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Abstract INTRODUCTION Congenital heart diseases are the most common congenital anurrmlies in children and infants. The incidence is about 0.8% of live births (Fridman. 1995). Hickey et a!. in ( 191!5! described cnuothelin as enuotensin or endothelial contracting factor. It was described as a naturally occurring polypeptide substance produced by vascular endothelial cells and has a potent vasoconstrictor and mitogenic activity on vascular smooth muscle cells (Mathias eta/., 1995). Endothelin is considered as a disease marker or an etiologic factor in pulmonary hypertension, systemic hypertension, congestive heart failure. myocardial and vascular wall hypertrophy, ischemic heart disease and atherosclerosis ( Praveen, 1995). Yoshibarashi et a/. in (1991) discovered that endothelin may be involved in pathophysiology of pulmonary hypertension. Also. Vincent et a/. I1993) suggested that the increase in pulmonary blood !low may be a stimulatory factor in production andlor release of endothelin in children with congenital heart defects. This was also reported by Ishikawa eta/. in (1995) who studied patients with left to right shunt. They found signiticantly higher levels of endothelin in their patients when compared to control subjects. AIM OF THE WORK: The aim of this work is to study the role of endothelin in the contribution in the haemodynamics disturbances and abnormal blood tlow in patients \Vith congenital heart disease. 2 |