In this section
In 'Double Outlet Right Ventricle' (DORV) the two Great Arteries
(Aorta and Pulmonary Artery) both originate from the right
ventricle and blood from the left ventricle passes across a VSD
into the RV to reach the great arteries. The lung circulation is
often exposed to very high pressure and increased blood flow (as
with a large VSD).
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. There are many
different varieties of this abnormality. 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 severe obstruction
to blood flow to the lungs or into the main circulation and may
require urgent surgery.
Treatment, which is surgical, is aimed at repairing the defect
and any associated problems, where that can be done. In many cases
the heart can be made to function sufficiently well to eliminate
symptoms and to allow normal schooling and near normal activities.
Most cases are amenable to 'Complete Repair', though more than one
operation may be needed.
In a minority of the more complex cases complete repair may not
be possible. In such cases treatment aims to make the heart work as
effectively as possible. This may involve several operations.
For some 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 ventricles then pump the red blood (high in oxygen), round the
body. This operation makes the child 'Pink' - but does not correct
the original problem.