Medical Management and Treatment of Pulmonary Arterial Hypertension

The treatment and management of pulmonary hypertension (PH) depends on the specific kind a person has.

Pulmonary arterial hypertension (PAH) can be:

Idiopathic
Familial
Drug and toxin-induced PAH
Related to other diseases, including:

Scleroderma
HIV
Portal hypertension
Congenital heart disease

In the last 25 years, the medical community has made remarkable progress in treating these forms of PH. They often respond to one form of therapy or a combination of therapies.

Our experts at the UPMC Comprehensive Pulmonary Hypertension Program work with each person to prescribe the right treatment or management method. This helps improve daily function and prolong life.

FDA-approved Medicines for Pulmonary Arterial Hypertension

There are currently 12 FDA-approved drugs for PAH.

Delivery methods of medicines include:

Oral
Through a vein (intravenous)
Under the skin (subcutaneous)
Inhaled

Therapy	Description	Medicines
Phosphodiesterase-5 (PDE-5) inhibitors	Pills that prevent the breakdown of the lungs’ own natural substance that causes the blood vessels to relax.	Oral: Tadalafil (Adcirca^®) Sildenafil (Revatio^®)
Soluble guanylate cyclase (sGC) stimulator	Pills that work in the same pathway as PDE-5 inhibitors, but cause cells in the pulmonary arteries to produce more cyclic GMP — the substance that causes the arteries to relax.	Oral: Riociguat (Adempas^®)
Endothelin receptor blockers (ERBs)	Pills that inhibit a substance made by the body (endothelin) that causes blood vessels to narrow from binding to the muscular layer in pulmonary arteries. This in turn leads to relaxation of the pulmonary arteries.	Oral: Bosentan (Tracleer^®) Ambrisentan (Letaris^®) Macitentan (Opsumit^®)
Prostacyclins	Medicines for more advanced PAH that must be given by continuous infusion. Prostaglandin is a steroid that the body produces naturally. It causes blood vessels in the lungs to relax and allows blood to flow through them easily. Prostacyclins are man-made substances that act like prostaglandin and cause the blood vessels become less narrow. Medicines that are infused subcutaneously or through a catheter intravenously come with a portable pump that patients: Wear on their belts or underneath clothing. Uses 24 hours per day, 7 days per week. Learn how to maintain pumps and catheters to live independently. Medicines that require inhalation come with a specific inhaler device that patients use for treatment between four and six times per day.	Intravenous: Epoprostenol (Flolan^®) Room-temperature stable epoprostenol (Veletri^®) Intravenous or subcutaneous: Treprostinil (Remodulin^®) Inhaled: Treprostinil (Tyvaso^®) Iloprost (Ventavis^®) Oral: Treprostinil (Orenitram™)

Other Medical Treatments for Pulmonary Arterial Hypertension

Other medical therapies for PAH may include:

Oxygen — inhaled by patients via a nasal cannula or face mask.
Diuretics — medicine that rids the body of excess fluid that puts pressure on the heart.
Calcium channel blockers (CCB) — medicine that helps to decrease blood pressure.
Warfarin (Coumadin) — medicine that “thins” blood and prevents it from clotting.

Ongoing Treatment Research for Pulmonary Hypertension

Current research has focused on combining existing and newer therapies to improve outcomes.

In our program, we are always at the cutting edge of new medical therapies for PH, either directly through clinical trials or through close communication with PH drug developers.

Make an Appointment for PH Treatment Today

To schedule an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at:

412-648-6161
1-877-PH4-UPMC, toll-free
PHprogram@upmc.edu

Learn More About Treating and Managing Pulmonary Hypertension

Links below open a new window.

From our HealthBeat blog:

UPMC Patient Education Materials:

Blood Clot Prevention: Coumadin

From our Health Library:

Pulmonary Hypertension