Does pulmonary valve replacement improve outcomes in repaired tetralogy of fallot? A systematic review and meta-analysis

Background In individuals with chronic pulmonary regurgitation following repair of Tetralogy of Fallot (TOF), pulmonary valve replacement (PVR) is commonly undertaken. Nevertheless, uncertainty persists regarding its long-term influence on survival, arrhythmic burden, heart-failure progression, and right ventricular (RV) structural recovery. We conducted a meta-analysis of modern cohort studies to compare clinical and functional outcomes between patients treated with PVR and those managed without intervention. Methods Five observational cohort studies encompassing approximately 3,479 patients were included. Assessed outcomes comprised all-cause and cardiovascular mortality, overall arrhythmias and sustained ventricular tachycardia (VT), advanced heart failure, RV end-diastolic volume index (RVEDVi), percentage predicted peak oxygen consumption (VO2), and RV systolic pressure. Random-effects models or Peto fixed-effect methods were applied as appropriate. Results PVR was not associated with a statistically significant reduction in all-cause mortality (OR 0.66, 95% CI 0.41–1.08; p=0.10). However, cardiovascular mortality was significantly lower in the PVR group (OR 0.54, 95% CI 0.29–0.98; p=0.043). No significant differences were observed in overall heart-failure events (OR 0.96; p=0.92) or sustained VT (OR 1.01; p=0.99). Although a non-significant trend toward increased arrhythmia incidence following PVR was noted (OR 2.01, 95% CI 0.85–4.75; p=0.11; I²=69%), this appeared largely attributable to heterogeneity driven by a single study with markedly elevated event rates. With respect to structural and functional measures, PVR was associated with a substantial reduction in RVEDVi compared with conservative management (mean difference –48.7 mL/m²; p<0.001), reflecting significant reverse remodeling. RV systolic pressure increased modestly after PVR (mean difference +7.45 mmHg; p<0.001), while percent predicted peak VO2 did not demonstrate meaningful improvement (mean difference –4.96%; p=0.32). Conclusions In patients with repaired TOF, PVR consistently produces marked RV volumetric reverse remodeling but shows neutral effects on overall mortality and major arrhythmic outcomes, despite a potential reduction in cardiovascular mortality. These findings reinforce the structural benefits of PVR while highlighting the necessity for prospective, adequately powered studies to clarify its long-term clinical impact.