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The surgical options for HLHS include cardiac transplantation and the Norwood Procedure. The Norwood Procedure is the first of a series of operations designed to have the blue (unoxygenated) blood flow directly to the lungs and then use the existing right heart to pump the red (oxygenated) blood to the body. All three stages are done through a median sternotomy (chest) incision.

Stage I - Norwood Procedure: The large main pulmonary artery and small aorta are fashioned together to make a new, larger aorta. There is a small tube (shunt) put in to connect the lung artery to the aorta. This shunt gives blood flow to the lungs. The wall that separates the top two heart chambers is removed (atrial septectomy). The narrowing of the aorta is opened. The connecting blood vessel is removed.

Stage II - Bidirectional Glenn Shunt: The blue blood from the head, neck and upper body is directed to the right lung artery through the superior vena cava. This allows the blood to flow into the lungs for oxygen. The temporary shunt from the Stage I operation is removed. The Stage II procedure reduces the work load of the heart.

Stage III - Extracardiac Fontan Procedure: The blue blood from the lower part of the body is directed to the lungs. This is done using the inferior vena cava and artificial material (Gortex(r) ). A conduit (tube) is fashioned from the material. It is connected from the inferior vena cava to the pulmonary artery. This bypass or rerouting allows the blue blood to enter the lungs without being pumped by the heart. The heart remains available to receive the red blood from the lungs and then pump it to the body. Sometimes, a small hole (fenestration) is placed in the baffle (with a snare around it) to allow the heart and body to adjust gradually to the new blood flow system. The single hole is closed later via a small incision. (Another method is to place several small holes in the baffle to allow the heart and body to adjust gradually to the new blood flow system. These holes close off on their own a few months after surgery.)

Goals of the procedure:

• separate the red and blue blood
• have the blue blood enter the lungs directly
• have the heart itself as the pump of oxygen-rich blood to the body

Read more about this condition:

• University of Michigan Children's Congenital Heart Services
• American Heart Association
• Children's Hospital Boston
• Cincinnati Children's Hospital
• Mayo Clinic
• Medline Plus - Natl Library of Medicine/National Institutes of Health
• Children's Hospital of Wisconsin

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