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Problems with the Chambers

1. Right Ventricle

Problem: Hypoplastic Right Ventricle

If the right ventricle is small and weak, this condition is called Hypoplastic Right Heart or Hypoplastic Right Ventricle. It happened because the tricuspid valve guiding the blood from the right atrium to the right ventricle did not open in the very early embryonic weeks of the baby's life, and so no blood came through to make the right ventricle grow.

2. Left Ventricle

Problem: Hypoplastic Left Heart Syndrome

If the left ventricle is small and weak, this condition is called Hypoplastic Left Ventricle or Hypoplastic Left Heart Syndrome. It happened because the mitral valve guiding the blood from the left atrium to the left ventricle did not open in the very early embryonic weeks of the baby's life, and so no blood came through to make the left ventricle grow.


Hypoplastic Right Heart Syndrome

The right ventricle is small and weak because the tricuspid valve guiding the blood from the right atrium to the right ventricle did not open in the very early embryonic weeks of the baby's life, and so no blood came through to make the right ventricle grow. This condition is also called Hypoplastic Right Heart.

Hypoplastic Left Heart Syndrome

This condition 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.

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.

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.

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.

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.

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:

1. The first procedure, or first stage, is called the "Norwood Procedure":

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2. The second procedure, or second stage, is called the "Glenn procedure":

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3. The third procedure, or third stage, is called the "Fontan Procedure":

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Children's Heart Institute

P. O. Box 10066 McLean, VA 22102

Central Registration (patient appointments):

phone - 703-481-5801

Billing Inquiries:

phone - 571-612-2600 / fax - 571-266-4096