Pulmonary arterial hypertension

What is the long-term outlook for people with pulmonary arterial hypertension?

The average survival of a person with pulmonary arterial hypertension (PAH) after diagnosis is estimated to be around 2.8 years, however individual prognosis may be better or worse depending on a variety of factors including age and severity of PAH. Studies have demonstrated survivals ranging from less than 1 year to over 7 years. Current treatments are helpful in controlling/managing symptoms experienced by people with PAH, but unfortunately no treatment has significantly improved survival.

Last updated on 05-01-20

How might pulmonary arterial hypertension be treated?

People with pulmonary arterial hypertension (PAH) benefit from receiving treatment at specialized centers. The Pulmonary Hypertension Association offers a Find a Doctor tool which may aid you in locating your nearest center.

Treatment of serious or life threatening PAH may involve continuous IV epoprostenol. Other treatment options, include treprostinil, iloprost, bosentan, ambrisentan, sildenafil, and tadalafil. Many of these treatments can be administered in various forms, such as by shot, IV, or inhalation. A small number of people with PAH respond well to long term oral calcium channel blockers. Blood thinners, diuretics, and supplemental oxygen may be prescribed as needed.

Many drugs can be harmful to people with PAH. The following should be avoided: appetite suppressants, cocaine, amphetamines (and related compounds), low oxygen environments (such as high altitudes), and possibly estrogen compounds (oral contraceptives and hormone replacement therapy).

Last updated on 05-01-20

Financial Resources

Patient Services, Inc (PSI)

Patient Services, Inc (PSI) provides financial support and guidance for qualified patients with specific chronic diseases. PSI helps patients find solutions to the social and economic problems confronting patients with chronic conditions.

Last updated on 04-27-20

Press Releases

FDA News - idiopathic pulmonary hypertension

The U.S. Food and Drug Administration (FDA) approved a new orphan drug called Letairis for the treatment of pulmonary arterial hypertension. To read the news release regarding this drug, click on the link.

Last updated on 04-27-20

Where To Start

American Lung Association

The American Lung Association has an information page on pulmonary hypertension. To access this information, click on the link above.

Last updated on 04-27-20

Name: American Heart Association 7272 Greenville Avenue
Dallas, TX, 75231-4596, United States
Phone: 214-570-5978 Toll Free: 800-242-8721 Email: https://www.heart.org/en/forms/general-questions-and-latest-research-information Url: https://www.heart.org
Name: Pulmonary Hypertension Association 801 Roeder Road, Suite 1000
Silver Spring, MD, 20910, United States
Phone: 301-565-3004 Fax : 301-565-3994 Email: PHA@PHAssociation.org Url: https://www.phassociation.org/
Name: Phaware Global Association phaware 1227 N. Avon St.
Burbank, CA, 91505,
Phone: 844-PHAWARE (844-742-9273) Email: info@phaware.global Url: http://www.phaware.global/

Note, these links are external searches against the National Laboratory of Medicine's drug database. You may need to adjust the search if there are no results found.

Drug Name Generic Name
Letairis® Ambrisentan
Remodulin;Orenitram treprostinil
Tracleer bosentan
Adempas riociguat
Flolan® Epoprostenol
Opsumit macitentan
Uptrav Selexipag
Ventavis Iloprost
Tyvaso treprostinil (inhalational)
Adcirca tadalafil

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