Hypoplastic Left Heart Syndrome

or shunt) is created between one of the branches of the aorta and a pulmonary artery. This secures blood flow to the lungs. Upon completion of this procedure, circulation is such that the right ventricle pumps blood to the body through the newly created common blood vessel via the aorta and to the lungs via the B-T shunt. Survival following this stage of the operation is around 80%.

Stage Two: The bi-directional Glenn shunt:

This stage is usually performed at about six months of age, and serves to redirect HALF of the blood returning from the body to the lungs. This is accomplished by connecting the suprior vena cava (the blood vessel coming from the upper part of the body) to the pulmonary artery: no longer being necessary, the B-T shunt is removed. After this stage, circulation is such that blood coming from the upper extremities of the body bypasses the heart entirely and flows directly into the lungs. This eases some of the heart's burden, as it no longer has to circulate blood to both the lungs and body. The outcome for this procedure is very good, with a survival rate of around 100%.

Stage three: The Fontan procedure:

This last stage of the Norwood procedure is performed at the age of around eighteen months, and serves to redirect ALL of the blood returning from the body to the lungs. This is called a complete Fontan connection. This is accomplished by connecting the inferior vena cava (the blood vessel coming from the lower part of the body) to the pulmonary arteries. After this stage, circulation is such that the blood returning from the lower part of the body also bypasses the heart and flows directly to the lungs. As a result, poorly oxygenated blood no longer has to be pumped through the only functional ventricle- the right ventricle- because said blood now drains DIRECTLY into pulmonary circulation. After returning from the lungs, the oxygenated blood is pumped to the body through the aorta. The survival rate following this operation is approximately 95%.

Overall, the survival rate at five years of age following the full Norwood procedure is around 70-75%. Unfortunately, the long-term outlook for this palliative intervention has yet to be determined, because the oldest patients to have benefitted from these staged operations are only in their twenties. However, it cannot be argued that the preliminary results are outstanding. Almost all Norwood patients grow

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