HLHS- I don't understand....

Hypoplastic Left Heart Syndrome? What does that mean? How can that be?  How can a baby have half a heart? How can such a terrible thing happen? How can he survive? Those are just a few of the questions I know I asked. Total distraught and fear was what I was feeling.

As difficult as it was, I knew that I had to swallow hard, try to stop the tears and find out all that I could to help make realilty a little more understandable and acceptable. I wanted to know what was going to happen with this precious little angel. I will share some of the results of my research.

HLHS is a congenital birth defect that effects the left side of the heart. It is a combination of several abnormalites of the heart and great blood vessels. The defects occur due to abnormal underdevelopment of sections of the fetal heart during the first 8 weeks of pregnancy, with no explainations or reasoning. No one knows why or how.

It is the most common fatal congenital heart disease in newborns.  Until 1983, this heart condition was considered lethal. There are now two surgical options available for survival.

Heart transplant is one option, normal rejection risks are involved and finding a newborn heart for transplant can be difficult.  The other option is a three step surgical procedure.  The first stage is called the Norwood. This procedure allows the right ventricle to be the main pumping chamber for blood flow to the body. The aorta is made larger to accomodate the increased blood flow. A connection is made to the pulmonary artery with a shunt to allow blood flow to the lungs. Because the oxygen rich blood is mixing with oxygen poor blood in the aorta, the baby will still have a bluish tint or what is called "cyanosis".   This surgery is performed within the first week of life.

When the baby's lung pressure has decreased, it is time to perform step 2, usually performed at about 4-6 months of age. The second step is the Hemi-Fontan or the Glenn.  This step reduces the work of the right ventricle by allowing it to pump blood to the body only, and allowing most of the blood to flow automatically from the body to the lungs.

The third and final stage, the Fontan, completes the series of surgeries. This step allows the remaining oxygen poor blood coming back from the body to go to the lungs, improving the oxygenation of the blood. This stage is normally performed at 3-4 years of age.

There are many risks involved with each of these surgeries.  The first one is the most risky, the newborn heart is so very small and fragile. At each step the baby is placed on a ventilator, blood by-pass machine, so there is always the chance of many possible complications. 

A lot of information to try to take in and make some kind of sense, huh? I see a rough and bumpy road ahead.