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Tetralogy of Fallot (ToF) occurs in approximately 1 in 3600 live births and accounts for 3.5% of infants born with congenital heart disease. Surgical repair was first introduced in the 1950s and there is now a large population of adults with repaired tetralogy. Many of the short term aspects of management have been resolved, although whether symptomatic neonates should undergo primary repair or first be palliated remains debatable. In 2001, independently validated data pooled from all 13 centres performing cardiac surgery in the United Kingdom indicated a 97% survival one year after operation.1 Other reports indicate that of patients alive 30 days after operation there is a 98% 20 year survival, and of those operated on as children 30 year survival is above 90%.2
This has focused attention on long-term follow up as many patients with repaired ToF are now middle-aged. Given that this population includes patients well beyond adolescence they have also outgrown the term “grown-up congenital heart disease” (GUCH) which could be considered patronising. They have adult congenital heart disease and will need specialised care into old age.
The diagnosis of ToF can now be made antenatally with particular benefit for the fetus with severe right ventricular outflow tract obstruction (RVOTO). Postnatally the need for a prostaglandin infusion to maintain ductal patency can be anticipated and hence potential hypoxic cerebral damage can be avoided.
Anterior craniocaudal or cephalad deviation of the insertion of the muscular outlet septum together with hypertrophy of trabeculations on the infundibular free wall constitute the essential features of ToF. Instead of nestling between the anterior and posterior limbs of the trabecula septomarginalis, the outlet septum is displaced and typically fuses with the anterior limb. This brings the aorta over the ventricular septum so that it has a biventricular origin and accounts for …