Pediatric REPlAcement of the PulmonaRy ValvE in Tetralogy of Fallot -

The purpose of this research study is to gather information on adolescents and young adults
to help understand and improve the lives of patients with TOF.

Some patients diagnosed with TOF will have a procedure called pulmonary valve replacement
(PVR) and some will not. PVR is done for valves that are too damaged to be repaired. This
requires a surgeon or an expert in a procedure called cardiac catheterization to replace the
damaged pulmonary valve with a valve made of tissue or amechanical valve. Multiple studies in
adult TOF patients have suggested that PVR may lessen many clinical symptoms but no one is
sure if it truly does. There is little information about PVR in adolescence but it is thought
that lessening the amount of leakage of the pulmonary valve at a young age may avoid future
complications such as right heart failure or abnormal beats of your heart. There is no
agreement among cardiologists, surgeons or other healthcare providers as to whether PVR truly
helps avoid complications in the future and if it does, when PVR should be done. Using the
information in this study, we hope to find out if PVR in adolescents is helpful in both the
short and long term.

The Investigators believe the results of this study will help provide doctors with enough
information to support a future large scale research study to further evaluate the outcomes
PVR. This study will involve randomization to either the PVR or no PVR cohort, medical
records review, exercise test and Cardiac Magnetic Resonance (CMR) , and questionnaires.

Inclusion Criteria:

1. Males or females with repaired Tetralogy of Fallot (TOF), currently between 13 and 21
years of age.

2. On clinical Cardiac Magnetic Resonance (CMR) : Right Ventricular End-Diastolic Volume
Index (RVEDVi) between 140 and 180 cc/m2 inclusive with Right Ventricular
End-Diastolic Function (RVEF) > 40% and Left Right Ventricular End-Diastolic (LVEF ) >
50%, RV outflow tract peak velocity < 3 meters/second; there will be no indexed Right
Ventricular end-systolic volume (RVESVi) criteria; by defining RVEDVi and RVEF,
Investigators will be inherently defining RVESVi

3. On clinical echocardiogram: RV outflow tract peak velocity < 3 meters/second and
tricuspid regurgitation with an RV pressure estimate < 1/2 systemic pressure.

4. On Exercise Stress Test (EST), aerobic capacity > 70% of predicted.

5. No QRS duration criteria on ECG.

Exclusion Criteria:

1. Any condition judged by the patient's physician that would cause this trial to be
detrimental to the patient.

2. Specific forms of TOF excluded are those with endocardial cushion defects, TOF with
absent pulmonary valve and TOF with multiple aorto-pulmonary collaterals requiring
unifocalization.

3. Unilateral branch pulmonary artery stenosis (one lung receives < 25% of total flow)

4. Contraindication to non-sedated exercise CMR (e.g. pacemaker/implanted cardioverter
defibrillator); need for sedation

5. Moderate or greater tricuspid regurgitation on echocardiogram or CMR or Qp/Qs > 1.5

6. Significant strokes/hemiplegia or inability to exercise

7. Genetic syndrome/developmental delay which would make QOL and EST date uninterpretable

8. Pregnancy