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

Randomized Single-blind Study of Intravenous Maintenance of Remodulin® for the Treatment of Pulmonary Hypertension After Fontan Operation

The aim of this study is to determine the safety and efficiency of Remodulin®（Treprostinil Injection）to reduce the pulmonary arterial pressure and prevent pulmonary hypertension (PH) after Fontan operation with univentricular physiology.

PH is a significant contributor to the postoperative morbidity and mortality of congenital heart disease, especially after Fontan operation with univentricular physiology. Mild increase of pulmonary vascular resistance may lead to failure of Fontan circulation. Remodulin® has been approved for the treatment of adults with PH, but little is known about the effects in children with PH after Fontan operation. The study aim is to determine the safety and efficiency of Remodulin® to reduce the pulmonary arterial pressure and prevent PH in children after Fontan operation. Meanwhile pharmacokinetics of the drug were checked with or without the peritoneal dialysis.

Inclusion Criteria:

After Fontan procedure, the criteria should be met

1. mPAP greater than 15 mmHg
2. TPG greater than 6 mmHg (exclude the obstruction of cavopulmonary anastomosis)

Exclusion Criteria:

After Fontan surgery :

1. Severe arrhythmia led to low cardiac output
2. Platelets smaller than 50,000*109/L and obvious bleeding