The Role of Cardiopulmonary Testing to Risk Stratify Tetralogy of Fallot Patients

Neonatal repair has completely changed the clinical history of patients with tetralogy of Fallot (ToF); however, these patients carry a significant risk of severe arrhythmias and sudden cardiac death in the long term. The exact mechanism for late sudden cardiac death is multifactorial and still not well defined, and the risk stratification for primary prophylaxis in these patients remains challenging. Cardiopulmonary exercise testing (CPET) is a well-established and safe method to assess cardiopulmonary function in children and adults with congenital heart disease. Several parameters obtained with CPET have been identified as potential prognostic of major adverse cardiovascular events in congenital heart disease. CPET is routinely used to assess functional capacity also in patients with ToF, and there is some evidence showing its usefulness in predicting the cardiac adverse events in patients with repaired ToF. Current guidelines recognize the importance of CPET in the evaluation and management of patients with ToF, but there is no clear consensus on which the CPET parameter or level of exercise intolerance, as measured by CPET, is truly predictive of an increased risk of arrhythmia and major adverse cardiovascular events in this population. Therefore, the aim of this narrative review is to describe the current evidence on the potential use of CPET in the risk stratification of patients with repaired ToF.