drug/toxin and associated conditionsDrug- and toxin-induced PAH; PAH associated with connective tissue diseases, HIV infection, portal hypertension, congenital heart diseases, schistosomiasis

Example — treatment responders and special formsPAH long-term responders to calcium channel blockers; Persistent pulmonary hypertension of the newborn; PAH with venous/capillary (PVOD/PCH) involvement