Hypoplastic Left Heart Syndrome (HLHS)
What to Ask
Welcome to the HLHS Parent information page.  This site was designed by parents of a child with HLHS to help other parents receiving the prenatal diagnosis of Hypoplastic Left Heart Syndrome (HLHS).  

(If your child has been diagnosed with another single ventricle defect such as: Hypoplastic Right Heart Syndrome (HRHS), Tricuspid Atresia, Double Inlet Left Ventricle (DILV), Double Outlet Right Ventricle (DORV), or Single Ventricle much of this site will still be applicable to you.  Please first see our single ventricle page for descriptions and links to information on these conditions.)

Receiving your baby's diagnosis of HLHS can be frightening and overwhelming.  The important thing to know is that there is hope.   Fortunately, there are now surgical options which can successfully treat babies with HLHS.  

If you have received the diagnosis while you are pregnant, you have time to learn about the condition, and make some important decisions:  

1) Which treatment option to pursue: Heart Transplant, the 3-Stage Surgical Procedure or a fetal intervention (see below).

2) Where to deliver the baby 

3) Where to have the baby's surgery performed.  

(Note:  There is also now a fetal intervention for which some may qualify.  This procedure is done during pregnancy to hopefull prevent HLHS from occurring at birth.  The results are mixed, and it does carry risks to the baby and mother.  To our knowledge, this procedure is only being done at a few facilities, such as Children's Hospital of Boston.  Please see their website for more information and a description of the procedure.

To help you make these decisions, this web site contains:
Some basic information on HLHS

Frequently Asked Questions about having a baby with HLHS
* Basic descriptions of the two surgical options - heart transplant and the 3-stage surgery
* A list of questions to ask your insurance company, the surgeon and the hospital  
* A list of some hospitals that perform the 3-Stage Procedure and/or Heart Transplants. (Note that this is not a complete list, the number of hospitals performing these procedures grows every year.  However, it is best to find out how much experience they have, and how many procedures are done annually.)
*  Additional resources

Please note that this web site was created by the parents of a child with HLHS, and not medical professionals.  It is not intended as medical advice, merely a guide to help you understand the condition and your options.  Consult your physician with any questions you may have regarding HLHS.
This web site is not intended as medical advice.  Please consult your physician with any questions you may have regarding your child's condition.

Copyright © 2001-2011 D.L. Hilton-Kamm, all rights reserved. 
Hypoplastic Left Heart Syndrome (HLHS)
Information Page
Hypoplastic Left Heart Syndrome (HLHS) is a condition in which the left side of the heart is underdeveloped.  Usually, the left ventricle, the left atrium, the mitral valve and the aorta are affected.  It is called a syndrome because it can encompass several different variations and varying degrees of development of these parts of the heart. 

To understand HLHS, it is helpful to understand how a normal baby's heart works.  The heart is comprised of four chambers: the upper chambers are called the left and right atria, and the lower chambers are called the left and right ventricles.  

Very simply, in a healthy heart blood flows from the right atrium to the right ventricle where it is then pumped through the pulmonary artery to the lungs to be oxygenated.  Blood then flows back to the heart via the left atrium to the left ventricle, which pumps this oxygenated blood through the aorta out to the body.  This is how the body's organs and tissue receive oxygen, which is vital.  When a baby has HLHS, the left side of the heart is underdeveloped so it cannot sufficiently pump the oxygenated blood out to the body. 

Babies with HLHS do not have problems while in the womb - it is only after birth that the heart fails to work properly.  This is because all babies receive oxygen from the placenta while in the womb, so blood does not need to go to the lungs.  In addition, there is an opening between the pulmonary artery and the aorta, called the patent ductus arteriosus (PDA) that is present in all babies.  It allows the blood to go from the right ventricle out to the body, bypassing the left side of the heart.  

The PDA usually closes a few days after birth, separating the left and right sides of the heart.  It is at this time that babies with undetected HLHS will exhibit problems as they experience a lack of blood flow to the body.  They may look blue, have trouble eating, and breathe rapidly.  If left untreated, this heart defect is fatal - usually within the first few days or weeks of life.  

Once HLHS has been diagnosed, a drug called prostaglandin is given to keep the PDA open until surgery is performed.  There are two surgical options for treating HLHS:  a Heart Transplant or the 3-Stage Surgical Procedure, the first stage of which is called the Norwood procedure.  (For bloodless surgical options, please see the Columbus Children's Hospital website.)

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This page was last updated on: May 29, 2012
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HLHS Survivors
Frequently Asked Questions
Did you find this website helpful?  If so, feel free to refer family and friends here, so they can understand more about HLHS and the decisions you face.    

You can also view our sister site, California Heart Connection,  to join a free online support group, and see more information and resources.   You can also create a webpage to keep family and friends informed of your baby's progress.  Our primary concern is that you get the support and information you need.  

If you would like to help us help other families, please consider donating to our nonprofit organization, California Heart Connection.  
Read our new article published in Pediatric CardiologyPrenatal Diagnosis of Hypoplastic Left Heart Syndrome: Impact of Counseling Patterns on Parental Perceptions and Decisions Regarding Termination of Pregnancy  and our previously published abstracts based upon a nationwide CHD Parent Survey. 

Other articles:  Successful pregnancies in two women with hypoplastic left heart syndrome.