Early Versus Later Re-valving in Tetralogy of Fallot With Free Pulmonary Regurgitation

Rigshospitalet

Status: Enrolling

Conditions: Pulmonary Regurgitation; Tetralogy of Fallot

Treatments: Procedure: Pulmonary valve replacement

Study type: Interventional

Funder types: Other

Identifiers

NCT04084132

VD-2018-512

Details and patient eligibility

About

Tetralogy of Fallot (ToF) is a congenital heart defect with four major features including right ventricular outflow tract obstruction. About 25 children are born with this condition in Denmark every year. Corrective surgery is usually performed within the first year. In 50 % of patients, enlargement with a patch is necessary to achieve relief of the outflow tract obstruction. This however results in severe pulmonary regurgitation, which eventually leads to volume overload, right ventricular dysfunction and arrhythmia. To avoid these late complications, pulmonary valve replacement with a prosthesis if performed when patients meet the current guideline criteria. Most patients meet the guideline criteria for revalving when they are between 20 and 30 years of age. The current guidelines however, are based solely on retrospective studies and novel research reveals that in more than 50 % of patients who are treated according to current practice, right ventricular volumes and function as well as exercise capacity and burden of arrhythmia do not normalize or improve. 500 patients with ToF will be enrolled in a multicentre, cross-sectional study, which will yield information about the long-term outcomes after initial repair of ToF, as well as suggestions about the optimal timing for re-valving. Among patients included in the cross-sectional study, 120 patients with free pulmonary regurgitation, will be randomized evenly for early or later re-valving with at least 10-years of follow-up, for evaluation of long-term efficacy and safety of early re-valving.

Enrollment: 120 estimated patients

Sex: All

Ages: 12+ years old

Volunteers: No Healthy Volunteers

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.

Trial design

Primary purpose: Treatment

Allocation: Randomized

Interventional model: Parallel Assignment

Masking: Single Blind

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120 participants in 2 patient groups

Early re-valving

Experimental group

Description:

60 patients who are assigned to early re-valving undergo pulmonary valve replacement within 3 months from randomization.

Treatment:

Procedure: Pulmonary valve replacement

Later re-valving

Experimental group

Description:

60 patients who are assigned to later re-valving undergo pulmonary valve replacement when the current European guideline criteria are met.

Treatment:

Procedure: Pulmonary valve replacement