Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease, with an incidence of 4 in 10 000 live births.1 Long-term survival after simple TOF repair is excellent, and the number of adults with repaired TOF is therefore increasing.2,3 However, surgical scarring predisposes to the occurrence of ventricular arrhythmias even decades after repair, and sudden cardiac death (SCD) is an important cause of death in this population.4–7
The implantable cardioverter defibrillator (ICD) is highly effective in preventing arrhythmic death, and patients with TOF represent the largest subgroup of ICD recipients among patients with congenital heart disease.8–10 Selection of candidates for primary prevention ICD is challenging, and current risk stratification is based on the presence of multiple (≥2) risk factors including left ventricular dysfunction, nonsustained ventricular tachycardia, QRS duration ≥180 ms, or inducible ventricular arrhythmia on programmed ventricular stimulation (PVS).11–14 However, long-term follow-up data in patients with TOF and ICDs remain scarce. The largest cohort published >10 years ago included 121 patients with a median follow-up of 3.7 years.15 The risk/benefit balance of ICD in patients with TOF needs to be further evaluated because ICD is known to be associated with significant adverse events, especially in younger populations.
Reference link- https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.120.046745
