This defect is a form of Tetralogy of Fallot in which there is
complete obstruction of the Pulmonary Artery resulting in total
diversion of blood from the right ventricle into the aorta.
Survival depends on the ductus remaining open in the early days of
life (in order for blood to reach the lungs), or on the presence of
other connecting blood vessels between the Aorta and the Pulmonary
Arteries in the lungs (Collaterals). Most babies will need a
'Shunt' operation during infancy, involving insertion of a tiny
piece of artificial tube (made from Goretex) between the Aorta, or
a branch (usually one of the arm arteries), and one of the branch
Pulmonary Arteries. Complete Repair is carried out at two to three
years. Complete repair for
Pulmonary Atresia is usually carried out after the first year of
life, though sometimes it may be performed earlier.
Complete repair for Pulmonary Atresia usually necessitates the
insertion of a 'Conduit', which is a tube containing a valve,
placed to connect the right ventricle to the pulmonary
artery. The VSD is also closed and the pulmonary arteries may
require enlargement with one or more patches.
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