Pulmonary Valve Replacement in Repaired Tetralogy of Fallot

Quick Takes

  • Pulmonary valve replacement was associated with a lower risk of the primary outcome of death and sustained ventricular tachycardia in a cohort of patients with repaired tetralogy of Fallot.
  • Patients with more advanced disease as demonstrated by RV end-systolic volume >80 mL/m2 were the most likely to benefit from pulmonary valve replacement.

Study Questions:

What is the impact of pulmonary valve replacement (PVR) on survival and sustained ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot (rTOF)?


An observational cohort study using propensity-score matching was performed using INDICATOR (International Multicenter TOF Registry). The primary outcome was time to the earliest occurrence of death or sustained VT.


Among 1,143 patients with rTOF (age 27 ± 14 years, 47% PVR, follow-up 8.3 ± 5.2 years), the primary outcome occurred in 82 patients. The adjusted hazard ratio (HR) for the primary outcome for PVR vs. no-PVR (matched cohort n = 524) was 0.41 (95% confidence interval [CI], 0.21-0.81; multivariable model p = 0.010). In patients with right ventricular end-systolic volume index (RVESVi) >80 mL/m2, PVR was associated with a lower primary outcome risk (HR, 0.32; 95% CI, 0.16-0.62; p < 0.001). There was no association between PVR and the primary outcome in patients with RVESVi ≤80 mL/m2 (HR, 0.86; 95% CI, 0.38-1.92; p = 0.70). There was no impact of PVR on the secondary outcome of advanced heart failure, nonsustained VT, or sustained supraventricular tachycardia.


The authors conclude that in patients compared with rTOF patients who did not receive PVR, propensity-score matched individuals receiving PVR had lower risk of a composite endpoint of death or sustained VT.


This is the first study to demonstrate survival benefit for PVR in individuals for rTOF. There are a fair number of subtleties in the paper, and it should be read in detail by those considering patients for PVR. The group who appears to have the most benefit from PVR is patients with more advanced RV volume as evidenced by larger indexed RVESV (>80 mL/m2). Interestingly, PVR did not positively impact the risk of the secondary endpoints of the study, which were heart failure, nonsustained VT, and atrial tachyarrhythmias. Additionally, the study does not justify a prophylactic approach of replacing pulmonary valves in all patients with TOF and pulmonary valve regurgitation as there was no benefit for patients who did not fulfill predefined criteria, those with a low propensity score, or those with RVESVs below 80 mL/m2. This is an important paper, which will contribute significantly to future guidelines related to indications for PVR.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Prevention, Valvular Heart Disease, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Quality Improvement, Acute Heart Failure

Keywords: Arrhythmias, Cardiac, Heart Defects, Congenital, Heart Failure, Pulmonary Valve Insufficiency, Risk, Secondary Prevention, Tachycardia, Paroxysmal, Tachycardia, Ventricular, Tachycardia, Supraventricular, Tetralogy of Fallot

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