Temporal Changes in the Timing of Pulmonary Valve Replacement and Impact on Outcomes in Tetralogy of Fallot

Abstract To assess temporal changes in the timing of pulmonary valve replacement (PVR) among patients with tetralogy of Fallot and to determine whether temporal shifts toward earlier PVR were accompanied by differences in ventricular volumes, ventricular function and exercise capacity after PVR. The timing of PVR was evaluated in a national, retrospective cohort using the Aalen-Johansen method to estimate cumulative incidence while accounting for death as competing risk. Trends in ventricular volumes, ventricular function and exercise capacity were assessed in patients with prior PVR, who underwent cardiac imaging and exercise testing in a cross-sectional study using robust linear regression. Among 463 potential candidates for PVR in the retrospective cohort, estimated time from repair to PVR was 34 years, 22 years and 16 years in patients born in 1940–1976, 1977–1999, and 2000–2021, respectively ( p < 0.001). In total, 153 patients with prior PVR born between 1942 and 2009 were examined in the cross-sectional study. Estimated right ventricular end-systolic volume index was lower by 5.43 mL/m 2 per decade ( p = 0.02), estimated right ventricular ejection fraction increased by 2.08% per decade ( p = 0.01). Left ventricular volumes, left ventricular ejection fraction and exercise capacity was not more favorable in later eras. The timing of PVR has shifted toward earlier intervention over time. The temporal shift toward earlier PVR coincided with lower right ventricular end-systolic volume index and higher right ventricular ejection fraction after PVR, whereas left ventricular parameters and exercise capacity showed no clear temporal change. These findings are compatible with, but do not prove, a beneficial effect of earlier intervention. Graphical Abstract Overview of temporal changes in the timing of pulmonary valve replacement and impact on outcomes. The median time from repair to pulmonary valve replacement for each birth era was based on a nationwide cohort of patients with tetralogy of Fallot and estimated using the cumulative incidence function. The temporal trends in ventricular volumes, ventricular function and exercise capacity were estimated based on the results from patients who underwent cardiac magnetic resonance imaging, transthoracic echocardiography and cardiopulmonary exercise testing as part of a national multicentre cross-sectional study and estimated using robust regression methods

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Journal
Pediatric Cardiology
Published
2026-05-18
DOI
https://doi.org/10.1007/s00246-026-04294-0
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

Temporal Changes in the Timing of Pulmonary Valve Replacement and Impact on Outcomes in Tetralogy of Fallot

Christian Jøns, Troels Højsgaard Jørgensen, Kim Munk, Henrik Nissen et al.
Pediatric Cardiology
Congenital Heart Disease Studies
article

Temporal Changes in the Timing of Pulmonary Valve Replacement and Impact on Outcomes in Tetralogy of Fallot

Christian Jøns, Troels Højsgaard Jørgensen, Kim Munk, Henrik Nissen, Hans Gustav Hørsted Thyregod, Mathis Gröning, Ulrik Markus Mortensen, Annette Schophuus Jensen, Morten Holdgaard Smerup, Morten Engholm, D Nerst, Dorte Guldbrand Nielsen, Klaus Juul, Helle Andersen, E. Søndergaard, M R Schmidt, Ole De Backer, Niels Vejlstrup, Jesper Bjerre
article en

Abstract

Abstract To assess temporal changes in the timing of pulmonary valve replacement (PVR) among patients with tetralogy of Fallot and to determine whether temporal shifts toward earlier PVR were accompanied by differences in ventricular volumes, ventricular function and exercise capacity after PVR. The timing of PVR was evaluated in a national, retrospective cohort using the Aalen-Johansen method to estimate cumulative incidence while accounting for death as competing risk. Trends in ventricular volumes, ventricular function and exercise capacity were assessed in patients with prior PVR, who underwent cardiac imaging and exercise testing in a cross-sectional study using robust linear regression. Among 463 potential candidates for PVR in the retrospective cohort, estimated time from repair to PVR was 34 years, 22 years and 16 years in patients born in 1940–1976, 1977–1999, and 2000–2021, respectively ( p < 0.001). In total, 153 patients with prior PVR born between 1942 and 2009 were examined in the cross-sectional study. Estimated right ventricular end-systolic volume index was lower by 5.43 mL/m 2 per decade ( p = 0.02), estimated right ventricular ejection fraction increased by 2.08% per decade ( p = 0.01). Left ventricular volumes, left ventricular ejection fraction and exercise capacity was not more favorable in later eras. The timing of PVR has shifted toward earlier intervention over time. The temporal shift toward earlier PVR coincided with lower right ventricular end-systolic volume index and higher right ventricular ejection fraction after PVR, whereas left ventricular parameters and exercise capacity showed no clear temporal change. These findings are compatible with, but do not prove, a beneficial effect of earlier intervention. Graphical Abstract Overview of temporal changes in the timing of pulmonary valve replacement and impact on outcomes. The median time from repair to pulmonary valve replacement for each birth era was based on a nationwide cohort of patients with tetralogy of Fallot and estimated using the cumulative incidence function. The temporal trends in ventricular volumes, ventricular function and exercise capacity were estimated based on the results from patients who underwent cardiac magnetic resonance imaging, transthoracic echocardiography and cardiopulmonary exercise testing as part of a national multicentre cross-sectional study and estimated using robust regression methods

Pediatric Cardiology
Odense University Hospital (DK), Aarhus University Hospital (DK), Copenhagen University Hospital (DK), Rigshospitalet (DK), Zealand University Hospital (DK)
Good health and well-being
Openalex Percentile: Top 6%
Congenital Heart Disease Studies
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