Hypoplastic Left Heart Syndrome

Fifteen to twenty years ago, the only course of treatment available to HLHS patients was that of heart transplantation. Although this possibility remains viable, there are many issues to contend with when considering this option, not the least of which is the ever-immanent possiblity that the body will reject the "foreign" tissue. In order to avoid this potentially devastating scenario, physicians are forced to aggressively treat transplant patients with powerful immunosuppressive drugs, which can debilitate the body's natural ability to ward off infection. Although initial heart-transplant survival rates are high, rejection usually sets in at one point or another, and the consequences of this can be lethal.

Another important issue to consider with regard to heart-transplantation is the lack of suitable donor organs. This matter is particularly crucial for neonates with HLHS, who remain alive on the sole basis of an open ductus (patent ductus arteriosis) which connects the blood circulation of the body to that of the lungs. Shortly after birth, this ductus (which was open in the womb) begins to close, and prostaglandins (hormones) must be administered intravenously to keep it open. This palliative treatment only buys the patient a little time: without transplant or surgical intervention, the baby will die. Let me use this opportunity to remind you how important it is to sign your organ donor card! Someone's life is depending upon it.

Until very recently, HLHS without a transplant was a virtual death-sentence. Today, amazing advances in the field of cardiac surgery have paved the way for a new, three-stage palliative repair sequence dubbed the Norwood procedure, named after the doctor who first innovated the technique.

The following is a very basic overview of how it works: (this can get very complicated! I have done my very best to simplify as much as possible, so please bear with me)

Stage One: The Norwood operation or blalock-taussig (B-T) shunt:

This stage is completed very shortly after birth, and serves to convert the right ventricle into the systemic ventricle- the main pumping mechanism of the heart. (Incidenatlly, this is the left ventricle in a heart that is functionning normally.) This is accomplished by connecting the main pulmonary artery, which carries blood to the lungs to be oxygenated, to the aorta, which carries oxygenated blood to the body. The atrial septum is also removed (the "wall" which divides the right atrium from the left atrium), and a prosthetic blood vessel (passage or

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