Pulmonary Hypertension

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Pulmonary Hypertension Week at the ATS

Pulmonary Hypertension Week at the ATS

Welcome to the Pulmonary Hypertension Week at the ATS!

Pulmonary arterial hypertension is an uncontrolled increase in the blood pressure of the pulmonary vessels that feed the lungs. It is caused by an unexplained remodeling and obstruction of the small arterial vessels that carry blood from the right ventricle into the lungs for the purpose of oxygenation and removal of carbon dioxide. The natural cause of the disease is a progressive worsening of the resistance to flow into the lungs leading ultimately to heart failure and death, typically within 3 to 4 years after the first symptoms if the patient remains untreated. This syndrome was first described in 1891 by a German physician specialized in the field of circulation in organs, Dr. Ernst von Romberg. Pulmonary hypertension affects millions of people around the world since some of the causes of the disease include parasitic infection (such as schistosomiasis) and hereditary problems affecting the red cells (such as sickle cell disease), which are quite common throughout the world.

We have learned much about the physiology of the disease after catheterization of the heart was made possible in the mid-1970’s, and we have gained more knowledge about the different conditions that can cause pulmonary hypertension after the NIH started a registry of patients afflicted with this syndrome. This led to a growing interest in the disease, several world conferences on pulmonary hypertension (held every 5 years and initially started under the umbrella of the World Health Organization), development of the first efficacious therapy for PAH in the mid-1990 with the use of a drug (prostacyclin) given intravenously by a pump, and the creation of a very dynamic patient support group, the Pulmonary Hypertension Association (PHA).

Over the past twenty years, there has been extensive research (with support from drug companies, the NIH, and the PHA) focusing on the biology of the cells that grow out of control leading to obstruction of the pulmonary vessels and pulmonary hypertension. This has led eventually to the current use of over 7 different FDA-approved drugs that can be given orally, by inhalation or intravenously. Although our PAH patients have significantly improved with these drugs and now live much longer (compared to just a few years when therapy was not available), PAH remains unfortunately a deadly disease with no definitive cure. More recently, basic and clinical research has focused on finding drugs that reverse the remodeling of the vessels, and we are confident that a definite cure may be around the corner.

Please join the American Thoracic Society and the Pulmonary Hypertension Association in recognizing this week at Pulmonary Hypertension Week at the ATS. 


Paul M. Hassoun, MD
ATS Pulmonary Circulation Assembly
American Thoracic Society


Rino Aldrighetti
President and CEO
Pulmonary Hypertension Association
Member, ATS Public Advisory Roundtable

ATS Pulmonary Hypertension Partner
Pulmonary Hypertension Association


The mission of the Pulmonary Hypertension Association (PAHA) is to find ways to prevent and cure pulmonary hypertension, and to provide hope for the pulmonary hypertension community through support, education, advocacy and awareness.

The PAHA is a young organization, changing the history of an illness. From simple beginnings - four women around a kitchen table in Florida in 1990 - PAHA has evolved in size and complexity. Membership now includes over 13,000 patients and family members as well as two medical membership sections, one for physicians and researchers, the other for allied health professionals. The synergy created from the partnership of these constituencies has resulted in a community of hope and effective change.

Disclaimer: "The ATS Lung Disease Week Web site is designed for educational purposes only. You should not rely on this information as a substitute for personal medical attention, a diagnosis from a physician, or direct medical care.  If you are concerned about your health or that of a family member, please consult your family's healthcare provider immediately. Do not wait for a response from our members, staff or partners."