Ambrisentan in Single Ventricle

• Status: Completed
• Trial end date: 2017-08-20
• Target enrollment: 0
• Participant gender: All

Summary

Purpose: To evaluate the pharmacokinetics, bioavailability and hemodynamic efficacy of ambrisentan after Fontan surgical palliation of single ventricle heart defects.

Study activities and population group: Children undergoing Fontan surgical palliation for single ventricle defects will be eligible for the study. Up to 20 subjects will be enrolled (16 ambrisentan, 4 placebo) and will receive 3 days (3 doses) of ambrisentan starting on post-operative day #1 upon returning from the operating room. Ambrisentan plasma levels will be obtained at specified time points during treatment. Post-operative monitoring lines will be used to measure effects of ambrisentan on hemodynamics and pulmonary / systemic endothelial function.

• Phase: Phase 2
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Kevin Hill
• Treatments: Ambrisentan

Criteria

Inclusion Criteria:

1. Age ≥ 24 months; ≤120 months.

2. History of congenital heart disease with severe hypoplasia of a right or left ventricle.

3. Undergoing Fontan surgery as part of standard clinical care.

4. Availability and willingness of the parent/legally authorized representative to provide written informed consent and, as appropriate, assent from the child.

Exclusion Criteria:

1. History of serious adverse event related to ambrisentan administration.

2. History of ambrisentan exposure within 48 hours of the study.

3. Presence of pulmonary venous obstruction.

4. Treatment with cyclosporin.

5. Any of the following - as determined by the attending physician

• Significant hemodynamic instability

• Sepsis.

• Need for ECMO support.

6. Renal failure defined as serum creatinine > 2 times higher than the upper limit of normal.

7. Liver dysfunction defined as alanine aminotransferase or aspartate aminotransferase > 3 times higher than the upper limit of normal.

8. Thrombocytopenia defined as a platelet count < 50 000 cells/µL.

9. Leukopenia defined as white blood cells < 2500 cells/µL.

10. Anemia defined as hemoglobin < 8mg/dL.

11. Atrial hypertension (mean LA pressure > 12mm Hg).