Bosentan Therapy in Children With Functional Single Ventricle

Bosentan Therapy for High Risk Staged Fontan Procedure in Children With Functional Single Ventricle

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.

Study Overview

• Status: Completed
• Conditions: Congenital Heart Defects; Functional Single Ventricle
• Intervention / Treatment: Drug: Bosentan

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 Type: Interventional
• Enrollment (Actual): 34
• Phase: Phase 2

Contacts and Locations

Study Locations

• China
  • Shanghai
    • Shanghai, Shanghai, China, 200127
      • Cardiac intensive care unit, Department of cardiothoracic vascular surgery, Shanghai Children's Medical Center, Medical college of Shanghai Jiaotong University

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 4 months to 18 years (ADULT, CHILD)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

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 Plan

How is the study designed?

Design Details

• Primary Purpose: TREATMENT
• Allocation: NON_RANDOMIZED
• Interventional Model: PARALLEL
• Masking: NONE

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
EXPERIMENTAL: Bosentan group; 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.	Drug: 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.; Other Names: Tracleer
NO_INTERVENTION: Routinely group; Routinely therapy in children with functional single ventricle during the period of staged Fontan procedure with high risk of increased PVR.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Length of hospital stay and ICU stay	12 months after Fontan operation

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Symptoms of increased PVR	facial edema plural effusion pericardium effusion	12 months after Fontan operation
WHO functional class		12 months after Fontan operation

Collaborators and Investigators

• Sponsor: Shanghai Jiao Tong University School of Medicine
• Investigators: Study Director: Zhuoming Xu, MD PhD, Cardiac intensive care unit, Department of Thoracic and cardiovascular Surgery, Shanghai children's Medical Center

Study record dates

Study Major Dates

• Study Start: January 1, 2010
• Primary Completion (ACTUAL): September 1, 2011
• Study Completion (ACTUAL): June 1, 2012

Study Registration Dates

• First Submitted: August 5, 2012
• First Submitted That Met QC Criteria: August 7, 2012
• First Posted (ESTIMATE): August 10, 2012

Study Record Updates

• Last Update Posted (ESTIMATE): August 10, 2012
• Last Update Submitted That Met QC Criteria: August 7, 2012
• Last Verified: August 1, 2012

More Information

Terms related to this study

• Keywords: Bosentan; Pulmonary vascular resistance; Heart defects, Congenital; Receptors, endothelin
• Additional Relevant MeSH Terms: Heart Diseases; Cardiovascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Heart Defects, Congenital; Univentricular Heart; Molecular Mechanisms of Pharmacological Action; Antihypertensive Agents; Endothelin Receptor Antagonists; Bosentan
• Other Study ID Numbers: SJTUMS-20120314