In this section
Affected children are blue ('cyanosed') because their arterial
(red) and venous (blue) blood mix together. Such patients have one
of several cardiac abnormalities that mean that the lung
circulation cannot work independently from the body circulation.
These problems may require a Fontan operation.
The main defects in this category are:
In all these situations, where a Fontan operation is
recommended, the problems in the heart make it impossible or
extremely hazardous to 'repair' the defect in a way that would
allow two ventricles to support the two circulations
The aim of the operation is to improve oxygen levels, by
redirecting venous (blue) blood directly to the lungs. This
produces two separate circulations and a decreased workload on the
heart, but means that only one ventricle functions as the 'pump'
(driving blood through the body circulation first and then into the
lung circulation afterwards, before it returns to the ventricle to
This operation works best if:
Many children or adults who are candidates for this type of
surgery will have had previous surgery and / or catheter
procedures. Before the Fontan operation can be performed they will
need comprehensive review with several tests, probably including an
up-to-date catheter test.
After the results have been discussed by the doctors and
surgeons the patient / family will need to see the surgeon to learn
more about the operation and what benefits it may achieve.
It is important that the family know that the Fontan operation
is not a cure and that patients after this surgery will need
The Fontan operation can be done in several different ways. At
the Royal Children's Hospital there have been three main ways of
ensuring as much blood flow to the lungs as possible.
1. Extracardiac Fontan
2. Lateral tunnel Fontan
3. Atriopulmonary Connection (now rarely used)
One or two days before surgery the patient will need to come to
the cardiac ward, for preliminary tests.
While there he or she will have:
The family will be visited by:
Nurses from this ward take the family to visit the Intensive
Care Unit, which introduces them to the area that the patient will
return to after the operation.
The anaesthetic and operation will take about 5 hours or
After the operation the patient will be in the intensive care
Because there is no separate ventricle to pump blood to the
lungs it is important that blood flow to the pulmonary arteries is
increased in every possible way! Such as...
The intensive care team will make sure that the patient is
breathing on his / her own as soon as possible (usually less than
12 hours). This will also assist venous return, as the need for an
artificial ventilator (positive pressure ventilation) makes it more
difficult for blood to return to the heart and ultimately, into the
lungs and out again.
Heart function is also optimised by administering adequate fluid
or blood to make sure that the pressure in the veins carrying blood
back to the heart, and from there to the lungs, is maintained high
enough to promote good blood flow through the lungs.
While the body is trying to adjust to this the patient may be
very prone to leakage of fluid into the sacs around the lungs or
elsewhere, which can cause ëpleural effusionsí (fluid around the
lungs), ascites (fluid in the abdomen) and liver congestion /
Therefore the nurses are carefully noting the amount of fluid
that is lost and is taken in. The absolute minimum of fluid intake
is given during this early stage to try to minimise these
To avoid this:
To avoid this: