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(Circulation. 2008;118:1115-1116.)
© 2008 American Heart Association, Inc.

Editors' Note

Pulmonary Vascular Diseases

Evangelos D. Michelakis, MD; Hunter C. Champion, MD, PhD

Series Editors, Pulmonary Vascular Diseases

An extract of the first 250 words of the full text is provided, because this article has no abstract.

The pulmonary circulation has fascinated researchers and clinicians for more than a century. Romberg first described the pathology of human pulmonary arterial hypertension (PAH) in 1891. In 1946, Euler and Liljestrand described the hypoxic pulmonary vasoconstriction: Whereas hypoxia dilates most systemic vascular beds, it constricts the pulmonary arteries. The physiological importance of hypoxic pulmonary vasoconstriction is now recognized as critical in the maintenance of adequate ventilation perfusion matching in all mammals, but the molecular identity of hypoxic pulmonary vasoconstriction remains unknown. The clinical syndrome of PAH was described by Dresdale in 1951, after the introduction of right heart catheterization allowed hemodynamic measurements in humans.

From 1960 to 1980, 3205 articles were published (PubMed key word pulmonary hypertension); from 1980 to 2000, the number jumped to 11 127. From 2000 until now, the number of articles has reached 9294. Despite some progress, the cause and molecular basis of PAH remains unknown. As can be seen by the number of publications, the interest of the research and clinical communities has been rapidly increasing. With the introduction of potential therapies over the past 20 years, the interest of the public, as well as that of the industry, has also been increasing. After the initial enthusiasm for the currently approved PAH therapies (parenteral prostacyclin analogues, oral endothelin-receptor antagonists, and oral phosphodiesterase type 5 inhibitors), it was realized that although these therapies alleviate symptoms in many patients, the oral therapies and possibly others do not have a significant impact on the disease in that . . . [Full Text of this Article]