Hypoplastic Left Heart Syndrome


The left ventricle and aorta are abnormally small an unable to support the circulation to the body.  Three stages of surgery are required.  The first stage called the Norwood operation reconstructs the heart to allow the right ventricle to pump blood to the body, and a BT shunt to maintain blood flow to the lungs.  Later in infancy, your child will outgrow the BT Shunt and require the second stage, Norwood II, which allows more blood flow to the lungs by connecting the superior vena cava to the pulmonary artery, and reduces the volume load on the ventricle. Between the ages of 2-5 the final stage of the operation will be necessary. This stage involves connecting the inferior vena cava to the pulmonary artery, and placing a patch to separate the right and left atrium. Now unoxygenated blood is directed into the lungs, and no mixing occurs unless deliberately left. 
 
 
 




 
 
 
 
 
 
 
 
 
 


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Aortic Valve Replacement


When the aortic valve is very abnormal and if it cannot be effectively repaired, a valve replacement operation may be recommended.  This may involve the use of a mechanical or porcine artificial valve or a homograft (human tissue) valve.

In many cases the patient's own normal pulmonary valve can be used.  This is called the Ross Operation.   In this procedure the healthy pulmonary valve is removed and sewn into the position of the damaged aortic valve.  The pulmonary valve itself is then replaced with a Homograft Valve.  The advantage of this operation is that the new aortic valve will grow with the child and the Homograft Valve, which can be large enough to allow for growth, is not subjected to high pressure and can last much longer in the position of the low pressure pulmonary valve - though it is likely that it will eventually need to be replaced at a future operation.

 


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