title: Pulmonary blood pressure, not flow, is associated with net endothelin-1 production in the lungs of patients with congenital heart disease and normal pulmonary vascular resistance.
abstract: OBJECTIVE
  Endothelin-1 concentrations are increased in patients with increased mean pulmonary arterial pressure, pulmonary blood flow, and pulmonary vascular resistance. However, endothelin-1 concentrations have not been well characterized in patients with congenital heart disease and normal pulmonary vascular resistance. In particular, it is unclear whether pressure or flow is the key regulator of endothelin- 1 in this setting. We tested the hypothesis that pulmonary blood pressure and not flow is associated with net endothelin-1 production in patients with congenital heart disease and normal pulmonary vascular resistance.
  
  
  METHODS
  With a commercially available immunoassay, we measured endothelin-1 concentrations in pulmonary arterial and pulmonary venous plasma of 56 consecutive patients with congenital heart disease and pulmonary vascular resistance less than 2 U. m(2) undergoing cardiac catheterization. We used multiple linear regression to analyze the effect of demographic and hemodynamic variables on pulmonary arterial and venous endothelin-1 concentrations and on the change of endothelin-1 concentration over the pulmonary vascular bed.
  
  
  RESULTS
  Multiple linear regression revealed that of all the hemodynamic variables tested, mean pulmonary arterial pressure had the greatest effect on increasing the change of endothelin-1 concentration over the pulmonary vascular bed (P <.0001). Pulmonary blood flow did not have any effect on endothelin-1 concentrations or on the change of endothelin-1 concentration over the pulmonary vascular bed.
  
  
  CONCLUSIONS
  This study shows that pulmonary blood pressure and not flow is associated with net endothelin-1 production in patients with congenital heart disease and normal pulmonary vascular resistance.
authors.affiliation: Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Klinik an der Technische Universität München, Germany. fratz@dhm.mhn.de
authors.forename: Sohrab
authors.initials: S
authors.lastname: Fratz
authors.forename: Ralf
authors.initials: R
authors.lastname: Geiger
authors.forename: Hans
authors.initials: H
authors.lastname: Kresse
authors.forename: Gabriele
authors.initials: G
authors.lastname: Roemer
authors.forename: Michael
authors.initials: M
authors.lastname: Hennig
authors.forename: Walter
authors.initials: W
authors.lastname: Sebening
authors.forename: John
authors.initials: J
authors.lastname: Hess
mesh_terms: D001794:Blood Pressure; D001830:Body Surface Area; D002648:Child; D019332:Endothelin-1 / Q000096:biosynthesis* / Q000097:blood; D005260:Female; D006330:Heart Defects, Congenital / Q000378:metabolism / Q000503:physiopathology*; D006801:Humans; D008168:Lung / Q000378:metabolism*; D008297:Male; D011652:Pulmonary Circulation; D012044:Regression Analysis; D014655:Vascular Resistance
chemical_list: D019332:Endothelin-1