Indications
The Fontan procedure has more recently been used in pediatric situations where an infant only has a single effective ventricle, either due to heart valve defects (e.g. tricuspid or pulmonary atresia) or an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome, hypoplastic right heart syndrome), or has complex congenital heart disease where a bi-ventricular repair is impossible or inadvisable.
Children with hypoplastic left heart syndrome have a single effective ventricle supplying blood to the lungs and the body (either from birth or after an initial surgery e.g. Norwood procedure). They are delicately balanced between inadequate blood supply to the lungs (causing cyanosis) and oversupply to the lungs (causing heart failure). In addition, the single ventricle is doing nearly twice the expected amount of work (because it has to pump blood for both lungs and body). As a result, these children can have trouble gaining weight, and are also vulnerable to decompensation in the face of otherwise minor illnesses (even a common cold). Sometimes medications (e.g. diuretics) can help them through this stage.
Therefore, when either they are large enough, and if the pressure in the pulmonary arteries is low enough, these children are referred for the Fontan procedure commonly after 2 years of life.
Read more about this topic: Fontan Procedure
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