Pulmonary Arterial Hypertension

POLICY NUMBER

L.5.01.465

Adcirca (tadalafil) tabletsAdempas (riociguat) tabletsFlolan (epoprostenol) injectionLetairis (ambrisentan) tabletsLiqrev (sildenafil) oral suspensionOpsumit (macitentan) tabletsOpsynvi (macitentan-tadalafil) tabletsOrenitram (treprostinil) tabletsRemodulin (treprostinil) injectionRevatio (sildenafil) tablets, oral suspension, injectionTadliq (tadalafil) oral suspensionTracleer (bosentan) tabletsTyvaso (treprostinil) inhalation solutionTyvaso DPI (treprostinil) inhalation powderUptravi (selexipag) tablets, injectionVeletri (epoprostenol) injectionVentavis (iloprost) inhalation solutionWinrevair (sotatercept-csrk) subcutaneous injectionYutrepia (treprostinil) inhalation powder

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Pulmonary arterial hypertension (PAH) is an abnormally high blood pressure in the arteries between the heart and lungs. PAH significantly reduces the ability of patients to exert themselves physically without becoming short of breath. PAH significantly shortens the life span of patients because it leads to heart failure.

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. 

Initial Criteria

The requested medication may be considered medically necessary when ALL of the following criteria are met:

1. ONE of the following:

   1. The individual has a documented diagnosis of pulmonary arterial hypertension (PAH), WHO Group 1 and ALL of the following:

      1. The individual’s diagnosis has been confirmed by right heart catheterization (medical records required) and ALL of the following:

         1. The individual's mean pulmonary arterial pressure (mPAP) is ≥20mmHg;

         2. The individual’s pulmonary capillary wedge pressure (PCWP) is <15 mmHg; AND

         3. The individual's pulmonary vascular resistance (PVR) is ≥3 Wood units;

      2. The requested agent is Adcirca (tadalafil), Adempas (riociguat), Flolan (epoprostenol), Letairis (ambrisentan), Opsumit (macitentan), Orenitram (treprostinil), Remodulin (treprostinil), Revatio (sildenafil), Tracleer (bosentan), Tyvaso (treprostinil), Uptravi (selexipag), Veletri (epoprostenol), or Ventavis (iloprost);

      3. The individual's WHO functional class is II or greater;

      4. ONE of the following:

         1. The requested agent will be utilized as monotherapy;

         2. The requested agent will be utilized as dual therapy that consists of an endothelin receptor antagonist (ERA) plus phosphodiesterase 5 inhibitor (PDE5i) as initial therapy;

         3. The requested agent will be utilized for add-on therapy to existing monotherapy (dual-therapy) [except combo requests for ERA plus PDEi for dual therapy] and BOTH of the following:

            1. The individual has unacceptable or deteriorating clinical status despite established PAH pharmacotherapy; AND

            2. The requested agent is in a different therapeutic class;

         4. The requested agent will be utilized for add-on therapy to existing dual therapy (triple therapy) and ALL of the following:

            1. The individual is WHO functional class III or IV;

            2. ONE of the following:

               1. A prostanoid has been started as one of the agents in the triple therapy; OR

               2. The individual has an intolerance, FDA-labeled contraindication, or hypersensitivity to ALL prostanoids;

            3. The individual has unacceptable or deteriorating clinical status despite established PAH pharmacotherapy; AND

            4. All three agents in the triple therapy are from a different therapeutic class; OR

         5. The requested agent will be utilized as part of triple therapy in a treatment naïve individual and BOTH of the following:

            1. The individual is WHO functional class IV; AND

            2. The triple therapy consists of an ERA, PDE5i, and a prostanoid; AND

      5. If the requested agent is Adempas (riociguat), the individual will not be using the requested agent in combination with a PDE5 inhibitor (e.g., tadalafil or sildenafil);

   2. The individual has a diagnosis of pulmonary hypertension associated with interstitial lung disease (PH-ILD), WHO Group 3 and ALL of the following:

      1. The requested agent is Tyvaso (treprostinil);

      2. The individual’s diagnosis has been confirmed by right heart catheterization (medical records required) and ALL of the following:

         1. The individual’s mean pulmonary arterial pressure (mPAP) is >20mmHg;

         2. The individual’s pulmonary capillary wedge pressure (PCWP) is <15 mmHg; AND

         3. The individual’s pulmonary vascular resistance (PVR) is >3 Wood units;

      3. The individual has an FVC <70% predicted;

      4. The individual has extensive parenchymal changes on computed tomography (CT); AND

      5. The individual is currently treated and will continue with standard of care therapy for ILD (e.g., Ofev); OR

   3. The individual has a diagnosis of chronic thromboembolic pulmonary hypertension (CEPH), WHO Group 4 and ALL of the following:

      1. The requested agent is Adempas (riociguat);

      2. The individual’s diagnosis has been confirmed by a ventilation-perfusion scan and a confirmatory selective pulmonary angiography;

