Wednesday, November 14, 2012

What is Hypoplastic Left Heart Syndrome?


HLHS and The 3 Open Heart Surgeries Explained

The above picture shows a normal heart compared Christian's heart.  He did not develop a left ventricle at all.
What is HLHS?
Hpoplastic Left Heart Syndrome is a Congenital Heart Defect where the left side of the heart is underdeveloped. In Christian's case, the Left Ventricle isn't there or small enough they couldn't see it. The Left Atrium and the Aorta is also smaller. This is causing the oxygen rich blood to mix in the heart with the oxygen depleted blood, causing his body to not get the right amount of oxygen needed.

 There are three open heart surgeries that are performed to help separate the oxygen rich blood (red blood) from the oxygen depleted blood (blue blood). The first stage is called the Norwood procedure. In this procedure the doctor will create a "new" Aorta which will be connected to the Right Ventricle. They will also place a tube or shunt from either the Right Ventricle or the Aorta to the pulmonary arteries, which send blood to the lungs to gather oxygen. This will allow his heart to pump blood to his lungs and the rest of his body. It will still be mixing red and blue blood, so the baby may still have a blueish coloring to his body. This surgery is performed in within the first 2 weeks after birth.

 The second stage is called the Bidirectional Glenn Shunt or Hemi-Fontan procedure. This one is performed between 4 and 6 months after birth. In this procedure they will take the vein that brings blood from the upper part of the body to the heart and connect it directly to the pulmonary arteries, taking it to get oxygen before it even goes into the heart. This creates less work for the Right Ventricle to perform. Even after this there will still be mixing of red and blue blood in the heart but it will be reduced.

 The third stage is called the Fontan procedure. It is perform anywhere between 18 months and 3 years after birth. This procedure takes the vein that returns blood from the lower part of the body to the heart and redirects it also to the pulmonary arteries, going straight to the lungs for oxygen. Once this is completed there will be no more mixing of the red and blue blood in the heart thus removing the blueish coloring from his body.

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