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Hypoplastic Left Heart Syndrome

Heart Disease Best of the Net - Hypoplastic Left Heart Syndrome - HLHS, Congenital heart disease,heart birth defects

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NEW ! Special Report
Dr.Mani's AFTER THE FONTAN
How Fontan survivors fare in the long term

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 is heart transplantation ?

Sometimes the heart may be so severely hypoplastic (small), or other conditions may co-exist that make a Norwood type operation more risky and likely to fail. The only choice then is to replace the heart with another healthy one - Heart Transplantation.
Adult heart transplantation is currently quite successful. But in a newborn infant it is a formidable operation. A heart transplant for HLHS was first performed successfully in 1985 by Drs. Bailey and Gundry at Loma Linda in California, USA - still the center with the most experience in neonatal heart transplantation.
The philosophy behind transplanting a child with severe HLHS is that when reconstruction by a Norwood operation is attempted

  • the right ventricle may fail after many years
  • the tricuspid valve may become leaky later
  • the pulmonary valve may become leaky due to the high pressures that it needs to withstand
  • the operation itself is very difficult technically.

However, transplantation too carries its price tag. There is an indefinite waiting period till a donor organ becomes available. Some patients will die before a donor becomes available. Also, since the operation is new, and is still limited to very few institutions world-wide, long term results are uncertain. Early development of coronary artery disease in the donated heart may limit late survival. Complications of the medicines used to prevent graft rejection include development of a form of cancer called lymphoma. And heart transplantation is also an expensive affair.
Maybe in the future, transplantation will be better reserved for the more severe forms of HLHS who are unsuited for reconstruction. In the others, repair techniques must be preferred. With improvement in technology and experience, most of these patients should be correctable successfully.

For some time to come, however, HLHS will remain a troublesome challenge to those of us who are concerned with treating congenital heart disease.

If there's anything more you want to read about, or some areas which aren't clear enough, don't hesitate to write and let me know.

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