Bosentan Therapy in Children With Functional Single Ventricle

Phase 2

Completed

• Conditions: Congenital Heart Defects; Functional Single Ventricle
• Interventions: Drug: Bosentan

• Registration Number: NCT01662037
• Lead Sponsor: Shanghai Jiao Tong University School of Medicine

Brief 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.

Detailed Description

Increased pulmonary vascular resistance (PVR) is a serous issues in children with functional single ventricle during the staged operative period. The purpose of this study is to investigate if Bosentan therapy can improve the survival and life quality after staged Fontan procedure in the children with high risk of increased PVR.

Study Timeline

• Study Start: 2010-01
• Primary Completion: 2011-09
• Study Completion: 2012-06
• Status Verified: 2012-08
• Study First Submitted: 2012-08-05
• Study First Submitted QC: 2012-08-07
• Last Update Submitted: 2012-08-07
• Study First Posted: 2012-08-10
• Last Update Posted: 2012-08-10

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 34

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
Bosentan group	Bosentan	Bosentan 2mg/kg/dose twice a day and routinely therapy in children with functional single ventricle during the period of staged Fontan procedure with high risk of increased PVR.

Primary Outcome Measures

Name	Time	Method
Length of hospital stay and ICU stay	12 months after Fontan operation

Secondary Outcome Measures

Name	Time	Method
Symptoms of increased PVR	12 months after Fontan operation	facial edema plural effusion pericardium effusion
WHO functional class	12 months after Fontan operation

Trial Locations

Locations (1)

Cardiac intensive care unit, Department of cardiothoracic vascular surgery, Shanghai Children's Medical Center, Medical college of Shanghai Jiaotong University; 🇨🇳 Shanghai, Shanghai, China