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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. Most babies are very ill in the early days of life and need urgent surgery to survive. The first stage of surgery is called a 'Norwood' operation.
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The Norwood operation 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, involving 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. Later in childhood it may be possible to carry out a modified Fontan operation.
Another option that is sometimes used, involves a Goretex tube (called a "Conduit") from the RV to the pulmonary artery instead of the "Shunt".
A second operation follows after about three months and is called a "Cavo-Pulmonary Shunt". At a later stage (around three to four years old) a Fontan Operation may be performed.
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.
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