83 © Springer Nature Switzerland AG 2019 J. Rhodes et al. (eds.), Exercise Physiology for the Pediatric and Congenital Cardiologist, https://doi.org/10.1007/978-3-030-16818-6_14 Repaired Tetralogy of Fallot Jonathan Rhodes, Alexander R. Opotowsky, and Mark E. Alexander Basic Anatomy The characteristic anatomy of anterior-superior displacement of the conal septum, resulting in a malalignment ventricular septal defect and a vari- able degree of subvalvar, valvar, and supravalvar pulmonary stenosis, is well known. Tetralogy of Fallot in fact represents a spectrum of diseases with multiple important anatomic variations. Furthermore, there have been substantial changes in surgical management in the 60 years that have elapsed since the initial anatomic repair in 1958. The details of the anatomy and management are needed to place any exercise result in context. Specifc details that should be considered include (but are in no way limited to) age at complete repair, extent of an infundibulotomy or use of a conduit, prior shunts, and subsequent surgical and catheterization management. Hemodynamics The hemodynamics of patients with repaired tetral- ogy of Fallot (rTOF) is often characterized by an incompetent pulmonary valve and variable degrees of residual pulmonic stenoses at the subvalvar, val- var, supravalvar, branch, or peripheral pulmonary arterial levels. These anatomic abnormalities can affect the cardiopulmonary response to exercise and produce alterations in cardiopulmonary exer- cise testing (CPET) data that refect interesting physiology with important clinical implications. Exercise Capacity Numerous studies have documented that the exercise capacity of patients with rTOF is often depressed. This is particularly notable in adults, J. Rhodes (*) Department of Pediatrics, Harvard Medical School, Boston, MA, USA Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA e-mail: jonathan.rhodes@cardio.chboston.org A. R. Opotowsky Department of Pediatrics, Harvard Medical School, Boston, MA, USA Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women’s Hospital, Boston, MA, USA Exercise Physiology Laboratory, Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA e-mail: alexander.opotowsky@cardio.chboston.org M. E. Alexander Department of Pediatrics, Harvard Medical School, Boston, MA, USA Exercise Physiology, Arrhythmia Service, Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA e-mail: mark.alexander@cardio.chboston.org 14