Pulmonary Valve Replacement and Ventricular Arrhythmias in TOF

Quick Takes

  • In this registry-based study of adults with tetralogy of Fallot (TOF) with implantable cardioverter-defibrillators (ICDs), the burden of appropriate ICD therapies was significantly reduced after pulmonary valve replacement (PVR).
  • PVR prior to ICD placement was independently associated with a lower risk of appropriate ICD therapy in primary prevention patients.

Study Questions:

What is the impact of pulmonary valve replacement (PVR) on ventricular arrhythmias in a population of tetralogy of Fallot (TOF) patients with continuous cardiac monitoring by implantable cardioverter-defibrillators (ICDs)?


Data from the DAI-T4F (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator) were utilized to study all TOF patients with an ICD since 2000. Survival data with recurrent events were used to compare the burden of appropriate ICD therapies before and after PVR in patients who underwent PVR over the study period.


A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% male) were included from 40 centers. Over a median follow-up period of 6.8 years, 26 patients (15.8%) underwent PVR. Among those patients, 18 (69.2%) experienced at least one appropriate ICD therapy. When comparing all ICD therapies delivered before (n = 62) and after (n = 16) PVR, the burden of appropriate ICD therapies was significantly lower after PVR (hazard ratio [HR], 0.21; 95% confidence interval [CI], 0.08-0.56; p = 0.002). Respective ICD therapy rates per 100 person-years were 44.0 before and 13.2 after PVR (p < 0.001). In the overall cohort, PVR before ICD implantation was also independently associated with a lower risk of appropriate ICD therapy in primary prevention patients (HR, 0.29; 95% CI, 0.10-0.89; p = 0.031).


The authors concluded that in this cohort of high-risk TOF patients implanted with an ICD, the burden of appropriate ICD therapies was significantly reduced after PVR, suggesting the importance of considering ventricular arrhythmias in the overall decision-making process for timing of PVR.


PVR has been known to improve symptoms and lead to reduction of right ventricular volumes in patients with repaired TOF and pulmonary valve regurgitation. There has been little evidence thus far to suggest that PVR has a significant impact on the rate of ventricular arrhythmias post-procedure. This study made use of a French national registry of patients with TOF and ICDs and showed a significant decrease in appropriate ICD therapies. This study also provides additional evidence as to the role of QRS fragmentation as a risk for ventricular arrhythmias, as it was the only independent predictor of appropriate ICD therapies in multivariable analysis. A general principle in the care of adult congenital patients has been to correct hemodynamic issues to minimize arrhythmia risk. This study provides additional evidence supporting this approach.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Prevention, Valvular Heart Disease, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, Interventions and Structural Heart Disease

Keywords: Arrhythmias, Cardiac, Cardiac Surgical Procedures, Defibrillators, Implantable, Heart Defects, Congenital, Hemodynamics, Primary Prevention, Pulmonary Valve Insufficiency, Tetralogy of Fallot

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