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

Shanghai Jiao Tong University School of Medicine36 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

PREVENTION

Masking

SINGLE

Enrollment

Conditions

Pulmonary Hypertension

Interventions

RemodulinDRUG

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.

distilled waterDRUG

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.

Eligibility

Sex: ALLMin age: 2 YearsMax age: 18 Years

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

The primary end point is a composite variable

consisting of death, failing Fontan or failed Fontan according to high pulmonary vascular resistance

Time frame: the first 48 hours after Remodulin used

Secondary Outcomes

Change from base line of pulmonary hemodynamic measurements

Pp/Ps reduce \>10% or TPG≤6mmHg.

Time frame: baseline and 48 hours

Data from ClinicalTrials.gov

This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.