Study of Placebo or Bosentan to Treat Patients With Single Ventricle Physiology.
Status:
Completed
Trial end date:
2013-03-01
Target enrollment:
Participant gender:
Summary
The purpose of this study is to determine whether Bosentan is an effective and safe treatment
to adolescent and adult (15 years and older) patients, born with one ventricle of the heart
instead of two (single ventricle physiology) and who have undergone TCPC as a palliative
surgical treatment. The aim of the TCPC operation is to use the one functioning ventricle to
pump the blood flow to the body, while the blood to the lungs is received directly from the
caval veins, and is thus a passive flow, without the aid of a ventricle to actively pump the
blood through the pulmonary circulation. The resistance in the pulmonary circulation is
therefore critical to these patients. These patients have markedly lower work capacity in
bicycle test than the general public. Furthermore they have a high risk of developing
complications e.g. loss of protein from the intestines.
Bosentan is a medication that lowers the resistance in the pulmonary circulation. It is
routinely used for patients with pulmonary hypertension. Some studies have shown that drugs
that lower the pulmonary resistance can increase exercise capacity significantly in patients
with single ventricle physiology.
In this study 80 patients will receive either placebo or Bosentan for 14 weeks. Before and
after the treatment, bicycle test along with blood samples, stool samples and quality of life
interviews will be performed. Every four weeks during the study blood samples, physical exam
and interviews will be performed to ensure the safety of the treatment.
The investigators expect to find a significant increase in work capacity after 14 weeks in
the treatment group compared with the placebo group.
Moreover the investigators hope to find a decrease in intestinal protein loss and an improved
quality of life.
Phase:
Phase 2
Details
Lead Sponsor:
Rigshospitalet, Denmark
Collaborators:
Aarhus University Hospital Actelion Bispebjerg Hospital