On Saturday, Jan. 31 at 2:45 p.m., during the “Adult Congenital Heart Disease” session, Jennifer Nelson, MD, of Nemours Children’s Hospital will present Indications and Timing of Pulmonary Valve Replacement in Repaired Tetralogy of Fallot. Her talk will highlight a new, comprehensive body of work designed to bring greater clarity and consistency to decision-making around pulmonary valve replacement (PVR) for one of the largest populations of patients living with repaired congenital heart disease.
PVR is a common and critical intervention for patients with repaired tetralogy of Fallot (TOF) and clinically significant pulmonary regurgitation; however, indications and timing have varied widely across practice settings. To reduce this variability, The Society of Thoracic Surgeons (STS), in collaboration with the World Society of Pediatric and Congenital Heart Surgery (WSPCHS) and the European Congenital Heart Surgeons Association (ECHSA), developed a three-part series of complementary clinical practice documents: Clinical Practice Guidelines, a pediatric-focused Expert Consensus Document, and an Expert Opinion paper on the role of exercise testing.
Developed through a rigorous literature review, adherence to PRISMA methodology, and a modified Delphi consensus process, these documents synthesize available evidence and expert judgment to establish practical, consensus-based recommendations.
Key considerations include symptoms, MRI-based ventricular assessment, arrhythmia risk, and procedural factors, emphasizing individualized, data-driven decisions over single thresholds. “Routine, standardized measurement during long-term follow-up is essential,” says Dr. Nelson, who also highlights the role of multidisciplinary review and exercise testing in revealing unrecognized functional limitations.
The new guidance incorporates emerging MRI-based evidence linking ventricular changes to mortality and supports earlier consideration of pulmonary valve replacement in select asymptomatic adults. It also emphasizes that arrhythmia risk persists after intervention, reinforcing the need for continued surveillance.
Together, this three-part series provides an updated framework to guide clinicians caring for children and adults with repaired TOF, balancing evolving evidence with real-world clinical complexity. “These recommendations matter because they should change practice,” Dr. Nelson adds. “They help clinicians better identify the right patient and the right time for pulmonary valve replacement, with the goal of improving long-term outcomes while minimizing unnecessary risk.”