Sildenafil After the Fontan Operation

• Status: Completed
• Trial end date: 2009-07-01
• Target enrollment: 0
• Participant gender: All

Summary

In this study, the investigators will evaluate the effect of sildenafil on exercise tolerance in patients with a single cardiac ventricle who have undergone the Fontan operation. The investigators will also evaluate echocardiographic measures of ventricular function and measure quality of life changes using two validated quality of life measures. The hypothesis is that sildenafil will result in increased exercise tolerance in patients who have had the Fontan operation as compared to placebo.

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

Details

• Lead Sponsor: Children's Hospital of Philadelphia
• Collaborator: The Mark H. and Blanche M. Harrington Foundation
• Treatments: Sildenafil Citrate

Criteria

Inclusion Criteria:

• 8 years of age or older

• All participants must have had Fontan completion

Exclusion Criteria:

• Height less than 132 cm

• Unable to participate in exercise testing due to medical restrictions or physical limitations

• Fontan baffle obstruction or single lung physiology

• Coarctation of the aorta or neo-aorta (BP gradient > 20 mmHg)

• Severe ventricular dysfunction assessed qualitatively by echocardiography

• Severe atrioventricular valvar regurgitation assessed qualitatively by echocardiography

• Presence of electronic pacemaker

• History of treatment with sildenafil in the six weeks prior to enrollment in study

• Patients with severe renal impairment

• Patients with severe hepatic impairment

• Patients taking medications that inhibit or induce Cytochrome P450 3A4 (CYP3A4) (including grapefruit juice and St. John's Wort)

• Patients taking alpha-blockers and nitrates

• Parents/guardians or subjects who, in the opinion of the investigator, may be non-compliant with study schedules or procedures