Subject: Specialty Quantity Limit PAH 1771-H P2025

Drug

Pulmonary Arterial Hypertension (PAH)

ADCIRCA  (tadalafil)

ADEMPAS  (riociguat)

ALYQ  (tadalafil)

LETAIRIS  (ambrisentan)

LIQREV  (sildenafil)

OPSUMIT  (macitentan)

OPSYNVI  (macitentan)

REVATIO  (sildenafil)

TADLIQ  (tadalafil)

TRACLEER  (bosentan)

TYAVSO  (treprostinil)

UPTRAVI  (selexipag)

VENTAVIS  (iloprost)

WINREVAIR  (sotatercept-csrk)

Policy:

Program Description

The standard limit is designed to allow a quantity sufficient for the most common uses of the medication. The recommended dosing parameters for all FDA indications fall within the standard limits. Coverage of an additional quantity may be reviewed on a case-by-case basis upon request.

Covered Quantities

Medication	Standard Limit
Adcirca (tadalafil) 20 mg tablets	60 tablets per 30 days
Adempas (riociguat) 0.5 mg tablets	90 tablets per 30 days
Adempas (riociguat) 1 mg tablets	90 tablets per 30 days
Adempas (riociguat) 1.5 mg tablets	90 tablets per 30 days
Adempas (riociguat) 2 mg tablets	90 tablets per 30 days
Adempas (riociguat) 2.5 mg tablets	90 tablets per 30 days
Alyq (tadalafil) 20 mg tablets	60 tablets per 30 days
Letairis (ambrisentan) 5 mg tablets	30 tablets per 30 days
Letairis (ambrisentan) 10 mg tablets	30 tablets per 30 days
Liqrev (sildenafil) 10 mg/mL oral suspension	732 mL per 30 days
Opsumit (macitentan) 10 mg tablets	30 tablets per 30 days
Opsynvi (macitentan/tadalafil) 10 mg/20 mg tablets	30 tablets per 30 days
Opsynvi (macitentan/tadalafil) 10 mg/40 mg tablets	30 tablets per 30 days
Revatio (sildenafil) 10 mg/mL oral suspension	784 mL per 30 days
Revatio (sildenafil) 20 mg tablets	360 tablets per 30 days
Revatio (sildenafil) 10 mg/12.5 mL single-dose vial	90 vials per 30 days
Tadliq (tadalafil) 20 mg/5 mL oral suspension	300 mL per 30 days
Tracleer (bosentan) 62.5 mg tablets	60 tablets per 30 days
Tracleer (bosentan) 125 mg tablets	60 tablets per 30 days
Tracleer (bosentan) 32 mg tablets for oral suspension	112 tablets per 28 days
Tyvaso (treprostinil) 0.6 mg/mL inhalation solution	81.2 mL (28 ampules) per 28 days
Tyvaso (treprostinil) DPI 16 mcg and 32 mcg   Inhalation Powder Titration Kit	1 kit (196 cartridges)  per 28 days
Tyvaso (treprostinil) DPI 16 mcg, 32 mcg, and 48 mcg   Inhalation Powder Titration Kit	1 kit (252 cartridges) per 28 days
Tyvaso (treprostinil) DPI 16 mcg Inhalation Powder Maintenance Kit	1 kit (112 cartridges) per 28 days
Tyvaso (treprostinil) DPI 32 mcg Inhalation Powder Maintenance Kit	1 kit (112 cartridges) per 28 days
Tyvaso (treprostinil) DPI 48 mcg Inhalation Powder Maintenance Kit	1 kit (112 cartridges) per 28 days
Tyvaso (treprostinil) DPI 64 mcg Inhalation Powder Maintenance Kit	1 kit (112 cartridges) per 28 days
Tyvaso (treprostinil) DPI  32 mcg and 48 mcg   Inhalation Powder Maintenance Kit	1 kit (224 cartridges) per 28 days
Uptravi (selexipag) 200 mcg tablets	140 tablets per 28 days
Uptravi (selexipag) 400 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) 600 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) 800 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) 1000 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) 1200 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) 1400 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) 1600 mcg tablets	60 tablets per 30 days
Uptravi (selexipag) Titration Pack (200 mcg/800 mcg tablets)	1 pack (140 of 200 mcg tablets, 60 of 800 mcg tablets) per 28 days
Uptravi (selexipag) 1800 mcg single-dose vial	60 vials per 30 days
Ventavis (iloprost) 10 mcg/mL inhalation solution	270 ampules per 30 days
Ventavis (iloprost) 20 mcg/mL inhalation solution	270 ampules per 30 days
Winrevair (sotatercept-csrk) 45 mg kit (one 45 mg single-dose vial)	1 kit (one vial) per 21 days
Winrevair (sotatercept-csrk) 60 mg kit (one 60 mg single-dose vial)	1 kit (one vial) per 21 days
Winrevair (sotatercept-csrk) 90 mg kit (two 45 mg single-dose vials)	1 kit (two vials) per 21 days
Winrevair (sotatercept-csrk) 120 mg kit (two 60 mg single-dose vials)	1 kit (two vials) per 21 days

