UnitedHealthcare PAH Agents Prior Auth Coverage

Coverage Criteria

Initial Authorization (general PAH agents)

Covered when ALL of the following are met:

Initial Authorization - general agents: Pulmonary arterial hypertension is symptomatic

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Prescriber requirement: Medication is prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist

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Diagnostic confirmation or current therapy: Diagnosis of pulmonary arterial hypertension confirmed by right heart catheterization OR patient is currently on any therapy for pulmonary arterial hypertension

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Initial Authorization (oral suspension PDE-5 inhibitors)

Covered when either A OR B is met:

Path A (typical): Pulmonary arterial hypertension is symptomatic AND diagnosis of pulmonary arterial hypertension confirmed by right heart catheterization

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Path B (alternate): Patient is currently on any therapy for the diagnosis of pulmonary arterial hypertension AND patient is unable to ingest a solid dosage form (tablet or capsule) due to age, oral-motor difficulties, or dysphagia AND medication is prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist

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Initial Authorization (Orenitram/Uptravi)

Covered when continuation of therapy OR when all alternative initial requirements are met:

Continuation path: Continuation of therapy for established users, including evidence of current use from manufacturer sample or manufacturer-sponsored assistance program; patient is not taking Orenitram/Uptravi in combination with a prostanoid/prostacyclin analogue; AND prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist

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Alternative initial path with step requirements: Pulmonary arterial hypertension is symptomatic AND diagnosis confirmed by right heart catheterization OR patient is currently on any therapy for PAH; AND history of failure, contraindication, or intolerance to BOTH a PDE-5 inhibitor (e.g., sildenafil or tadalafil) AND an endothelin receptor antagonist (e.g., ambrisentan, macitentan, bosentan/ERA) ; AND patient is not taking Orenitram/Uptravi in combination with a prostanoid/prostacyclin analogue; AND prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist

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Reauthorization

Covered when documentation of ongoing clinical benefit is provided:

Reauthorization - general agents: Documentation the patient is receiving clinical benefit from therapy (Adempas, Opsumit, Opsynvi, Letairis, Tracleer, Tyvaso, Tyvaso DPI, or Ventavis)

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Reauthorization - oral suspensions: Documentation the patient is receiving clinical benefit from Revatio/Liqrev/Tadliq suspension therapy AND patient remains unable to ingest a solid dosage form due to age, oral-motor difficulties, or dysphagia

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Reauthorization - Orenitram/Uptravi: Documentation the patient is receiving clinical benefit from Orenitram or Uptravi therapy AND patient is not taking Orenitram/Uptravi in combination with a prostanoid/prostacyclin analogue

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Reauthorization - Adempas for CTEPH:

Initial Authorization (Adempas for CTEPH)

Covered when ALL of the following are met:

Adempas for CTEPH (initial): Diagnosis of inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) AND CTEPH is symptomatic AND prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist OR patient is currently on any therapy for CTEPH with specialist prescribing/consultation

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Initial Authorization (PH associated with interstitial lung disease)

Tyvaso or Tyvaso DPI are covered when ALL of the following are met:

Tyvaso/Tyvaso DPI for PH-ILD: Diagnosis of pulmonary hypertension associated with interstitial lung disease (WHO Group 3) confirmed by right heart catheterization AND interstitial lung disease demonstrated by computed tomography of the chest showing diffuse parenchymal lung disease AND pulmonary hypertension is symptomatic AND prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist12 months authorization period

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Initial Authorization — Adempas for CTEPH (alternate reference)

Covered when ALL of the following are met:

Adempas for inoperable or persistent/recurrent CTEPH (initial)

Prescriber: Prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist

Reauthorization — Adempas

Reauthorization is covered when the following is provided:

Adempas reauthorization: Documentation that the patient is receiving clinical benefit from Adempas therapy12 months authorization period

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Initial Authorization — Tyvaso / Tyvaso DPI (PH-ILD)

Covered when ALL of the following are met:

Tyvaso or Tyvaso DPI initial authorization: Diagnosis of pulmonary hypertension associated with interstitial lung disease (WHO group 3) confirmed by right heart catheterization AND interstitial lung disease diagnosed by CT evidence of diffuse parenchymal lung disease AND pulmonary hypertension is symptomatic AND prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist12 months authorization period

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Reauthorization — Tyvaso / Tyvaso DPI

Reauthorization is covered when the following is provided:

Tyvaso reauthorization: Documentation of positive clinical response to Tyvaso or Tyvaso DPI (improved exercise ability)12 months authorization period

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The intravenous (IV) formulation of sildenafil is not covered under the UnitedHealthcare pharmacy benefit because it is not self‑administered. This policy’s pharmacy prior authorization criteria and coverage rules apply only to outpatient, self‑administered formulations (for example, oral suspensions) and therefore exclude IV sildenafil from coverage under the pharmacy benefit.

Certain branded products — including Letairis, Liqrev oral suspension, Opsynvi, and Revatio oral powder for suspension — are typically excluded from coverage by plan policy. Plan‑specific benefit design and exclusions may apply; providers should consult the member’s plan documents for any tried/failed or exclusion rules that could affect coverage eligibility.

