Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension.
OBJECTIVE
Primary pulmonary hypertension (PPH) is a rare disease of unknown etiology that is characterized by a poor prognosis. This study was undertaken to investigate possible correlations between endothelin (ET)-1 and big ET-1 plasma levels and the severity of PPH.


METHODS
Sixteen consecutive patients with PPH were included.


METHODS
Hemodynamics of patients with PPH were measured by right-heart catheterization, and a 6-min walk test was performed.


METHODS
Plasma levels of the biologically active peptide ET-1 and its precursor big ET-1 were determined in blood samples from the pulmonary artery, peripheral artery, and peripheral vein by radioimmunoassay.


RESULTS
A strong correlation was shown between pulmonary vascular resistance, mean pulmonary artery pressure, cardiac output, cardiac index, 6-min walk data, and elevated plasma levels of big ET-1 as well as mature ET-1 plasma levels at all sites of blood sampling (p < 0.01 and p < 0.05, respectively).


CONCLUSIONS
Levels of circulating ET-1 might become a prognostic marker for patients with PPH and serve as a tool for the selection of patients who may benefit from treatment with ET-receptor antagonists.
Rubens
C
C
Department of Cardiology and Pulmonology, Benjamin Franklin Hospital, Free University of Berlin, Berlin, Germany. ChristophRubens@aol.com
Ewert
R
R
Halank
M
M
Wensel
R
R
Orzechowski
H D
HD
Schultheiss
H P
HP
Hoeffken
G
G
D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D001794:Blood Pressure; D002302:Cardiac Output; D019332:Endothelin-1 / Q000097:blood*; D016232:Endothelins / Q000097:blood*; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000175:diagnosis* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011498:Protein Precursors / Q000097:blood*; D011651:Pulmonary Artery / Q000503:physiopathology; D014655:Vascular Resistance
D015415:Biomarkers; D019332:Endothelin-1; D016232:Endothelins; D011498:Protein Precursors