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Published on: May 11, 2015
Relation of elevated plasma endothelin in congenital heart disease to increased pulmonary blood flow
J A Vincent1, R D Ross, J Kassab
1Division of Cardiology, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit 48201.
Insights
Endothelin-1 (ET) is elevated in children with congenital heart defects and increased pulmonary blood flow. This study found higher ET levels in patients with left-to-right shunts, linked to greater pulmonary blood flow, not pulmonary artery pressure.
Area of Science:
- Cardiology
- Pediatrics
- Endocrinology
Background:
- Endothelin-1 (ET) is a potent vasoconstrictor.
- ET levels are elevated in children with pulmonary hypertension and congenital heart defects.
- The relationship between pulmonary blood flow and ET in this population requires further investigation.
Purpose of the Study:
- To evaluate the effect of pulmonary blood flow on ET concentrations in children with congenital heart defects.
- To determine if elevated ET is associated with increased pulmonary blood flow or pulmonary hypertension.
Main Methods:
- Collected peripheral blood samples from 35 pediatric patients undergoing cardiac catheterization.
- Measured plasma ET concentrations using radioimmunoassay.
- Classified patients into groups based on pulmonary blood flow (Qp/Qs ≥ 1.5) and analyzed ET levels in relation to flow and pressure.
Main Results:
- ET concentrations were significantly higher in patients with increased pulmonary blood flow (Group A) compared to those without (Group B).
- Patients with increased pulmonary blood flow also had higher pulmonary blood flow and pulmonary artery pressure.
- No significant difference in ET concentrations was observed between patients with and without pulmonary hypertension within the increased flow group.
Conclusions:
- ET is elevated in children with congenital heart disease and left-to-right shunts.
- The elevation in ET appears to be related to increased pulmonary blood flow, independent of pulmonary artery pressure.
- This suggests a potential role for ET in the hemodynamic alterations associated with congenital heart defects and increased pulmonary perfusion.
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.
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