The Children's Heart Institute - Hasan Abdallah, M.D.Patient Education
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Congenital Heart Defects:
Hypoplastic Left Heart Syndrome

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.

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.


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.

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.


Problems with the Walls:
Atrial Septal Defect (ASD) : Fix | Ventricular Septal Defect (VSD) : Fix

Problems with the Doors:
Tricuspid Valve Atresia : Fix 1 | Fix 2 | Fix 3 | Ebstein's Anomaly
Hypoplastic Left Heart Syndrome : Fix 1 | Fix 2 | Fix 3
Pulmonary Valve Stenosis
| Aortic Valve Stenosis

Problems with the Chambers:
Hypoplastic Right Ventricle

Problems with the Hallways:
Coarctation of the Aorta : Fix | Branch Pulmonary Artery Stenosis
Transposition of the Great Arteries : Fixed
Total Anomalous Pulmonary Venous Connection

Problems with the Plumbing:
Anomalous Origin of the Coronary Arteries from the Pulmonary Artery

Problems Involving More Than One Part of the Heart:
Tetralogy of Fallot : Fixed
Truncus Arteriosus : Fixed | Common Atrioventricular Canal : Fix 1 | Fix 2

Ductus Arteriosus : Fix 1 | Fix 2

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Next >>
Next we will show you how doctors fix this operation in a three stage operation starting with the Norwood Operation.

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