Carl R. Darnall Army Medical Center - Health Library

Hypoplastic Left Heart Syndrome

(HLHS)

Definition

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
  • Absent
  • 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.

Heart Chambers and Valves
heart anatomy

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Blood Flow Through the Heart

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Causes  ^

HLHS is present at birth. It is caused by a genetic defect. It is not known exactly why the heart does not develop normally.

Risk Factors  ^

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  ^

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

Diagnosis  ^

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:

Treatment  ^

Talk with your doctor about the best plan for your child. Some defects are hard to treat. Your child may have:

Medications

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

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

Lifelong Monitoring

Your child will need to see a heart specialist regularly. Heart medicine will be needed throughout your child's life.

Prevention  ^

HLHS can’t be prevented.

RESOURCES:

American Heart Association
http://www.heart.org

Family Doctor—American Academy of Family Physicians
http://familydoctor.org

CANADIAN RESOURCES:

Canadian Cardiovascular Society
http://www.ccs.ca

Heart and Stroke Foundation of Canada
http://www.heartandstroke.ca

REFERENCES:

Hypoplastic left heart syndrome. EBSCO DynaMed Plus website. Available at:http://www.dynamed.com/topics/dmp~AN~T114438/Hypoplastic-left-heart-syndrome. Updated September 11, 2017. Accessed June 29, 2018.

Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp. Updated September 12, 2017. Accessed June 29, 2018.

Last reviewed June 2018 by Kari Kassir, MD  Last Updated: 6/29/2018