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The unnatural history of tetralogy of Fallot: surgical repair is not as definitive as previously thought
  1. C van Doorn
  1. Correspondence to:
    Dr Carin van Doorn, Cardiac Unit, Great Ormond Street Hospital for Children, London WC1N 3JH, UK;

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Surgical correction of tetralogy of Fallot has proven very successful in the short term, although from longer term follow up it is increasingly apparent that many patients are haemodynamically compromised and their condition has not been cured

Tetralogy of Fallot (TOF) is a complex congenital cardiac abnormality representing approximately 10% of all cardiac malformations. It has a pathophysiology characterised by a ventricular septal defect (VSD) and right ventricular outflow tract obstruction (RVOTO) causing a right-to-left-shunt with low pulmonary blood flow and cyanosis. The extent of the RVOTO is highly variable and may include hypoplasia and dysplasia of the pulmonary valve as well as obstruction at the subvalvar and pulmonary artery level. The RVOTO is progressive, as is the compensatory right ventricular hypertrophy that adds to the obstruction. Without specific surgical management up to 35% of children will die within the first year of life, 50% by the third year, and only few will survive into adulthood.1 With this dismal natural history, interventions to change the outcome are much needed.

In 1944, TOF was the first congenital heart lesion to be palliated2 and 10 years later also the first complex cardiac lesion to undergo successful open repair.3 Over the last decades, advances in paediatric cardiology, surgery, and intensive care have transformed the outlook for patients with TOF. Currently in the UK, surgical correction carries a 30 day mortality of less then 3% (Central Cardiac Audit Database, personal communication, 2000–2001) and many operative survivors are expected to reach adulthood. Unfortunately, …

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