      3. ALL of the following:

         1. The individual’s mean pulmonary arterial pressure (mPAP) is >20mmHg;

         2. The individual’s pulmonary capillary wedge pressure (PCWP) is <15 mmHg; AND

         3. The individual’s pulmonary vascular resistance (PVR) is >3 Wood units;

      4. ONE of the following:

         1. The individual is NOT a candidate for surgery; OR

         2. The individual has had a pulmonary endarterectomy and has persistent or recurrent disease; AND

      5. The individual will NOT be using the requested agent in combination with a PDE4 inhibitor (e.g., tadalafil or sildenafil);

2. The prescriber is a specialist or has consulted with a specialist in the area of the individual’s diagnosis (e.g., cardiologist, pulmonologist);

3. The individual does not have any FDA-labeled contraindications to the requested agent; AND

4. The dosage prescribed is within the program quantity limits based on approved FDA-labeled dosing.

 Length of Approval: 12 months

Renewal Criteria

The requested agent may be approved for RENEWAL when ALL of the following criteria are met:

1. The individual has been previously approved for therapy with the requested agent through BCBSMS PA process;

2. The individual has had clinical benefit with the requested agent (e.g., stabilization, decreased disease progression);

3. The prescriber is a specialist or has consulted with a specialist in the area of the individual’s diagnosis (e.g., cardiologist, pulmonologist);

4. The individual does not have any FDA-labeled contraindications to the requested agent; AND

5. The dosage prescribed is within the program quantity limits based on approved FDA-labeled dosing.

 Length of Approval: 12 months

 Winrevair (sotatercept-csrk) is considered not medically necessary as its place in therapy has not yet been addressed in clinical practice guidelines.

Liqrev (sildenafil oral suspension), Opsynvi (macitentan-tadalafil), Tadliq (tadalafil oral suspension), Tyvaso DPI (treprostinil inhalation powder), and Yutrepia (treprostinil inhalation powder) are considered not medically necessary as other formulary options exist.

POLICY EXCEPTIONS

State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A.  consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B.  appropriate with regard to standards of good medical practice; and

C.  not solely for the convenience of the Member, his or her Provider; and

D.  the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

POLICY HISTORY

10/15/2002: Approved by Pharmacy & Therapeutics (P & T) Committee.

10/27/2005: Description section updated. Policy section updated: changed preferred provider to Accredo. Phone # 1-800-235-8498 changed to 1-866-240-3373. Fax # changed from 1-888-355-6682 to 1-800-711-3526. Sources updated: Ventavis® and Revatio® added.

11/4/2005: Code Reference section updated, HCPCS codes Q4077, S0090 added; the drug Iloprost (Ventavis®) was added to the descriptor for code J3490.

11/2005: Approved by Pharmacy and Therapeutics (P&T) Committee.

2/6/2006: Code Reference table updated; codes J3285 and Q4080 added, deletion date added to codes Q4077 and S0114.

3/14/2006: Coding updated. HCPCS 2006 revisions added to policy.

3/22/2006: Policy reviewed, no changes.

7/16/2007: Ambrisentan (Letairis®) added to policy as medically necessary.

12/19/2007: Coding updated per 2008 CPT/HCPCS revisions.

01/01/2009: Accredo preferred provider information removed. BCBSMS information added.

8/10/2009: Tadalafil (Adcirca®) information added to the Policy Description section, Policy Statement section updated to include Tadalafil (Adcirca®) medically necessary information, and Source section updated to include Tadalafil (Adcirca®) Prescribing Information as a reference, Added Tadalafil (Adcirca®) to unclassified code J3490 under Covered table, Removed deleted HCPCS codes Q4077 and S0114 from Covered table. Added ICD-9 Diagnosis codes 416.1 and 416.9 to covered table.

 9/4/2009: Policy updated with the addition of  Treprostinil (Tyvaso®). Description section and policy statement section updated to include Treprostinil (Tyvaso®) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with NYHA Class III symptoms. Sources section updated with addition of Tracleer® and Tyvaso® Prescribing Information as sources. Code reference section updated with Tyvaso® added to HCPC code J3285

12/15/2009: Coding Section revised for 2010 CPT4 and HCPCS revisions.

02/28/2011: Added new HCPCS code J7686 to the Code Reference section.

03/18/2014: Policy updated to include Macitentan (Opsumit®) and Riociguat (Adempas®) in the policy description and policy statement. Removed deleted HCPCS code Q4080 from the Code Reference section.

09/02/2014: Policy description updated to include Orenitram™. Added the following policy statement: Treprostinil (Orenitram™) is considered medically necessary for the treatment of pulmonary arterial hypertension in patients with WHO Group I to improve exercise capacity. Sources section updated to add Orenitram™ Prescribing Information as a reference. Added (Orenitram™) to the description of HCPCS code J3285 in the Code Reference section.

08/31/2015: Medical policy revised to add ICD-10 codes. Code description updated for CPT code Q4074.

02/02/2016: Approved by Pharmacy & Therapeutics (P&T) Committee. Policy guidelines updated to add medically necessary definition.

05/31/2016: Policy number L.5.01.465 added.

