Hypoplastic Left Heart Syndrome
En Español (Spanish Version)

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

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Blood Flow Through the Heart

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Causes
This is a congenital heart defect. This means that the heart forms incorrectly when the baby develops in the womb. The baby is born with the condition. It is not known why the heart forms this way.

Risk Factors
These factors increase your chance of having a child with hypoplastic left heart syndrome:

  • Previous pregnancy with fetal heart abnormalities or fetal loss
  • Family history of congenital heart defect
Symptoms
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
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:

Other tests may be done to evaluate your child's heart. This can be done with:

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

Prevention
There is no way to prevent this condition. Getting proper prenatal care is always important.




RESOURCES:
FamilyDoctor.org—American Academy of Family Physicians

American Heart Association

CANADIAN RESOURCES:
Canadian Cardiovascular Society

Heart and Stroke Foundation of Canada

References:
Hypoplastic left heart syndrome. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp. Updated March 26, 2014. Accessed June 3, 2014.

Hypoplastic left heart syndrome. Children’s Hospital Boston website. Available at: http://www.childrenshospital.org/az/Site502/mainpageS502P0.html. Accessed June 3, 2014.

Hypoplastic left heart syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated January 20, 2014. Accessed June 3, 2014.

Hypoplastic left heart syndrome. Johns Hopkins University, Cove Point Foundation website. Available at: http://www.pted.org/?id=hypoplasticleft4. Updated May 16, 2011. Accessed June 3, 2014.

Last Reviewed May 2014



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