The considerable progress, performed for more than 30 years, in paediatric and interventional cardiology, imaging, surgery, anaesthesia and critical care in the congenital heart diseases allowed the survival the adulthood of more than 85 % of the affected children. The univentricular repair in total cavopulmonary connection or Fontan procedure, are realized in three stages, now, before the age of 5 years, with a different physiology after each stage. This point makes anaesthetic care more complicated for a non-cardiac surgery. The precise knowledge of the physiology of the "Fontan" is necessary before proceeding with anaesthesia. It allows to anticipate the pitfalls and to define specific strategies to be applied.
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