Comparing early and late pulmonary valve replacement in children with Tetralogy of Fallot

Early Versus Later Re-valving in Tetralogy of Fallot With Free Pulmonary Regurgitation - Combined Cross-sectional and Prospective, Multi-centre, Randomized, Parallel-group Clinical Trial

This study is testing whether replacing the pulmonary valve earlier or later in children with Tetralogy of Fallot can lead to better heart health and fewer problems down the road.

Quick facts

• Phase: Not applicable
• Study type: Interventional
• Enrollment: 120 (estimated)
• Ages: 12 Years and up
• Sex: All
• Sponsor: Rigshospitalet, Denmark (Academic / other)
• Locations: 3 sites (Aarhus and 2 other locations)
• Trial ID: NCT04084132 on ClinicalTrials.gov

What this trial studies

This clinical trial investigates the timing of pulmonary valve replacement in children diagnosed with Tetralogy of Fallot who experience free pulmonary regurgitation. The study aims to determine whether early intervention leads to better outcomes compared to later re-valving, as current guidelines are based on retrospective data. Participants will undergo MRI assessments to evaluate their right ventricular anatomy and function, which will guide the timing of the valve replacement procedure. The trial seeks to improve long-term heart function and reduce complications associated with pulmonary regurgitation.

Who should consider this trial

Good fit: Ideal candidates are children with Tetralogy of Fallot who have undergone repair with a transannular patch and are suitable for adult-sized conduit implantation.

Not a fit: Patients with significant associated anomalies or those who are symptomatic may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to improved heart function and reduced complications for patients with Tetralogy of Fallot.

How similar studies have performed: While this approach is based on existing guidelines, it aims to provide new insights that could challenge current practices, indicating a novel exploration in this area.

Eligibility criteria

Inclusion Criteria:

1. ToF with pulmonary stenosis repaired with a TAP within the first two years of life.
2. RVOT anatomy is suitable for implantation of an adult sized conduit ( 18 mm homograft or Contegra graft) as assessed by MRI.

Exclusion Criteria:

1. ToF with pulmonary atresia, ToF with common atrioventricular canal, ToF with absent pulmonary valve syndrome, major aortopulmonary collateral arteries and other significant associated anomalies.
2. Palliation with a shunt (Blalock-Taussig or central) at any time.
3. The patient is symptomatic.
4. Sustained supraventricular or ventricular arrhythmia.
5. RVEDVi \> 140 mL/m2 as assessed by MRI (appendix 1).
6. RVESVi \> 60 mL/m2 as assessed by MRI.
7. RVEF \< 50 % as assessed by MRI.
8. Moderate or severe tricuspid regurgitation as assessed by echocardiography or MRI.
9. Significant residual lesions requiring intervention (e.g. ventricular septal defect, aortic regurgitation, branch pulmonary artery stenosis).
10. Co-morbidity preventing exercise testing (e.g. genetics, neuro-cognitive dysfunction, physical disability).
11. Contraindication for MRI (e.g. permanent pacemaker, intra-cardiac defibrillator, intracranial ferro-magnetic device).
12. Pregnancy at time of inclusion.
13. Age \< 12 or unable to comply with instructions given during MRI or exercise testing.

Where this trial is running

Aarhus and 2 other locations

• Aarhus University Hospital — Aarhus, Denmark (Not_yet_recruiting)
• Rigshospitalet — Copenhagen, Denmark (Recruiting)
• Odense University Hospital — Odense, Denmark (Not_yet_recruiting)

Study contacts

• Principal investigator: Lars Søndergaard, MD, DMSc — Rigshospitalet, Denmark
• Study coordinator: Mathis Gröning, MD, DMSc

Conditions Tetralogy of Fallot; Pulmonary Regurgitation