Background: Studies have consistently shown that ventricular tachycardia (VT) and sudden cardiac death (SCD) complicate the long-term outcome after tetralogy of Fallot repair, yet the diagnostic and predictive value of electrophysiological testing in this population is uncertain.
Methods and results: A multicenter cohort of 252 patients with repaired tetralogy of Fallot undergoing programmed ventricular stimulation was followed up for 18.5+/-9.6 and 6.5+/-4.5 years after corrective surgery and electrophysiological testing, respectively. Clinical VT and/or SCD occurred in 24.6%. Sustained monomorphic VT and polymorphic VT were induced in 30.2% and 4.4%. Including polymorphic VT in the definition of inducibility improved sensitivity (66.1+/-6.0% versus 77.4+/-5.3%, P=0.0082) with a marginal reduction in specificity (81.6+/-2.8% versus 79.5+/-2.9%, P=0.0455). Positive and negative predictive values were 55.2+/-5.3% and 91.5+/-2.2%. Independent risk factors for inducibility were age at study > or =18 years (OR, 3.3), palpitations (OR, 2.8), prior palliative surgery (OR, 3.1), modified Lown criteria > or =2 (OR, 5.6), and cardiothoracic ratio > or =0.6 (OR, 3.3). Event-free survival rates in noninducible and inducible patients at 1, 5, 10, and 15 years were 97.9%, 92.8%, 89.3%, and 89.3% versus 79.4%, 62.6%, 58.7%, and 50.3%, respectively (P<0.0001). Both inducible monomorphic VT [relative risk (RR), 5.0; P=0.0002] and polymorphic VT (RR, 12.9; P<0.0001) predicted future clinical VT and SCD. In a multivariate analysis, inducible sustained VT was an independent risk factor for subsequent events (RR, 4.7; 95% CI, 1.2 to 18.5; P=0.0268).
Conclusions: Programmed ventricular stimulation is of diagnostic and prognostic value in risk stratifying patients with repaired tetralogy of Fallot. In this patient population, inducible sustained polymorphic VT should not be disregarded as nonspecific.