Hypoplastic Left Heart Syndrome - HLHS : Heart Disease : Heart Disease Online

	From your Guide Dr.MANI
*Hypoplastic Left Heart Syndrome*

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If your child - someone you love very much - has had the Fontan operation, you must have often wondered what the future holds ... Can my child study ? Play ? Work ? Marry ? Have kids ? YOU NEED MORE INFORMATION ! Dr.Mani's NEW report, AFTER THE FONTAN, deals with survival after the Fontan, quality of life issues and complications of the Fontan procedure. To learn more about this essential report, CLICK HERE

What are the surgical options ?

There are two major lines of treatment for HLHS. The first is a series of operations called the NORWOOD PROCEDURE. The other alternative is HEART TRANSPLANTATION. Both are formidable procedures, and carry some risks. But the good news is that results are steadily improving. Let me first describe what these operations are.

What is the Norwood operation ?

This is a multi-stage procedure to treat HLHS. The first stage is usually performed within the first week of life. It is an "open-heart" operation, done using the heart-lung machine to support the circulation. The aorta, which is very small in HLHS, is entirely reconstructed by an ingenious technique.
The initial portion of the pulmonary artery, the small aorta, and an AORTIC HOMOGRAFT are stitched together in such a way that an almost normal-sized new aorta - called a "NEO-AORTA" is built. This neo-aorta has a connection with the right ventricle through the pulmonary artery. So the right ventricle now acts as the chamber pumping blood to the whole body. At this time, the wall between the two upper chambers (atrium) - the interatrial septum - is also cut away. This is done so that there is no obstruction to blood returning from the lungs into the left atrium and flowing into the right ventricle. To provide blood flow into the lungs, a new channel is created by making a modified BLALOCK-TAUSSIG SHUNT using a 3.5 to 4 millimeter sized PTFE (Gore-tex) tube graft.
(I have described in detail elsewhere the aortic homograft and Blalock-Taussig shunt)
At the second stage of the Norwood procedure, the modified BT shunt is closed, and a BIDIRECTIONAL GLENN SHUNT constructed, just as in Tricuspid Atresia and other Single Ventricle conditions. At the same time, if the pulmonary arteries are found to be deformed, they are repaired. This procedure is called PULMONARY ARTERIOPLASTY.
At the third stage, a complete Fontan circulation is established. The details of the Fontan operation are just as in Tricuspid Atresia.

What are the problems with a Norwood staged operation ?

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