PREDICTIVE VALUE OF ENDOTHELINE-1 IN DIAGNOSIS OF PULMONARY ARTERY HYPERTENSION IN PATIENTS OF ACYANOTIC CHD WITH LEFT TO RIGHT SHUNT

Abstract

Background: Eisenmenger's syndrome represents the most advanced form of pulmonary hypertension
with congenital heart disease (PAH-CHD). The current current European Society of Cardiology
guidelines provide an anatomical-pathophysiological classification of the congenital left-to-right shunts
associated with PAH. This study aimed to evaluate the role of Endothelin-1 (ET-1) blood level as a
diagnostic marker of pulmonary hypertension in children with CHD. Patient and methods: A casecontrol study included 48 children and conducted in Pediatric Cardiology Unit, Pediatrics Department
in Zagazig University Hospitals as a first time after obtaining the required permissions and informed
consent from the participating patients. They were divided into Group (A) involved 24 children with
acyanotic CHD with left to right shunt with PAH; Group B involved 24 children with acyanotic CHD
with left to right shunt but no PAH. All participants were subjected to careful history taking, thorough
clinical examination, plain X ray chest postero-anterior view, echocardiography and measurement of
endothelin-1 level. Results: SBP, HR and RR were significantly higher among Group A while SPO2
was significantly lower among group A. No significant difference between groups regard distribution
of type of anomalies and the majority were ASD and PDA in both groups. No significant difference
between groups regard Murmur.Group A was significantly higher regard pulmonary blood pressure.
Endotheline_1 was significantly higher among Group A as it was distributed as 2.31±0.76 and
1.06±0.42 respectively. Conclusion: ET-1 can be considered an indicator of endothelial injury and a
biomarker for predicting PAH complicated by CHD and provides a new clinical thought for diagnosing
PAH complicated by CHD.