Accessible Unlicensed Requires Authentication Published by De Gruyter June 1, 2005

Endothelin-1- and Endothelin-Receptors in Lung Biopsies of Patients with Pulmonary Hypertension due to Congenital Heart Disease

Jens Lutz, Matthias Gorenflo, Matthias Habighorst, Martin Vogel, Peter E. Lange and Berthold Hocher
From the journal


Endothelin-1 (ET-1), with its vasoconstrictive and proliferation-stimulating effects, could play a role in the pathogenesis of primary pulmonary hypertension. We investigated the relationship between the ET-1 like immunoreactivity and the ET-receptor density, the grade of the pulmonary vasculopathy, and properties of the pulmonary circulation in patients with pulmonary hypertension due to congenital heart disease.

Twenty-six patients with a median age of 1 year and 1 month (6 weeks–17 years–9 months) were assigned to group I (n = 15) with a pulmonary to systemic flow ratio (Qp/Qs) ≥ 1.5 and a pulmonary to systemic resistance ratio (Rp/Rs) ≤ 0.3 (“high flow–low resistance group”) and to group II (n = 11) with a Qp/Qs < 1.5 and an Rp/Rs > 0.3 (“low flow–high resistance group”).

Patients belonging to group II showed a higher ETA-receptor density in lung arteries (p < 0.05) and parenchyma (p < 0.01) than patients in group I. Patients with the highest ET-1 like immunoreactivity in lung artery walls also showed a trend towards a higher ETA-receptor density. The ETB-receptor expression was low and not related to any of the above factors.

Our results suggest that the paracrine lung ET-1 system is up-regulated in pediatric patients with secondary pulmonary hypertension associated with congenital heart disease.

Published Online: 2005-06-01
Published in Print: 1999-04-01

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