Endothelin Blockade in Patients With Single Ventricle Physiology

Endothelin Blockade in Patients With Single Ventricle Physiology

Recent studies have shown beneficial effects of sildenafil in patients with single ventricle congenital heart disease. The purpose of this study is to determine whether Bosentan, a drug with similar effects, will have similar benefit in this patient population.

Patients enrolled in the study will take Bosentan. Baseline and follow up measurements will be collected then analyzed.

Inclusion Criteria:

• At least 12 years of age
• Single ventricle congenital heart disease
• Require cardiac magnetic resonance imaging (MRI) for clinical purposes.

Exclusion Criteria:

• < 12 years of age
• Pregnancy or women who may become pregnant (sexually active and unwilling to use birth control)
• Patients taking cyclosporin or glyburide
• Patients with baseline liver dysfunction (aspartate aminotransferase or alanine aminotransferase > 3 times upper limit of normal)
• Contraindications to MRI (including pacemakers)
• Use of Bosentan within one month prior to enrollment