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Hypoplastic Left Heart Syndrome

(HLHS)

Definition

Hypoplastic left heart syndrome is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood then moves out to the rest of the body.

With this syndrome, structures on the left side of the heart, which includes the aorta, aortic valve, left ventricle, and mitral valve, may be:

  • Too small
  • Absent
  • Abnormally developed

Since the heart cannot function properly, oxygen-rich blood flow to the body is limited. This condition requires immediate care from a doctor.

Heart Chambers and Valves

heart anatomy
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Blood Flow Through the Heart

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Causes    TOP

Hypoplastic left heart syndrome is present at birth. It is not known exactly why the heart does not develop normally.

Risk Factors    TOP

Factors that may increase your chance of having a child with hypoplastic left heart syndrome include:

  • Previous pregnancy with fetal heart abnormalities or fetal loss
  • Family history of congenital heart defects

Symptoms    TOP

Symptoms usually appear within days after birth. Tell the doctor if you notice the following in your infant or child:

  • Blue/gray skin color
  • Cool skin
  • Rapid or difficult breathing
  • High heart rate
  • Sweaty, clammy skin
  • Poor feeding

Diagnosis    TOP

You will be asked about your child's symptoms and medical history. A physical exam will be done.

Images may be taken of your child's chest. This can be done with:

Electrocardiogram (EKG) can monitor the heart's electrical activity.

Treatment    TOP

Talk with your doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:

Medications

Medications are necessary to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and the aorta, which is the largest artery in the body. It usually closes within a few days after birth. Keeping this passage open is a temporary treatment. Other medications may be used as well.

Surgery

Surgery may be done to improve blood flow. This can be done through a variety of reconstructive and shunting procedures. Surgeries are usually done in stages:

  • After birth
  • 4-6 months of age
  • 2-4 years of age

Lifelong Monitoring    TOP

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

Prevention    TOP

There are no current guidelines to prevent hypoplastic left heart syndrome because the cause is unknown. Getting proper prenatal care is always important.

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.... Updated January 25, 2016. Accessed June 6, 2016.
Hypoplastic left heart syndrome. Johns Hopkins Children's Center website. Available at:
...(Click grey area to select URL)
Updated May 16, 2011. Accessed June 6, 2016.
Single ventricle defects. American Heart Association website. Available at:
...(Click grey area to select URL)
Updated October 21, 2015. Accessed June 6, 2016.
Last reviewed June 2016 by Kari Kassir, MD
Last Updated: 6/4/2014

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This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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