Study of Remodulin® in Pediatric Pulmonary Hypertension With Single Ventricular Physiology After Fontan Surgery

After Fontan operation, the mean pulmonary arterial pressure (mPAP)evaluated by CVP or transpulmonary pressure gradient(TPG) evaluated by Echo will be measured firstly, if the data met the criteria, and the patients were enrolled in the experimental group, the patients would be administrated intravenously of Remodulin® with the beginning of 5ng/kg/min, with the rate of 10ng/kg/min every 30 minutes up to 80ng/kg/min.

After Fontan operation, the mPAP (evaluated by CVP) or TPG (evaluated by Echo) will be measured firstly, if the data met the criteria, and the patients were enrolled in placebo group, then the patients would be administrated intravenously of distilled water with the beginning of 0.15ml/hr, with the rate of 0.3ml/hr every 30 minutes up to 2.45ml/hr.