ABSTRACT
Introduction Recent years have seen a sharp decline in mortality in infants and children with major cardiac abnormalities, such that patients with radically altered circulation are surviving and living within the general population. These patients can then present the anaesthetist with a major challenge when they arrive at hospital for noncardiac surgery. The key to the safe management of these children is a clear understanding of the pathophysiology of the individual lesions allowing an appropriate selection of anaesthetic technique. In this group of patients, poor understanding of the lesion and inappropriate anaesthetic management can have dire consequences. If in doubt it is essential to contact a regional paediatric centre that undertakes cardiac surgery and discuss the individual problems and pitfalls with a consultant paediatric cardiac anaesthetist. As a representative example, this chapter discusses the management of adenotonsillectomy in a child with a total cavopulmonary anastamosis.
