Implantable cardioverter-defibrillator therapy in adult patients with tetralogy of Fallot

AIMS: Adults with repaired tetralogy of Fallot (TOF) are at risk of sudden cardiac death (SCD). ESC and AHA guidelines suggest the use of implantable cardioverter defibrillators (ICDs) to protect from this. Few data are available on the benefits of these devices in this population, and there are no randomized studies. METHODS AND RESULTS: We analysed outcomes with respect to death, ICD therapy delivery, and complications for 20 patients with repaired TOF and 39 dilated cardiomyopathy (DCM) patients followed up at a UK teaching hospital. All TOF patients had clinical ventricular tachycardia (VT), electrophysiological study-inducible VT, or previous arrest due to tachyarrhythmia and received dual-chamber devices with individualized atrial detection algorithms. Tetralogy of Fallot patients were younger than DCM patients, but follow-up duration was not different between the groups. Tetralogy of Fallot patients were more likely to have experienced oversensing (45 vs. 13%; P < 0.02), inappropriate anti-tachycardia pacing delivery (20 vs. 2%; P < 0.05), and inappropriate cardioversion (25 vs. 4%; P = 0.06) than DCM patients and less likely to receive appropriate therapies than DCM patients. The death rate in TOF patients was significantly lower than that in DCM patients (5 vs. 21%; P < 0.05). CONCLUSION: Tetralogy of Fallot patients have a higher risk of inappropriate therapies and other complications yet a lower incidence of appropriate therapies from their ICD than DCM patients. Further research into identification of factors predicting SCD in TOF and the benefits of ICD implantation is essential given the potential complications of ICD implantation in young congenital heart disease patients.