The Evolving Clinical Profile of Patients with Repaired Tetralogy of Fallot Undergoing Pulmonary Valve Replacement

Timely pulmonary valve replacement (PVR) is increasingly used to treat right ventricular outflow dysfunction in patients with repaired tetralogy of Fallot (rTOF). Although PVR procedural volume has increased over the last decade, the evolution of the clinical profiles of patients referred for surgical or transcatheter PVR (sPVR, tPVR) has not been clearly elucidated.

To evaluate the clinical characteristics of rTOF patients referred for PVR and to compare trends over time in patients undergoing sPVR versus tPVR.

This was a retrospective, single-center cohort study conducted at Boston Children's Hospital that analyzed clinical, arrhythmia, imaging, and exercise data from patients with rTOF who had undergone PVR, comparing it with data from matched controls without PVR, stratified by era (1995-2003 vs. 2004-2012 vs. 2013-2021). We also compared time trends in referral patterns for sPVR versus tPVR.

Over time, patients underwent TOF repair at a younger age. Among patients referred for PVR, age at the time of PVR remained stable, but the burden of heart failure symptoms and arrhythmias decreased, as did the severity of RV dilation and dysfunction. The percentage of tPVR increased from 0% in Era 1 to 21% in Era 2 to 38% in Era 3 (p<0.001). The increase in tPVR was particularly pronounced among patients with TOF and pulmonary stenosis, as well as among those with a transannular patch repair (from 0% to 27% to 47% across eras; p<0.001). In contrast, the frequency of sPVR in patients with a right ventricle-to-pulmonary artery conduit decreased from 20% to 12% to 5% across eras (p<0.001).

This study highlights more proactive referral to PVR in rTOF and increasing use of tPVR. Further study is required to determine whether these evolving practices improve clinical outcomes.

Pulmonary valve replacement (PVR) is the standard treatment for pulmonary regurgitation in repaired tetralogy of Fallot (rTOF), yet the evolution of clinical profiles of patients referred for PVR and the shift from surgical (sPVR) to transcatheter (tPVR) PVR have not been well characterized. This study demonstrated that over time, patients with rTOF were increasingly referred for tPVR at younger ages, with fewer symptoms and less advanced right ventricular dilation and dysfunction, suggesting a shift toward a more proactive, minimally invasive approach during the study period. These findings highlight the evolving landscape of PVR referral and intervention patterns, providing insight into clinical criteria and procedural timing for patients with rTOF.