There are a number of other conditions where a child may need to go down the Fontan treatment route.

There are a number of congenital (something a baby is born with) heart conditions where the treatment plan is not always clear at the first diagnosis. Some children are born with a heart on the borderline between having two pumps or just one. If possible, cardiac doctors will plan to create a two-pump heart: this may mean creating a wall within the heart or creating a tube to take blood from one pump to the lungs.

It may be that surgeons will do a small amount of surgery to keep the child safe and give them an ability to grow and then wait to see if the heart shows it can support a two-pump circulation.

In some cases as the child grows it becomes clear that a two-pump heart is not possible and doctors will choose to follow the Fontan treatment path. (See The Fontan Procedure).

Conditions which might be borderline are described below.

Ebstein’s Anomaly

This condition means that there are problems with the tricuspid valve, the valve between the atrium and ventricle on the right side of the heart. It has not formed properly and may well have caused the right atrium (collecting chamber) to be bigger than normal and the right ventricle (the pumping chamber) to be much smaller than normal. The surgeons will always try to keep the valve and chambers working as normal but sometimes the ventricle is not big enough and they will offer a treatment path similar to that offered for Tricuspid Atresia.

It may be that surgeons will do a small amount of surgery to keep the child safe and give them an ability to grow and then wait to see if the heart shows it can support a two-pump circulation.

In some cases as the child grows it becomes clear that a two-pump heart is not possible and doctors will choose to follow the Fontan treatment path. (See The Fontan Procedure (Stage Three) for all single ventricle conditions).

Conditions which might be borderline are described below.


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Atrioventricular Septal Defect (AVSD)

This condition means that there is a problem with the wall within the heart that separates the right and left sides of the heart. The septal defect (hole) runs from the atria (collecting chambers) in the lower part of the heart.

Surgeons can often create a wall so that the heart has four chambers as it should, but sometimes the defect is too big to repair and a surgical pathway similar to that for a Univentricular Heart will be planned.


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One and a Half Ventricles

Some children are born with a heart condition where there is a difference in the size of the ventricles (heart pumps) from normal and there is a large hole where the wall should be between the two pumps. Where possible, the surgeon will close the hole and create a two-pump heart but this is only possible if each pump is big enough to do its job properly. Having offered treatment that will allow the baby to grow, an assessment will be made of the ventricle size and the ability of the ventricle to pump. Where one of the two pumps is not adequate in size, the treatment path will be to a Fontan circulation. These hearts have the advantage of having one pump that is stronger than normal because it is supported by the action of the other small ventricle, hence the one-and-a-half-pump heart.

Updated: June 2017

Review due: June 2019

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