Comparing Early Versus Later Pulmonary Valve Replacement in Patients Aged 12 and Older with Tetralogy of Fallot and Pulmonary Regurgitation

What this Trial Is About

Tetralogy of Fallot ToF is a congenital heart defect characterized by four major features, including obstruction of the right ventricular outflow tract. About 25 children in Denmark are born with ToF each year, and corrective surgery is typically done within the first year of life. Many patients require enlargement of the outflow tract with a patch, which can lead to severe pulmonary regurgitation and later complications like right ventricular dysfunction and arrhythmias. This trial aims to evaluate the long-term outcomes after initial ToF repair and to determine the best timing for pulmonary valve replacement in patients with free pulmonary regurgitation. The study will enroll 500 patients in a multicenter, cross-sectional evaluation, with 120 patients who have free pulmonary regurgitation randomized into two groups. One group will undergo early pulmonary valve replacement within 3 months after randomization, while the other will have the procedure later when they meet current European guideline criteria. The valve replacement involves surgical implantation of an adult-sized homograft or Contegra graft under cardiopulmonary bypass through a sternotomy. Participants will be followed for at least 10 years to assess long-term safety and effectiveness. Participants will be monitored regularly with yearly assessments including heart function measurements by MRI, exercise testing, arrhythmia monitoring, quality of life evaluations, and health system contacts. Primary outcomes include right ventricular end-diastolic volume indexed to body surface area and mortality rates measured 3 years after randomization. Secondary outcomes cover additional cardiac function parameters, complication rates after surgery, stroke and mortality rates, and quality of life changes over 10 years. The study also tracks valve deterioration and other clinical events to gain comprehensive information on the impact of early versus later re-valving.

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