A single center, open lable clinical study of bosentan therapy in pediatric patients with severe pulmonary arterial hypertension due to congenital heart disease

• Register: ChiCTR
• Last refreshed on: 18 April 2017
• Main ID: ChiCTR-ONC-11001799
• Date of registration: 2011-12-15
• Prospective Registration: No
• Primary sponsor: Beijing Anzhen Hospital
• Public title: A single center, open lable clinical study of bosentan therapy in pediatric patients with severe pulmonary arterial hypertension due to congenital heart disease
• Scientific title: A single center, open lable clinical study of bosentan therapy in pediatric patients with severe pulmonary arterial hypertension due to congenital heart disease
• Date of first enrolment: 2011-02-17
• Target sample size: All groups:50;
• Recruitment status: Completed
• URL: http://www.chictr.org.cn/showproj.aspx?proj=7748
• Study type: Observational study
• Study design: historical control
• Phase: Post-market

Countries of recruitment

China

Contacts

• Name: Hong Gu

• Name: Peggy Chong Ma
• Affiliation: Real Data Medical Research Inc.

Key inclusion & exclusion criteria

Inclusion criteria: 1) Signed informed consent prior to initiation of any study-mandated procedure2)Males or females <=18 years old;
3) Patients with symptomatic PAH in WHO class II-IV due to congenital heart disease4)PAH-CHD diagnosed by 1) Signed informed consent prior to initiation of any study-mandated procedure;
2) Males or females <=18 years old;
3) Patients with symptomatic PAH in WHO class II-IV due to congenital heart disease;
4) PAH-CHD diagnosed by right heart catheter within 3 months prior to screening showing: PVR >=6 woods;
5) Congenital heart disease, including: VSD, ASD, PDA, CAVSD, and the combination of any of these conditions;
6) Didn't receive endothelin receptor antagonists, or prostanoids within 3 months prior to entering this study (except medication used for vaso-reaction test);
7) Patients received PDE-5 inhibitors should be in stable dosage at least 3 months prior to entering this study.
Exclusion criteria: 1. Patient with Eisenmenger syndrome;
2. Patients with known pulmonary lung disease or idiopathic pulmonary arterial hypertension;
3. Patients with uncorrected hemorrhagic disease or severe liver disease;
4. Patients who have hemodynamic instability;
5. Participated or planed to participate in other clinical studies within 3 months prior to entering this study;
6. Received endothelin receptor antagonists, or prostanoids within 3 months prior to entering this study (excluding drugs used in acute drug studies).

Age minimum: 0
Age maximum: 18
Gender: Both

Health Condition(s) or Problem(s) studied

pediatric patients with severe pulmonary arterial hypertension due to congenital heart disease

Intervention(s)

All groups:Bosentan therapy ;

Primary Outcome(s)

PVR;

Source(s) of Monetary Support

Actelion Pharmaceuticals Ltd