Application Progress of Endothelin-1 Receptor Antagonists in Treatment of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Endothelin-1(ET-1) is a powerful vasoconstrictor and mitogen for smooth muscle and plays an important role in the development of pulmonary arterial hypertension(PAH).An activation of the ET-1 system has been demonstrated in PAH patients as well as in animal models of PAH.The most efficient way to antagonize the ET-1 system is the use of ET-1 receptor antagonists which mainly include bosentan,sitaxsentan and ambrisentan.Lots of researches have showed ET-1 receptor antagonists can significantly reduce pulmonary artery pressure of patients with idiopathic PAH.Recently,the use of ET-1 receptor antagonists in the treatment of PAH associated with congenital heart disease has made some progress.