RT Journal Article
SR Electronic
T1 Clinical and prognostic value of endothelin-1 and big endothelin-1 expression in children with pulmonary hypertension
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP 1052
OP 1058
DO 10.1136/heartjnl-2015-308743
VO 102
IS 13
A1 Heiner Latus
A1 Georg Karanatsios
A1 Ulrike Basan
A1 Kirstin Salser
A1 Simon Müller
A1 Markus Khalil
A1 Joachim Kreuder
A1 Dietmar Schranz
A1 Christian Apitz
YR 2016
UL http://heart.bmj.com/content/102/13/1052.abstract
AB 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.