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Hypoplastic Left Heart Syndrome
consists of an underdeveloped Left Atrium and Left Ventricle.
The whole left side of the heart which pumps the blood to the
body is underdeveloped and small and is unable to perform that
function. This means the baby is unable to get blood to his body
through the Aortic Valve, the Aortic Arch and the front parts.
However there is usually a connection
between the main Pulmonary Artery and the Aorta at birth called
a Ductus Arteriosus. |
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Ductus Arteriosus
The blood which then comes out from the Right Ventricle to the
Pulmonary Valve can also go through the Ductus to supply blood
to the different arteries of the body through the Aortic Arch.
However, the Ductus Arteriosus
is a passageway which normally closes within a few days after
birth and needs to stay open. Doctors temporarily fix this problem
by starting the newborn on a medication called Prostaglandin.
To keep this passageway open, doctors must perform surgery. |
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Symptoms:
A newborn with this defect will look very pale at birth with
no blood in his face because the heart is not pumping enough
blood though the Aorta to the body. He will be breathing very
fast because most of the blood is going to the lungs making them
congested. He will be sweating and very weak and unable to suck
from his formula. |
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This condition is not compatible
with life and if the Ductus closes on its own no blood will be
able to get to the baby's brain and organs and he may die in
shock. |
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Treatment Options:
This condition is still considered one of the most difficult
issues in pediatric cardiology. Managing this condition depends
on the family's wishes and the severity of the condition. Treatments
include:
Compassionate Care Only
After the doctors discuss the condition with the family, the
family may wish to only give compassionate care to the baby and
let the baby die in peace. However this is a very involved process
and requires input from the medical profession and very involved
family support. We recommend that you discuss this condition
very carefully with your doctors. |
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Heart Transplant
Doctors will keep your baby on Prostaglandin until a baby's heart
becomes available and then a transplant has to be performed at
one of the centers which is geared towards doing heart transplants
in newborn infants.
Surgery
The surgical option is a three-stage process.
To see the three operations
performed to fix this defect follow the purple tour bus links
at the bottom of this page. |
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