Hypoplastic left heart syndrome (HLHS) is a rare heart defect. With this syndrome, structures on the left side of the heart, such as the aorta, aortic valve, left ventricle, and mitral valve, may be:
- Too small
- Poorly developed
Since the heart can’t work the right way, oxygen-rich blood flow to the body is lower. Your child will need medical care right away.
HLHS is present at birth. It is caused by a genetic defect. It is not known exactly why the heart does not develop normally.
Factors that may raise your chance of having a child with HLHS are:
- People in your family who have congenital heart defects
- Prior pregnancy with fetal heart problems or miscarriage
Symptoms often appear within days after birth. Tell the doctor if your baby has:
- Blue/gray skin color
- Cool skin
- Problems breathing
- Fast heart beat
- Sweaty, clammy skin
- Poor feeding
You will be asked about your child's symptoms and health history. A physical exam will be done.
Pictures may be taken of your child's chest. This can be done with:
Talk with your doctor about the best plan for your child. Some defects are hard to treat. Your child may have:
Medicines are needed to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and the aorta. It should close within a few days after birth. Keeping this passage open is a short term treatment. Other medicines may be used as well.
Surgery may be done to help the blood flow. This can be done through a reconstructive and shunting procedures. This is often done in stages:
- After birth
- 4-6 months of age
- 2-4 years of age
Your child will need to see a heart specialist regularly. Heart medicine will be needed throughout your child's life.
- Reviewer: Kari Kassir, MD
- Review Date: 05/2018 -
- Update Date: 06/29/2018 -