In this section
The left side of the heart is very poorly formed and cannot
support the main circulation (round the body). The left ventricle
and aorta are abnormally small (hypoplastic). This is amongst the
most severe forms of heart defect.
Affected infants usually become severely symptomatic soon after
birth (as the ductus closes). This is one of the most serious
cardiac malformations and leads to death in the newborn period in
almost all affected babies, unless surgery or Heart Transplantation
can be offered.
Surgery consists of a 'Norwood' operation. This involves
connecting the origin of the pulmonary artery to the aorta, to
allow the right ventricle to pump blood to the main circulation and
a 'Shunt' operation. The atrial septum is removed to allow
blood to pass freely from the left atrium to the right side of the
heart. The 'Shunt' involves insertion of a tiny piece of artificial
tube (made from Goretex) between the right arm artery and the right
pulmonary artery, to maintain blood flow to the lungs. Another
option is to use a Goretex "Conduit" from RV to PA, instead of a
Other surgery is carried out later in childhood and may involve
several operations. The second operation is usually performed at
about three months and is a "Bi-directional Cavo-pulomonary Shunt"
(BCPS). This is an intermediate stage towards the final operation.
It involves channeling blood from the SVC to the pulmonary
The final operation is called the 'Fontan Operation', which is
carried out at three to four years (or later). This leads to blue
blood (low in oxygen) being channelled through the lungs, without
any 'pump' driving it. The right ventricle then pumps the red blood
(high in oxygen), round the body. This operation makes the child
'Pink' - but does not correct the original problem. Not all
affected children need to have a Fontan operation. If they are
well, with less drastic surgery, they may not have this 'Final' big
operation. Some children may be unsuitable for a Fontan operation
and alternative forms of treatment may be offered.