Ventricular Arrhythmia and Life-Threatening Events in Patients With Repaired Tetralogy of Fallot

Abstract

Risk stratification for malignant arrhythmias and risk of sudden cardiac death in tetralogy of Fallot (TOF) remains challenging. We aimed to ascertain factors associated with life-threatening arrhythmic events. A multicenter retrospective case-control study including 72 TOF patients with documented cardiac arrest and/or sustained ventricular tachycardia, compared with 216 controls matched for era of surgery. The mean age at event in the cases was 27.3 ± 12.5 years. The majority (57%) presented with sustained ventricular tachycardia. Fatal events occurred in 9. Random forest analysis and a decision tree demonstrated surgical era specific risk factors (< vs ≥ 1980). For both eras, arrhythmic symptoms and left ventricular dysfunction were strongly associated with malignant arrhythmias. In addition, right ventricular dysfunction and age at repair ≥ 6.5 years preceded by a shunt were associated with a higher risk group in the early era, whereas a trans-annular patch type repair was associated with a lower risk group in the recent era. For the moderate and high-risk groups, the decision tree showed a sensitivity of 88.4% and specificity of 68.1%. An "importance factor" was calculated for each predictor, creating a risk score and 4 risk categories. In conclusions, this risk stratification scheme, based on clinical history and noninvasive testing, allows categorization of TOF patients at high risk of malignant arrhythmia. A multicenter prospective evaluation of the accuracy of this scoring system is now being planned.