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Phase 2 N=28 Randomized Quadruple-blind Treatment

Sildenafil After the Fontan Operation

Hypoplastic Left Heart Syndrome · Tricuspid Atresia

Enrolled (actual)
28
Serious AEs
1.8%
Results posted
May 2015
Primary outcome: Primary: Change From Baseline in Mean Oxygen Consumption (mL/kg/Min) at 6 Weeks — 30.5; 30.5; 31.3; 31.3 mL/kg/min — p=0.73

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Sildenafil (Drug); Placebo (Drug)
Age
Pediatric, Adult · 8+ yrs
Sex
All
Sponsor
Children's Hospital of Philadelphia
Primary completion
Apr 2009

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline in Mean Oxygen Consumption (mL/kg/Min) at 6 Weeks
30.5; 30.5; 31.3; 31.3 0.73
SECONDARY
Change From Baseline in Mean Heart Rate (Bpm) at 6 Weeks
163; 163; 163; 163 0.37
SECONDARY
Change From Baseline in Mean Respiratory Rate (Breaths/Min) at 6 Weeks
53.7; 53.0; 51.0; 53.0 0.05
SECONDARY
Change From Baseline in Mean Minute Ventilation (L/Min) at 6 Weeks
68.8; 68.1; 67.2; 68.8 0.04 sig

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.

Eligibility 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
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00507819). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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