Tetralogy of Fallot (ToF) repair in infancy has dramatically improved mid- and long-term survival, yet almost all patients develop chronic pulmonary regurgitation due to loss of pulmonary valvular competence. This sequela leads to progressive right ventricular (RV) dilation and dysfunction, substantially increasing the risk of life-threatening arrhythmias and long-term morbidity. Pulmonary valve replacement (PVR) is the standard intervention for severe pulmonary regurgitation, but questions remain regarding the optimal timing, risk stratification, and potential functional and survival benefits of the procedure. Clinical decision-making is complicated by evolving predictors—including RV dimensions, biventricular function, QRS duration and fragmentation, and cardiopulmonary exercise testing (CPET) parameters—yet consensus on appropriate thresholds and indications is lacking. Furthermore, outcome data regarding arrhythmia reduction, exercise capacity, and durability of prosthetic valves—across surgical and percutaneous approaches—remain inconclusive.

This special issue seeks to advance the evidence base underpinning the timing, technique, and long-term impact of PVR in patients with repaired ToF. We aim to clarify risk predictors, refine clinical decision-making, and determine the influence of intervention on long-term survival, functional status, arrhythmic burden, and device implantation rates.

We invite high-quality original research articles, longitudinal cohort studies, and systematic reviews that address critical aspects of PVR in repaired ToF, including but not limited to:
• Adverse outcomes post-PVR: sudden cardiac death, cardiac arrest, heart failure mortality, ventricular and supraventricular arrhythmias;
• Short- and long-term changes in functional capacity as assessed by CPET (VO2 predicted value, VE/VCO2 slope, oxygen pulse, etc.);
• Longitudinal monitoring of RV and biventricular size/function, and the relationship with prosthetic valve performance;
• Comparative durability of prosthetic valves by type and implantation technique, and analysis of re-intervention rates;
• Incidence and clinical correlates of ICD and/or pacemaker implantation after PVR.

We encourage submission of studies employing advanced imaging, contemporary electrophysiological monitoring, and innovative exercise testing modalities, as well as those exploring thresholds for intervention. Both pediatric and adult data are welcome. This Research Topic aims to inform clinical guidelines and improve future outcomes for this growing patient population.

Are you interested in contributing a manuscript to this Research Topic?

Optimizing Pulmonary Valve Replacement Outcomes in Tetralogy of Fallot Repair