Pulmonary valve replacement reduces right ventricular volumes by approximately 30–40% within 6 to 12 months in patients with repaired tetralogy of Fallot and chronic severe pulmonary regurgitation.
Does pulmonary valve replacement improve ventricular remodelling and clinical outcomes in patients with chronic pulmonary regurgitation after tetralogy of Fallot repair?
Pulmonary valve replacement provides consistent reverse remodelling and symptomatic benefit in patients with chronic pulmonary regurgitation after TOF repair, but the optimal timing remains uncertain due to a lack of conclusive survival benefit and conflicting data on arrhythmic outcomes.
Effect estimate: ~30-40% reduction in RVEDVi and RVESVi within 6-12 months post PVR
Chronic pulmonary regurgitation (PR) after the repair of tetralogy of Fallot (TOF) and other right ventricular outflow tract (RVOT) interventions leads to progressive right ventricular (RV) dilatation, altered ventricular–ventricular interaction, and an increased risk of arrhythmia and heart failure. Pulmonary valve replacement (PVR), whether surgical or transcatheter, effectively eliminates or reduces PR and is associated with short- and mid-term improvement in RV size, symptoms, and electrocardiographic markers. However, the optimal timing of intervention remains unresolved: operating late can result in irreversible myocardial damage and arrhythmogenic substrates, whereas operating early can lead to repeated reinterventions, the impact of which on hard outcomes is uncertain. This review summarizes contemporary evidence on ventricular remodelling after PVR, focusing on cardiovascular magnetic resonance (CMR) and echocardiographic markers, and critically appraises proposed criteria for timing PVR. Classic CMR-derived thresholds (RV end-diastolic volume index RVEDVi 150–170 mL/m2, RV end-systolic volume index RVESVi 80–90 mL/m2) and QRS duration cut-offs are discussed alongside emerging markers of risk, including the RV mass-to-volume ratio, diffuse myocardial fibrosis (extracellular volume fraction), strain imaging, and diastolic dysfunction. Meta-analyses show consistent reverse remodelling and symptomatic benefit after PVR, but no conclusive survival benefit has been demonstrated, and data on arrhythmic outcomes remain conflicting. Key gaps include (i) the lack of prospective randomized or carefully matched comparative studies of “early” versus “deferred” PVR; (ii) limited understanding of how myocardial fibrosis, RV hypertrophy, and diastolic dysfunction interact with volume load and timing to influence long-term outcomes; (iii) under-representation of adult and older adult TOF cohorts; and (iv) insufficient integration of multiparametric risk scores and machine-learning approaches into clinical decision-making. Future research should prioritize multicentre longitudinal cohorts with standardized imaging, electrophysiological and clinical endpoints, incorporate advanced imaging techniques (e.g., strain, 3D late gadolinium enhancement, and T1 mapping), and explore precision-medicine strategies to individualize PVR timing.
Ortiz-Garrido et al. (Fri,) conducted a review in Patients with repaired tetralogy of Fallot and chronic severe pulmonary regurgitation undergoing pulmonary valve replacement (n=3,118). Pulmonary valve replacement (surgical or transcatheter) vs. No pulmonary valve replacement or deferred PVR was evaluated on Right ventricular remodeling after pulmonary valve replacement measured by CMR-derived RV end-diastolic volume index (RVEDVi), RV end-systolic volume index (RVESVi), and other functional parameters (~30-40% reduction in RVEDVi and RVESVi within 6-12 months post PVR). Pulmonary valve replacement reduces right ventricular volumes by approximately 30–40% within 6 to 12 months in patients with repaired tetralogy of Fallot and chronic severe pulmonary regurgitation.