06/30/2016: Policy description updated regarding Selexipag (Uptravi®). Added policy statement that Selexipag (Uptravi®) is considered medically necessary for the treatment of pulmonary arterial hypertension in WHO Group I symptoms. Sources section updated.

02/07/2017: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee.

09/29/2017: Code Reference section updated to add new ICD-10 diagnosis codes I27.20, I27.21, I27.22, I27.23, I27.24, and I27.29. Effective 10/01/2017.

02/06/2018: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee.

04/01/2018: Added statement to perform a formulary drug search to ensure the prescription drug is covered under the member's benefit plan. Policy description updated to remove FDA approved indications. Policy section updated to add initial and renewal approval criteria for the treatment of pulmonary arterial hypertension.

02/05/2019: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Policy section updated to add that prior authorization is required and that the use of samples by a Member will not be considered current or stable therapy for purposes of Medical Policy review. Sources section updated. Code Reference section updated to add HCPCS code J8499 and ICD-10 diagnosis codes I27.83 and P29.30. Removed deleted ICD-10 diagnosis code I27.2. Effective 01/01/2019.

07/23/2019: Policy Exceptions updated to add that for State Health Plan members, the drugs listed in this policy do not require prior authorization. However, they will be reviewed for medical necessity based on medical policy guidelines. Effective 04/01/2019.

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

01/20/2025: Policy updated to add Winrevair (sotatercept-csrk) as not medically necessary as its place in therapy has not yet been addressed in clinical practice guidelines. Sources updated.

06/03/2025: Policy reviewed by Pharmacy & Therapeutics (P&T) Committee; no changes.

08/01/2025: Effective 10/01/2025 - Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy revised to specify dosage forms of drugs and to add Liqrev (sildenafil) oral suspension, Opsynvi (macitentan-tadalafil) tablets, Tadliq (tadalafil) oral suspension, and Tyvaso DPI (treprostinil) inhalation powder to the list of drugs. Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Policy statement revised to remove list of drugs and state that the requested medication may be considered medically necessary when ALL of the listed criteria are met. Medically necessary criteria updated regarding individuals with a documented diagnosis of pulmonary arterial hypertension (PAH) WHO Group 1, individuals with a diagnosis of pulmonary hypertension associated with interstitial lung disease (PH-ILD), WHO Group 3, individuals with a diagnosis of chronic thromboembolic pulmonary hypertension (CEPH), WHO Group 4, prescriber requirements, and dose requirements. Renewal critera updated regarding clinical benefit, prescriber requirements, and dose requirements. Added statement that Liqrev (sildenafil oral suspension), Opsynvi (macitentan-tadalafil), Tadliq (tadalafil oral suspension), and Tyvaso DPI (treprostinil inhalation powder) are considered not medically necessary as other formulary options exist. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity." Sources updated. Policy update effective 10/01/2025.

11/01/2025: Policy updated to add Yutrepia (treprostinil) inhalation powder to the list of drugs. Policy statement revised to add Yutrepia (treprostinil inhalation powder) as not medically necessary as other formulary options exist. Sources updated.

SOURCE(S)

1. Adcirca prescribing information. United Therapeutics Corporation. November 2024. Last accessed April 2025.

2. Adempas prescribing information. Bayer HealthCare Pharmaceuticals Inc. January 2023. Last accessed April 2025.

3. Flolan prescribing information. GlaxoSmithKline LLC. October 2023. Last accessed April 2025.

4. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43:3618.

5. Letairis prescribing information. Gilead Sciences, Inc. February 2025. Last accessed April 2025.

6. Liqrev prescribing information. CMP Pharma, Inc. May 2024. Last accessed April 2025.

7. Opsumit prescribing information. Actelion Pharmaceuticals US, Inc. March 2025. Last accessed April 2025.

8. Opsynvi prescribing information. Actelion Pharmaceuticals US, Inc. April 2024. Last accessed April 2025.

9. Orenitram prescribing information. United Therapeutics Corporation. November 2024. Last accessed April 2025.

10. Revatio prescribing information. Pfizer Laboratories Div Pfizer Inc. January 2023. Last accessed April 2025.

11. Remodulin prescribing information. United Therapeutics Corporation. December 2024. Last accessed April 2025.

12. Tadliq prescribing information. CMP Pharma, Inc. October 2022. Last accessed April 2025.

13. Tracleer prescribing information. Actelion Pharmaceuticals US, Inc. February 2024. Last accessed April 2025.

14. Tyvaso prescribing information. United Therapeutics Corporation. December 2024. Last accessed April 2025.

15. Uptravi prescribing information. Actelion Pharmaceuticals US, Inc. July 2024. Last accessed April 2025.

16. Veletri prescribing information. Actelion Pharmaceuticals US, Inc. November 2022. Last accessed April 2025.

17. Ventavis prescribing information. Actelion Pharmaceuticals US, Inc. July 2022. Last accessed April 2025.

18. Winrevair prescribing information. Merck Sharp & Dohme LLC. March 2024. Last accessed April 2025.

19. Yutrepia prescribing information. Liquidia Technologies, Inc. September 2025. Last accessed October 2025.

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy. 

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Covered Codes

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