ABSTRACT
Tetralogy of Fallot (TOF) is one of the most frequently encountered congenital cardiac surgical lesions, occurring in 3 of every 10 000 live births. It is the commonest cause of cyanotic cardiac disease in patients beyond the neonatal age, and accounts for up to one-tenth of all congenital cardiac lesions. TOF is, therefore, an important entity for all paediatric cardiac surgeons. In fact, TOF was the first cyanotic lesion to be formally described, and some of the initial palliative and definitive operations for congenital heart disease were performed for TOF. Probably more is known about TOF than about any other complex cardiac malformation and, consequently, the lesion has served as a model for the natural history of cyanotic congenital heart disease and our ability to alter that history with surgery. Our understanding of cardiac physiology, myocardial protection, cardiopulmonary bypass (CPB), developmental anatomy, molecular biology, genomics, and other areas has been enhanced by the study of TOF. Finally, TOF is a potentially lethal lesion without treatment, but one that now has a quite favorable natural history when an appropriate surgical strategy is employed.
