Evaluation and Management of Sudden Death Risk in Repaired Tetralogy of Fallot

Although substantial progress has been made to prevent sudden cardiac death in repaired tetralogy of Fallot patients, ventricular arrhythmia and sudden death continue to be major causes of morbidity and mortality in these patients. Greater survival in contemporary cohorts has been attributed to enhanced surgical techniques, more effective management of heart failure, and increased efforts in risk stratification and management of ventricular arrhythmias. More recently, our understanding of predictive risk factors has evolved into personalized risk prediction tools that rely on comprehensive demographic, imaging, functional, and electrophysiological data. However, the universal applicability of these different scoring systems is limited due to differences between study cohorts, types of anatomic repair, imaging modalities, and disease complexity. Noninvasive risk stratification is critical to identify those who may derive benefit from catheter ablation or cardioverter defibrillator implantation for primary prevention. Ultimately, assessment and risk stratification by a multidisciplinary team is crucial to analyze the various complex factors for every individual patient and discuss further options with patients and their families.