Bosentan Therapy in Children With Functional Single Ventricle

• Status: Completed
• Trial end date: 2012-06-01
• Target enrollment: 0
• Participant gender: All

Summary

Bosentan is a kind of dual endothelin receptor antagonist.The purpose of this study is to investigate if Bosentan therapy can modify the outcome of children with functional single ventricle.

• Phase: Phase 2
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Shanghai Jiao Tong University School of Medicine
• Treatments: Bosentan

Criteria

Inclusion Criteria:

• Signed informed consent obtained from patient's legally acceptable representative.

• Pediatric patients waiting for staged Fontan procedure with high risk of increased PVR after bidirectional cavopulmonary connection (BCPC)

• Transpulmonary pressure gradiant (TPG) > 10mmHg when the obstruction of anastomosis and lung problem were excluded.

• With the diagnosis of high risk of increased PVR, such as associated with TAPVC, after pulmonary artery banding, after systemic to pulmonary shunt more than 6 months, and et al.

• Diagnosed as increased PVR with catheterization.

Exclusion Criteria:

• PAH associated with conditions other than those mentioned above, e.g., iPAH, PAH secondary to portal hypertension, HIV patient with opportunistic infection

• Psychotic, addictive or other disorder limiting the ability to provide informed consent or to comply with study requirements

• AST and/or ALT > 3 times the upper limit of normal ranges.

• Hemoglobin concentration < 75% the lower limit of normal ranges

• Treatment or planned treatment with another investigational drug within 3 months of screening

• Treatment with calcineurin-inhibitors (e.g., cyclosporine A and tacrolimus), fluconazole, glibenclamide (glyburide) within 1 week of enrollment of this study

• Known hypersensitivity to bosentan or any of the excipients