Non‑self‑administered IV formulations are outside the pharmacy benefit and therefore are not covered under this policy. Where intravenous administration is indicated and billed as a medical (facility or physician) service, coverage will be determined under the applicable medical benefit, not under these pharmacy PAH criteria.

Concomitant use of oral phosphodiesterase‑5 (PDE‑5) inhibitors with Adempas (riociguat) is contraindicated. The policy identifies the combination as potentially not medically appropriate and may result in denial of coverage when these agents are prescribed together.

Coding and Drug Lists

Covered NDCs (oral suspension/powder formulations)NDCCovered

(NDCs to be populated)	Revatio (sildenafil) oral powder for suspension* — oral suspension formulation; IV formulation not covered under pharmacy benefit
(NDCs to be populated)	Liqrev (sildenafil) oral suspension — oral suspension formulation
(NDCs to be populated)	Tadliq (tadalafil) oral suspension — oral suspension formulation

Relevant ICD-10 diagnosis codesICD-10Covered

I27.0	Primary pulmonary hypertension (pulmonary arterial hypertension)
I27.2	Other secondary pulmonary hypertension (includes pulmonary hypertension due to lung diseases and hypoxia)
I27.20	Pulmonary hypertension, unspecified
I27.21	Pulmonary hypertension due to left heart disease
I27.22	Pulmonary hypertension due to lung disease and/or hypoxia
I27.23	Chronic thromboembolic pulmonary hypertension (CTEPH)
J84.9	Interstitial pulmonary disease, unspecified (used with group 3 PH)

Inability to ingest solid dosage form — reasons (age, oral-motor difficulties, dysphagia)

Reasons patient unable to ingest solid dosage formage; oral-motor difficulties; dysphagia

Authorization period — typical approval duration

Authorization periodAuthorization will be issued for 12 months

Provider Actions and Authorization Procedures

Prior Authorization

Prior Authorization Required

Prior authorization is required for initiation and continuation of pulmonary arterial hypertension (PAH) agents. Authorizations are typically issued for 12 months unless otherwise specified. The medication must be prescribed by or in consultation with a cardiologist, pulmonologist, or rheumatologist. Documentation must include a diagnosis of pulmonary arterial hypertension confirmed by right heart catheterization, evidence the disease is symptomatic, and prior treatment history where step therapy is required. For reauthorization, submit documentation of clinical benefit (for example, improved exercise tolerance or symptoms).

Authorizations issued for 12 months (unless otherwise specified).
Prescriber requirement: prescribing clinician or consultation must be a cardiologist, pulmonologist, or rheumatologist.
Diagnostic confirmation: right heart catheterization required to confirm PAH when specified.
Reauthorization requires documentation of clinical benefit (e.g., improved exercise ability, symptom improvement).
Providers must submit prior treatment history when step therapy criteria apply.

Step Therapy

Definitions

Pulmonary arterial hypertension (PAH) — disease description and clinical features

DefinitionPulmonary arterial hypertension (PAH) is a progressive disease characterized by elevated pressure in the pulmonary vessels leading to ventricular dysfunction, reduced exercise capacity, risk of right‑sided heart failure, and increased mortality

Mechanisms and drug classesPAH pathogenesis involves multiple mechanisms; treatment classes include ERAs, PDE‑5 inhibitors, prostacyclin analogs, sGC stimulators, and prostacyclin receptor agonists

Clinical featuresPatients commonly have symptomatic exercise intolerance and may show hemodynamic abnormalities measured by right heart catheterization

Oral suspension formulations — scope and exclusions

Scope of policyCriteria explicitly apply to oral suspension formulations of certain agents (e.g., sildenafil oral suspension)

IV formulations excluded

Background

Pulmonary arterial hypertension (PAH) is a progressive vascular disease characterized by elevated pressure in the pulmonary circulation that leads to reduced exercise capacity and can progress to right‑sided ventricular dysfunction and heart failure. Multiple drug classes are used to treat PAH — including endothelin receptor antagonists, phosphodiesterase‑5 inhibitors, prostacyclin analogs, soluble guanylate cyclase stimulators, and prostacyclin receptor agonists — and prescribing decisions are guided by disease severity, functional status, and formulation considerations. This policy focuses on outpatient, self‑administered therapies (notably oral suspensions) and excludes non‑self‑administered IV formulations from the pharmacy benefit.

Revision History

2014-12program_creation

Policy program established (New_program) for prior authorization/medical necessity of PAH agents.

2015-05criteria_update_step_therapies

Added new step requirement for Adcirca (sildenafil), Adempas, and Orenitram; added criteria for Revatio solution.

2015-05criteria_cleanup

Removed PAH diagnosis language in favor of requiring submission of medical records documenting diagnosis confirmed by right heart catheterization; removed some step edits for Adcirca and Adempas; added Adempas as an alternative in Orenitram step; decreased Orenitram initial authorization period to 6 months and reauthorization to 12 months.

OpenPayer

Prior Authorization / Medical Necessity — Pulmonary Arterial Hypertension (PAH) Agents (pharmacy benefit)

Coverage Criteria

Coding and Drug Lists

Provider Actions and Authorization Procedures

Definitions

Background

Revision History