title: Clinical and prognostic value of endothelin-1 and big endothelin-1 expression in children with pulmonary hypertension.
abstract: Pulmonary arterial hypertension is known to be associated with increased expression of endothelin (ET)-1 and its precursor big ET-1. Therefore, we hypothesised that in children with pulmonary hypertension (PH) altered levels of ET-1 and big ET-1 may have clinical and prognostic impact.
  
  
  
  Sixty-six children with different forms of PH (mean age 10.4±9.7 years) were included. Blood samples were taken from the pulmonary artery and a systemic artery. Levels of ET-1/big ET-1 were measured via ELISA method and compared with clinical and haemodynamic data. To assess prognostic relevance, Kaplan-Meier survival analysis was conducted with definition of end point as the composite of mortality, lung transplantation, use of intravenous prostanoids and Potts shunt creation.
  
  
  
  ET-1 levels ranged between 0.09 and 11.64 (mean 1.48±2.34) fmol/mL, and big ET-1 levels between 0.05 and 2.92 (mean 0.84±0.58) fmol/mL. No significant relationships were found between ET-1/big ET-1 levels and functional class as well as haemodynamic indices of PH severity. Mean follow-up after catheterisation was 63.2±44.1 months. While 31 of the 66 (47%) patients with PH reached a predefined end point, there was no significant relation between levels of ET-1/big ET-1 and patient outcome.
  
  
  
  Although children with PH had alterations in ET-1/big ET-1 expression, which may reflect changes in net release or lung clearance, levels of ET-1/big ET-1 showed no correlation with clinical and haemodynamic parameters, and were not able to predict outcome.
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Heiner
authors.initials: H
authors.lastname: Latus
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Georg
authors.initials: G
authors.lastname: Karanatsios
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Basan
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Kirstin
authors.initials: K
authors.lastname: Salser
authors.affiliation: MUON-STAT Statistical Consulting, Stuttgart, Germany.
authors.forename: Simon
authors.initials: S
authors.lastname: Müller
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Khalil
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Joachim
authors.initials: J
authors.lastname: Kreuder
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
authors.affiliation: Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany Division of Pediatric Cardiology, University Children's Hospital Ulm, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Apitz
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000097:blood; D001800:Blood Specimen Collection / Q000379:methods; D002648:Child; D002675:Child, Preschool; D019332:Endothelin-1 / Q000097:blood*; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000503:physiopathology; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D015415:Biomarkers; D019332:Endothelin-1