This web site is not intended as medical advice.  Please consult your physician with any questions you may have regarding your child's condition.

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Surgical Options 
Babies with HLHS can be put on a list to receive a heart transplant.  When the baby's name is at the top of the list, he or she will receive a healthy donor heart.  To fight against rejection of the new heart, transplant patients need to take anti-rejection medications for the rest of their lives.  Unfortunately, there are not enough donor hearts for all babies waiting for a transplant, and thus, some do not survive while waiting for a heart.  Here are a few sites that explain heart transplants in general.  Speak with your cardiologist or surgeon about infant heart transplants specifically for babies with HLHS.
Stanford Children's Health
​Johns Hopkins

An alternative to a heart transplant is the 3-Stage Surgical Procedure.  The 3-Stage Procedure reconstructs the child's heart allowing it to work using only two of the heart's four chambers.  Here are a few resources that explain each stage.  These links were chosen for their simple descriptions and use of diagrams or videos.  There are many other websites that also explain the surgical procedures.
CovePoint - interactive diagrams to see each stage
Cincinnati Children's Hospital - videos of each stage
Nemours - diagrams and descriptions
Children's Hospital of Philadelphia
Nationwide Children's - include information on the Hybrid Procedure

Basic descriptions of the surgical options:

The first stage is called the Norwood procedure, or the "Modified Norwood" which was developed in 1979 by Dr. William Norwood.  Because the left ventricle cannot pump blood adequately out to the body, the Norwood procedure allows the right ventricle to pump blood to both the lungs and the body.  This surgery is usually performed during the baby's first week of life.  A newer version of this first surgery is called the "Sano Shunt."  Ask the surgeon about the pros and cons of the traditional Norwood vs. the Sano Shunt, how many of each he has performed, and what the success rates are.  (Another variation is the "Hybrid Procedure" which does the 3 stage in a catheterization, a surgery, then another catheterization.  The benefit is fewer surgeries, however, only a certain number of babies are candidates, and the results may or may not be better than the regular 3-stage.)

The second stage procedure, called the Hemi-Fontan or Bidirectional Glenn, reduces the work of the right ventricle by allowing it to pump blood only to the body, and allowing most of the blood to flow automatically from the body into the lungs.  The second stage is performed when the pressure in the baby's lungs has decreased, no longer requiring the pumping action of the right ventricle to get blood into the lungs.  This surgery is usually performed at about 4-6 months of age when the traditional Norwood has been performed.  When the Sano Shunt is performed, the second surgery may be done earlier - at about 3-4 months of age, and the babies may need constant monitoring to determine when they are ready for the second surgery.

The third and final stage, the Fontan, allows the rest of the blood coming back from the body to go to the lungs.  There are two main different ways to perform the Fontan:  The Lateral Tunnel Fontan, the more traditional approach, and the newer External Conduit Fontan.  The Lateral Tunnel Fontan is usually performed at about 18 months - 3 years of age, and the External Conduit Fontan is usually performed at about 3-4 years of age.  

The External Conduit Fontan uses an external conduit, or tube, that is placed just outside the heart.  It is hoped that by using this conduit that the potential for arrhythmias will be greatly reduced in the future.  However, this is a relatively new procedure and the outcomes will not be known for several years.  It is usually done later than the Lateral Tunnel Fontan because the child has to be big enough to handle a conduit that can handle the blood flow as the child grows.  

There are pros and cons of each type of Fontan procedure, so make sure you discuss with your surgeon the different types of Fontan operations and what the risks and benefits are of each.

See our Resources Page for websites that contain more detailed explanations and diagrams of each of the surgeries.

Bloodless Surgeries
For parents whose religious beliefs restrict them from allowing blood products, surgeons
Stanford - article on bloodless open heart surgery on an infant
Johns Hopkins bloodless surgery program

Surgical Options
Choosing the best procedure for your baby

Your child's specific condition may dictate that a certain procedure be performed.  Of those parents who have a choice, some choose a heart transplant so that the baby will receive a fully functioning 4-chamber heart, rather than reconstructing the baby's 2-chamber heart as is done in the 3-Stage Procedure.  Others choose the 3-Stage Procedure to eliminate the wait for a donor heart, the risk of rejection, and the child having to take anti-rejection medications for life.  

Not all hospitals offer heart transplants and the 3-stage surgeries.  Some do only transplants, and some do only the 3-stage surgeries.  Hospitals may also vary in their heart transplant policy.  Some hospitals may not put HLHS babies who are candidates for the 3-Stage Procedure on their transplant list, reserving transplants for babies with no other option.  

It is up to you to discuss the pros and cons of each procedure with your surgeon to determine what is best for your baby's particular condition and choose a hospital where that procedure can be performed.

Regardless of the type of procedure to be done, we strongly encourage you to familiarize yourself with hospitals which perform many of that type of surgery.  Heart transplants and the 3-Stage Procedure are extremely complex surgeries.  Surgeons and hospitals which have a history of performing these surgeries and perform many of them annually, usually have better success rates.  These are called "high volume" centers, since they perform many surgeries.  
Hypoplastic Left Heart Syndrome (HLHS) Information Page
- Fetal Intervention
- Heart Transplant
- 3-Stage Procedure
- Choosing the best procedure for your baby (transplant or 3-stage)