Absence of any connection between the right atrium and the right
ventricle leads to blood being diverted from the right atrium to
the left atrium. The right ventricle (RV) is usually small
(Hypoplastic) Survival depends on an associated VSD (often quite
small), in order for blood to reach the lungs, or on the ductus
remaining open in the early days of life. 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.
Go to Normal Heart
At a second operation some blood from veins in the upper part of
the body may be connected directly to the lung arteries
(BCPC)
Definitive surgery is usually delayed until the age of at least two
years. It involves the so called Fontan operation. This involves
connecting the veins from the main circulation (SVC & IVC)
directly to the pulmonary arteries. Blue blood is thus directed
into the lungs rather than to the left atrium. A patch is placed to
prevent blood passing from the RA to the LA - though sometimes a
small hole (a 'Fenestration') is deliberately left.
Bidirectional Cavo Pulmonary
Connection (BCPC)
A Cavo-Pulmonary shunt involves connecting the main vein from
the upper circulation (the SVC) to the right pulmonary artery
(RPA), in order to direct the blood from the upper part of the main
circulation into the lung circulation. The main vein from the lower
circulation (the IVC) still carries blood into the right atrium.
This operation will often improve cyanosis (blueness), but does not
eliminate it completely. Many children will go on to have a Fontan
operation later, when the IVC blood will also be channelled into
the pulmonary arteries and the cyanosis will be completely, or
almost completely, relieved.
Fontan operation
The Fontan operation involves connecting the veins from the main
circulation (SVC & IVC) directly to the pulmonary arteries.
Blue blood is thus directed into the lungs rather than to the left
atrium. A patch is placed to prevent blood passing from the RA to
the LA - though sometimes a small hole (a 'Fenestration') is
deliberately left.
