FDA Approved PH Treatments

FDA-Approved Treatments for Pulmonary Hypertension

There are several FDA-approved medications used to treat pulmonary hypertension (PH). The right treatment depends on the type of PH you have, the severity of your disease, your symptoms, and how you respond to treatment.

Some medications are specifically approved for pulmonary arterial hypertension (PAH), Group 1 PH, while others are approved for specific forms of PH, including chronic thromboembolic pulmonary hypertension (CTEPH) and PH associated with interstitial lung disease (PH-ILD).

Treatments for Pulmonary Arterial Hypertension (PAH)

Endothelin Receptor Antagonists (ERAs)

These medicines block the effects of a substance called endothelin that can cause the blood vessels in the lungs to narrow.

  • Ambrisentan (Letairis®) - oral medication

  • Bosentan (Tracleer®) - oral medication

  • Macitentan (Opsumit®) - oral medication

Phosphodiesterase-5 (PDE5) Inhibitors

These medicines help relax the blood vessels in the lungs and improve blood flow.

  • Sildenafil (Revatio®) - oral medication

  • Tadalafil (Adcirca®, Alyq®) - oral medication

Soluble Guanylate Cyclase (sGC) Stimulator

This type of medicine helps the blood vessels in the lungs relax through the nitric oxide pathway.

  • Riociguat (Adempas®) - oral medication

Riociguat is FDA-approved for both PAH (Group 1) and certain patients with chronic thromboembolic pulmonary hypertension (CTEPH, Group 4).

Prostacyclin-Based Therapies

These medicines work through the prostacyclin pathway to help relax and widen the blood vessels in the lungs.

  • Epoprostenol (Veletri®) - continuous intravenous (IV) infusion

  • Treprostinil (Remodulin®) - continuous IV or subcutaneous infusion

  • Treprostinil (Orenitram®) - oral medication

  • Treprostinil (Tyvaso®) - inhaled medication

  • Treprostinil (Tyvaso DPI®) - inhaled dry powder

  • Treprostinil (Yutrepia®) - inhaled dry powder

  • Iloprost (Ventavis®) - inhaled medication

  • Selexipag (Uptravi®) - oral medication or IV formulation

Treprostinil is available in several FDA-approved formulations and routes of administration. Tyvaso, Tyvaso DPI, and Yutrepia are also FDA-approved for PH associated with interstitial lung disease (PH-ILD, Group 3).

Activin Signaling Inhibitor

This newer type of treatment targets a different pathway involved in the development and progression of PAH.

  • Sotatercept-csrk (Winrevair®) - subcutaneous injection

Winrevair is FDA-approved for adults with PAH (Group 1). It is generally used as part of an overall treatment plan and may be used along with other PAH therapies.

Combination Therapy

  • Macitentan/tadalafil (Opsynvi®) - a single oral tablet that combines an endothelin receptor antagonist (macitentan) with a PDE5 inhibitor (tadalafil).

Opsynvi is FDA-approved for the chronic treatment of adults with PAH (Group 1), WHO Functional Class II–III.

FDA-Approved Treatments for Other Forms of PH

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

  • Riociguat (Adempas®) - FDA-approved for adults with persistent or recurrent CTEPH after surgical treatment or adults with inoperable CTEPH.

Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

  • Treprostinil (Tyvaso®)- inhaled medication

  • Treprostinil (Tyvaso DPI®) - inhaled dry powder

  • Treprostinil (Yutrepia®) - inhaled dry powder

These medications are FDA-approved to improve exercise ability in adults with PH associated with interstitial lung disease (PH-ILD, Group 3).

Supportive Treatments

In addition to PH-targeted medications, your healthcare team may recommend other treatments to help manage symptoms, improve quality of life, or treat related health problems.

These may include:

  • Diuretics (“water pills”) - help remove excess fluid and reduce swelling.

  • Oxygen therapy - may be recommended for people with low blood oxygen levels.

  • Anticoagulants (blood thinners) - may be recommended for certain patients, depending on their condition and risk of blood clots.

  • Calcium channel blockers - may be appropriate for a small, carefully selected group of PAH patients who have a positive response to an acute vasodilator challenge.

  • Treatment of underlying conditions - particularly important when PH is caused by heart disease, lung disease, blood clots, or another medical condition.

These supportive treatments are not the same as PH-targeted medications, and not every person with PH needs them. Your healthcare team will determine which treatments are appropriate for you.

A Note About PH Treatment

There is no single treatment that is right for everyone with pulmonary hypertension. Some people take one medication, while others may need two or more PH-targeted therapies that work in different ways. Treatment may also change over time as your disease or response to therapy changes.

Because PH medications can have important side effects and interactions, treatment should be prescribed and monitored by a healthcare professional experienced in pulmonary hypertension.