No registrations found.
ID
Source
Brief title
Health condition
aerobic capacity (peak V’O2)
quality of life
prevalence of arrhythmias
prevalence of protein losing enteropathy
Sponsors and support
Intervention
Outcome measures
Primary outcome
The primary objective of this study is to determine changes in aerobic capacity (peak V’O2) in adult patients with a Fontan circulation before and after treatment with the bosentan and compared to non-treated patients.
Secondary outcome
To determine the effect of the following parameters.
- changes in six-minute-walk-distance
- changes in quality of life score
- changes in prevalence of arrhythmias
- changes in congestive heart failure
(hospital admission, use of medication for congestive heartfailure)
- changes in prevalence of protein losing enteropathy
- changes in serum neurohormone level (NT-pro BNP, endothelin-1, albumin)
- changes in cardiac output
- changes in arterial oxygen saturation
- changes in number of deaths
Background summary
The Fontan procedure is a palliative surgical procedure used in patients with complex congenital heart defects. It involves diverting the venous blood from the right atrium to the pulmonary arteries without passing through the right ventricle. A low pulmonary vascular resistance (PVR) is crucial to preserve the Fontan circulation. Plasma endothelin-1 level, a vasoconstrictor which increases pulmonary vascular resistance, is elevated in patients with Fontan circulation. Treatment with bosentan, an endothelin receptor antagonist (ERA) lowers the pulmonary vascular resistance, which may result in improvement of the cardiopulmonary circulation.
Study objective
In adult Fontan patients, treatment with bosentan, an endothelin receptor antaganost (ERA) lowers the pulmonary vascular resistance, which may result in improvement of the cardiopulmonary circulation and functional capacity.
Study design
baseline, 3 months, and after 6 months of bosentan treatment.
During bosentan treatment regularly laboratory testing will be performed
Intervention
One group receives a 125 mg tablet of Bosentan twice daily for 6 months. The other group does not receive study medication for the first 3 months, followed by treatment with study medication for 6 months.
Department of cardiology <br>
Room F3 – 115
B.J.M. Mulder
Meibergdreef 9
Amsterdam 1105 AZ
The Netherlands
+31 (0)20 5666051
b.j.mulder@amc.uva.nl
Department of cardiology <br>
Room F3 – 115
B.J.M. Mulder
Meibergdreef 9
Amsterdam 1105 AZ
The Netherlands
+31 (0)20 5666051
b.j.mulder@amc.uva.nl
Inclusion criteria
1. All adult Fontan patients are potentially eligible for this study.
Exclusion criteria
- Patients are not eligible for this study if the following inclusion criteria apply:
1. Systemic arterial pressure < 85 mmHg
2. Incapable of giving informed consent
3. Hypersensitivity to bosentan or any of its help substances
4. Current treatment with bosentan or treatment for pulmonary arterial hypertension
5. Moderate to severe liver disease: Child-Pugh class B or C
6. Raised plasma transaminases level > three times limiting value
7. Simultaneous use of cyclosporine A
8. Desire to have children within the study period or women who do not use reliable contraceptive methods
9. Pregnant or nursing women
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| NTR-new | NL1487 |
| NTR-old | NTR1557 |
| Other | : 03602 |
| ISRCTN | ISRCTN wordt niet meer aangevraagd |