Journal article

Publication year: 2016

Pages: 1052-1058

Journal: Heart

Volume number: 102

Issue number: 13

ISSN: 1355-6037

eISSN: 1468-201X

DOI Link: https://doi.org/10.1136/heartjnl-2015-308743

Publisher: BMJ Publishing Group

Abstract: 

Objective 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.

Methods 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.

Results 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.

Conclusions 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.

Harvard Citation style: Latus, H., Karanatsios, G., Basan, U., Salser, K., Mueller, S., Khalil, M., et al. (2016) Clinical and prognostic value of endothelin-1 and big endothelin-1 expression in children with pulmonary hypertension, Heart, 102(13), pp. 1052-1058. https://doi.org/10.1136/heartjnl-2015-308743

APA Citation style: Latus, H., Karanatsios, G., Basan, U., Salser, K., Mueller, S., Khalil, M., Kreuder, J., Schranz, D., & Apitz, C. (2016). Clinical and prognostic value of endothelin-1 and big endothelin-1 expression in children with pulmonary hypertension. Heart. 102(13), 1052-1058. https://doi.org/10.1136/heartjnl-2015-308743