FDA-Recommended Dosing

Adcirca, Alyq

40 mg per day

Adempas

The recommended starting dosage is 1 mg taken 3 times a day.

For patients who may not tolerate the hypotensive effect of Adempas, consider a starting dose of 0.5 mg taken three times a day. If systolic blood pressure remains greater than 95 mmHg and the patient has no signs or symptoms of hypotension, up-titrate by 0.5 mg taken three times a day. Dose increases should be no sooner than 2 weeks apart. The dose can be increased to the highest tolerated dosage, up to a maximum of 2.5 mg taken 3 times per day. If at any time, the patient has symptoms of hypotension, decrease the dosage by 0.5 mg taken three times per day.

Letairis

Initiate treatment at 5 mg once daily with or without tadalafil 20 mg once daily. At 4-week intervals, either the dose of Letairis or tadalafil can be increased, as needed and tolerated, to Letairis 10 mg or tadalafil 40 mg.

Liqrev

20 mg three times a day; may titrate to a maximum dose of 80 mg three times a day

Opsumit

10 mg once daily

Opsynvi

Treatment-naïve to any PAH specific therapy or transitioning from an endothelin receptor antagonist (ERA) monotherapy: Initiate treatment at one 10 mg/20 mg tablet once daily for one week. If tolerated, up titrate to one 10 mg/40 mg tablet once daily.

Transitioning from PDE5 inhibitor monotherapy or PDE5 inhibitor and ERA therapy in combination: One 10 mg/40 mg tablet once daily.

Revatio

Patients greater than or equal to 18 years of age

Oral dose of 20 mg three times a day; may titrate to a maximum dose of 80 mg three times a day. Intravenous dose of 10 mg administered as an intravenous bolus injection three times a day. A 10 mg intravenous dose is predicted to provide pharmacological effect equivalent to that of a 20 mg oral dose.

Patients 1 to 17 years of age

• Less than or equal to 20 kg: 10 mg three times a day
• Greater than 20 kg to less than 45 kg: 20 mg three times a day
• Greater than or equal to 45 kg: 20 mg three times a day; may be titrated to a maximum dose of 40 mg three times a day

Tadliq

40 mg (10 mL) per day

Tracleer

Patients greater than 12 years of age

• Greater than 40 kg: Initiate treatment at 62.5 mg twice daily for 4 weeks and then increase to maintenance dose of 125 mg twice daily
• Less than 40 kg: Initial and maintenance dosing: 62.5 mg twice daily

Patients less than or equal to 12 years of age

Initial and maintenance dosing:

• Greater than or equal to 4-8 kg: 16 mg twice daily
• Greater than 8-16 kg: 32 mg twice daily
• Greater than 16-24 kg: 48 mg twice daily
• Greater than 24-40 kg: 64 mg twice daily

Tyavso

Inhalation Solution

Initial dosage: Three breaths of Tyvaso (18 mcg of treprostinil) per treatment session, four times daily. If three breaths are not tolerated, reduce to one or two breaths and subsequently increase to three breaths, as tolerated.

