Tetralogy of Fallot (TOF)

Tetralogy of FallotTetralogy of Fallot (TOF) is the most common cyanotic cardiac defect. It is the result of malalignment of the ventricular septum with the infundibular septum, thereby resulting in a malalignment VSDVentricular septal defect (VSD), aortic overrideAortic override, hypoplastic or obstructed right ventricular outflow tract and right ventricular hypertrophy. Depending on the extent of right ventricular outflow obstruction, patients present with intermittent cyanosis (spells). If tolerated or adequately managed medically, patients are repaired electively between 3 to 6 months of age. Earlier repair (e.g. as a neonate) may be necessary in symptomatic patients. Primary repair is the preferred approach, consisting of VSDVentricular septal defect (VSD) closure, right ventricular muscle bundle resection, pulmonary valvotomy or transannular patching and patching main/branch pulmonary arteries. Management of the right ventricular outflow tract is a key component of repair, aiming at adequate relief of obstruction and sparing the native pulmonary valve annulus when possible. Outcome is generally good with low surgical mortality and good long-term survivalSurvival. However, reinterventions on the right ventricular outflow tract are common and most patients with transannular patchTransannular patch will comeInfundibulotomy to need a pulmonary valve replacementValve replacement laterPulmonary valve insufficiency in life.