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Single Ventricle Anomalies—Child

(Single Ventricle Lesions—Child)

Definition

Single ventricle anomalies refer to a category of rare heart conditions that can develop in the growing fetus. In a normal heart, the two ventricles work by collecting blood and pumping it to the lungs or the rest of the body. With this condition, one of the ventricles does not develop properly. The defect can be mild to severe. Other heart problems may be present, as well.
Examples of single ventricle anomalies include:
  • Tricuspid atresia—tricuspid valve does not develop
  • Pulmonary atresia—pulmonary valve does not develop
  • Hypoplastic left heart syndrome—left side of the heart does not develop properly
  • Double inlet left ventricle—large left ventricle and small right ventricle
Heart Chambers and Valves
heart anatomy
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Causes

These anomalies are congenital defects. This means that the baby is born with the condition. The abnormality develops while the baby is forming in the womb. It is not known exactly why the heart develops this way in some babies.

Risk Factors

Specific risk factors for single ventricle anomalies are often unclear, but they may include:
  • Family history of congenital heart defect
  • Other heart defects
  • Certain chromosomal disorders
  • Environmental exposure to chemicals that cause birth defects

Symptoms

Symptoms may include:
  • Blue or pale grayish skin color
  • Fast breathing
  • Fast heart rate
  • Sweating
  • Poor feeding/poor weight gain
The doctor may also detect a heart murmur during the exam.
These symptoms may be due to other conditions. If your child has any of these, talk to the doctor right away.

Diagnosis

The doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:
  • Blood tests
  • Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
  • Chest x-ray —an imaging test that uses radiation to create an image of the chest
  • Electrocardiogram —a test that measures the electrical activity of the heart
  • MRI scan —a test that uses magnetic waves to make pictures of structures inside the chest
  • CT scan —a type of x-ray that uses a computer to make pictures of structures inside the chest
  • Cardiac catheterization —a test that uses a catheter (tube) and x-ray machine to assess the heart and its blood supply

Treatment

Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:

Medication

Your child may be given a medicine through a vein in their arm. This medicine, called prostaglandin, can help keep pathways open so blood can get to the lungs to give them oxygen.

Surgery

The goal of surgery is to:
  • Restore connections between the heart, lungs, and body
  • Improve circulation
Depending on the type of defect and how severe it is, there are several surgery options. For example, a shunt may be placed to restore connections between the heart and lungs, as well as the heart and the rest of the body. If there is too much blood flow, a band may be placed around the pulmonary artery. Fontan procedure is another option. This involves a series of surgeries with the goal being to reroute blood travel through the heart and lungs. In other cases, a heart transplant may be needed.

Lifelong Monitoring

Your child will have regular exams from a heart specialist. Your child may also need antibiotics prior to medical or dental procedures. This is to prevent an infection in the heart. Your child’s activity may also be limited.

Prevention

Preventing fetal heart defects may not always be possible, but you can reduce your risk by:
  • Practicing good prenatal care:
    • Visit the doctor regularly to monitor your health and the health of the baby. (Prenatal tests may detect a heart defect in a growing fetus.)
    • Make sure you:
      • Have a healthy lifestyle
      • Eat nutritious food and take prenatal vitamins
      • Do not drink alcohol, smoke , or use drugs during pregnancy
  • Practicing good hygiene and staying away from people who are sick

RESOURCES

American Family Physician http://www.aafp.org/

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

CANADIAN RESOURCES

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

Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

References

American Heart Association. How your cardiologist diagnoses heart defects. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=152 . Accessed July 6, 2010.

American Heart Association. Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects%5FUCM%5F307037%5FArticle.jsp . Accessed July 6, 2010.

Children’s Hospital of Philadelphia. Single ventricle heart defects. Children’s Hospital of Philadelphia website. Available at: http://www.chop.edu/service/cardiac-center/heart-conditions/single-ventricle.html#treatment . Updated January 2010. Accessed July 23, 2010.

Cincinnati Children’s Hospital. Single ventricle anomalies and Fontan circulation. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/info/heart/diagnose/sv.htm . Updated March 2010. Accessed July 23, 2010.

Mayo Clinic. Atrioventricular canal defect. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/atrioventricular-canal-defect/DS00745/DSECTION=risk-factors . Accessed July 7, 2010.

Revision Information

  • Reviewer: Kari Kassir, MD
  • Review Date: 06/2012 -
  • Update Date: 06/06/2012 -

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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