In this section
This is one of a group of very complex
defects where both Atriums connect to the same ventricle and/or one
ventricle is absent or very tiny. In most cases the infant develops
symptoms in the early weeks of life - either with 'Cyanosis' (Blueness of the skin) or with
breathlessness and failure to gain weight normally. Many affected
patients have associated defects in the heart or main arteries,
including such problems as Pulmonary Stenosis (PS), Pulmonary
Atresia, other valve abnormalities or Coarctation of the Aorta,
etc. Such problems may cause sever obstruction to blood flow to the
lungs or into the main circulation and may require urgent
The RV (usually very small) may be on the opposite side of the
heart to normal. This is similar in some respects to
the situation with "Corrected Transposition.
Treatment is aimed at repairing the associated
problems, where that can be done and performing staged surgery
(often with several operations being needed) to make the heart work
effectively. The basic defect cannot be repaired, but in many cases
the heart can be made to function sufficiently well to eliminate
cyanosis and to allow normal schooling and near normal
For many children the final operation
is called the 'Fontan Operation'. This leads to blue blood (low in
oxygen) being channelled through the lungs, without any 'pump'
driving it. The large 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.