title: Relation of elevated plasma endothelin in congenital heart disease to increased pulmonary blood flow.
abstract: Endothelin-1 (ET), a potent vasoconstrictor peptide, has been found to be elevated in children with pulmonary hypertension associated with congenital heart defects. To evaluate the effect of pulmonary blood flow on ET concentrations, 5 ml blood samples were obtained peripherally at cardiac catheterization from 35 patients, ages 0.13 to 17 years (median 2). Plasma was extracted and ET measured by radioimmunoassay. Patients were classified into 2 groups based on the presence (group A) or absence (group B) of increased pulmonary blood flow defined as a Qp/Qs > or = 1.5. When the 13 patients (37%) in group A were compared with the 22 patients (63%) in group B there were no significant differences in age, cardiac index, or pulmonary and systemic resistances. ET concentrations were significantly higher in group A patients (median 3.25, range 0 to 16.5 vs median 0, range 0 to 6.35 pg/ml; p < or = 0.05). Pulmonary blood flow and pulmonary artery pressure were also higher in group A patients (p < or = 0.01). When patients within group A were subdivided into those with and without pulmonary hypertension, no difference was present in their ET concentrations (mean/SD: 4.4/4.3 vs 4.0/6.4 pg/ml, p = NS). Thus, ET is elevated in patients with congenital heart disease associated with left-to-right shunts and it appears that this increase is related to increased pulmonary blood flow independent of pulmonary artery pressure.
authors: Division of Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit 48201.
authors: J A
authors: JA
authors: Vincent
authors: R D
authors: RD
authors: Ross
authors: J
authors: J
authors: Kassab
authors: J M
authors: JM
authors: Hsu
authors: W W
authors: WW
authors: Pinsky
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016232:Endothelins / Q000097:blood*; D005260:Female; D006330:Heart Defects, Congenital / Q000097:blood* / Q000503:physiopathology; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood; D007223:Infant; D008297:Male; D011652:Pulmonary Circulation / Q000502:physiology*
chemical_list: D016232:Endothelins