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Defibrillator Therapy in Patients With Tetralogy of Fallot
Quick Takes
- ICD placement for both primary and secondary prevention is associated with high annualized rates of appropriate ICD therapies.
- ICD-related complications are common in patients with tetralogy of Fallot.
- QRS fragmentation (defined as ≥3 R-waves/notches in the R/S complex in ≥2 contiguous leads) is strongly associated with appropriate ICD therapies.
Study Questions:
What are the outcomes for patients with tetralogy of Fallot (TOF) receiving implantable cardioverter defibrillators (ICDs) using a national registry?
Methods:
This study used the Nationwide French Registry, which included all TOF patients with an ICD beginning in 2010. The primary endpoint was time to the first appropriate ICD shock. Secondary outcomes included ICD complications including inappropriate shocks as well as heart transplantation and death.
Results:
A total of 165 patients (mean age 42 ± 13.3 years, 70.1% men) were included from 40 centers, of which 104 patients (63%) received ICDs for secondary prevention indications. The median follow-up was 6.8 years, during which 78 patients (47.3%) received at least one appropriate ICD therapy. The overall annual incidence of appropriate shock was 10.5%, with a rate of 7.1% for primary prevention and 12.5% for secondary prevention (p = 0.03). ICD complications occurred in 71 (43%) patients, with inappropriate shocks in 42 (25.5%) and lead dysfunction in 36 (21.8%) patients. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47; 95% confidence interval, 1.19-10.11).
Conclusions:
The authors concluded that patients with TOF and ICD experience high rates of appropriate therapies, including those implanted for primary prevention. TOF patients have a high rate of device-related complications. A combination of criteria including QRS fragmentation may improve risk stratification.
Perspective:
The optimal indications for ICD placement in patients with TOF remain unclear. This study used a nationwide registry in France to assess outcomes with ICD placement in patients with TOF receiving ICD for both primary and secondary prevention. There was a relatively high rate of appropriate shocks for both the primary prevention and secondary prevention groups. As has been seen in other studies of device therapy in patients with congenital heart disease, there is a large burden of device-related complications, particularly inappropriate shocks and lead-related complications. This study suggests a role for the use of QRS fragmentation as an additional risk factor, which is an interesting finding that suggests a need for further study. Interestingly, the study showed that patients with left ventricular ejection fraction <35% and no other risk factors had appropriate shocks, suggesting a need for caution in applying standard adult criteria for ICD placement in this patient population.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Transplant, Interventions and Structural Heart Disease
Keywords: Arrhythmias, Cardiac, Defibrillators, Implantable, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Primary Prevention, Risk Assessment, Risk Factors, Secondary Prevention, Shock, Tetralogy of Fallot
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