Risk stratification in surgically repaired tetralogy of Fallot

Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients. Indeed, sudden death of presumed arrhythmic etiology is the most frequent mode of demise in patients with surgically repaired tetralogy of Fallot, often in early-to-mid adulthood. Nevertheless, the overall annual incidence of sudden death is actually low. Therein lies the major challenge of risk stratification. This review highlights some of the complexities involved in risk stratifying patients with tetralogy of Fallot, underscoring the importance of avoiding oversimplification by means of a rigid algorithm that dictates therapy. To complement sound clinical judgment, a quantitative probabilistic approach is presented, which considers the body of literature from primary prevention ICD trials, risk factors identified by observational studies, the value of programmed ventricular stimulation and insights gleaned from studying ICD recipients with tetralogy of Fallot.