Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension

Chest. 2001 Nov;120(5):1562-9. doi: 10.1378/chest.120.5.1562.


Study objectives: 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.

Patients: Sixteen consecutive patients with PPH were included.

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

Measurements: 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.

MeSH terms

  • Adult
  • Aged
  • Biomarkers / blood
  • Blood Pressure
  • Cardiac Output
  • Endothelin-1 / blood*
  • Endothelins / blood*
  • Female
  • Hemodynamics
  • Humans
  • Hypertension, Pulmonary / blood
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / physiopathology
  • Male
  • Middle Aged
  • Prognosis
  • Protein Precursors / blood*
  • Pulmonary Artery / physiopathology
  • Vascular Resistance


  • Biomarkers
  • Endothelin-1
  • Endothelins
  • Protein Precursors