Dosage should be increased by an additional three breaths per treatment session, four times daily at approximately 1- to 2- week intervals. Studies establishing effectiveness have used target doses of nine to twelve breaths per treatment session, four times daily (up to 72 mcg four times a day).

Inhalation Powder

Initial dosage: One 16 mcg cartridge per treatment session, four times daily.

Dosage should be increased by an additional 16 mcg per treatment session at approximately 1- to 2- week intervals, if tolerated.

Titrate to target maintenance doses of 48 mcg to 64 mcg per treatment sessions, four times daily.

If the prescribed dose is higher than 64 mcg per treatment session, more than 1 cartridge will be needed per session.

Uptravi

Starting dose: 200 mcg twice daily. Increase the dose by 200 mcg twice daily at weekly intervals to the highest tolerated dose up to 1600 mcg twice daily.

Use injection in patients who are temporarily unable to take oral therapy. Administer twice daily by intravenous infusion at a dose that corresponds to the patient's current dose of tablets.

Ventavis

5 mcg up to nine times per day. The 20 mcg/mL concentration is intended for patients who are maintained at the 5 mcg dose and who have repeatedly experienced extended treatment times which could result in incomplete dosing.

Winrevair

Administer once every 3 weeks. Start dosing at 0.3 mg/kg. Increase to the target dose of 0.7 mg/kg. Continue treatment at 0.7 mg/kg every 3 weeks unless dosage adjustments are required.

Place of Service:

Outpatient

The above policy is based on the following references:

1. Adcirca [package insert]. Indianapolis, IN: Eli Lilly and Company; September 2020.
2. Adempas [package insert]. Whippany, NJ: Bayer HealthCare Pharmaceuticals Inc.; January 2023.
3. Alyq [package insert]. Parsippany, NJ: Teva Pharmaceuticals; April 2023.
4. Ambrisentan [package insert]. Cranbury, NJ: Sun Pharmaceutical Industries, Inc.; January 2024.
5. Bosentan [package insert]. Pennington, NJ: Zydus Pharmaceuticals (USA) Inc. February 2024.
6. Letairis [package insert]. Foster City, CA: Gilead Sciences, Inc.; August 2019.
7. Liqrev [package insert]. Farmville, NC: CMP Pharma, Inc.; April 2023.
8. Opsumit [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; April 2025.
9. Opsynvi [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; April 2025.
10. Revatio [package insert]. Morgantown, WV: Viatris Specialty LLC; December 2024.
11. Sildenafil [package insert]. Piscataway, NJ: Camber Pharmaceuticals, Inc.; February 2024.
12. Sildenafil for oral suspension [package insert]. East Windsor, NJ: Aurobindo Pharma USA, Inc.; January 2024.
13. Tadalafil [package insert]. Bridgewater, NJ: Ajanta Pharma USA Inc.; May 2023.
14. Tadliq [package insert]. Farmville, NC: CMP Pharma, Inc.; October 2023.
15. Tracleer [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; February 2024.
16. Tyvaso [package insert]. Research Triangle Park, NC: United Therapeutics Corp.; May 2022.
17. Tyvaso DPI [package insert]. Research Triangle Park, NC: United Therapeutics Corp.; October 2024.
18. Uptravi [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; July 2022.
19. Ventavis [package insert]. Titusville, NJ: Actelion Pharmaceuticals US, Inc.; March 2022.
20. Winrevair [package insert]. Rahway, NJ: Merck Sharp & Dohme LLC; March 2024.

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

October 12, 2025