The classic lesions of tetralogy of Fallot (TOF) include pulmonary stenosis, ventricular septal defect (VSD), overriding aorta, and right ventricular hypertrophy. The hemodynamically significant lesions are the VSD and pulmonary stenosis that results in right ventricular outflow tract (RVOT) obstruction. Originally, a two-stage repair was performed for repair of TOF. First pulmonary blood flow was increased with systemic-to-pulmonary artery shunts (classic and modified Blalock–Taussig shunt, Waterston shunt, or Potts shunt) followed by a complete repair when the child was older. Complications associated with this strategy include pulmonary hypertension and congestive heart failure from the augmented pulmonary blood flow and stenosis of the pulmonary artery at the anastomotic site.

The current treatment for TOF with pulmonary stenosis is a primary single-stage repair in the first year of life, usually at 3–6 months of age. This obviates the need for multiple surgeries and prevents the associated complications. Definitive repair consists of closing the VSD and relieving the RVOT obstruction. The older surgical technique relieved the RVOT obstruction using a right ventricular free-wall incision with insertion of an infundibular patch graft or homograft to enlarge the outflow tract and also included a repair of the VSD with a GORE-TEX patch (Figs. 29.1 and 29.2). The pulmonary homograft replaced the pulmonary valve and proximal pulmonary artery. This repair is no longer performed due to the frequent complication of dilatation of the RVOT homograft leading to progressive pulmonary valve regurgitation requiring valve replacement and the development of right ventricular failure (Fig. 29.3).