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Tricuspid Atresia—Child

(Single Ventricle Defect—Child)

Definition

In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle through the tricuspid valve. The blood travels to the lungs through the pulmonary valve to pick up fresh oxygen. Next, the blood returns to the left atrium, goes into the left ventricle, and goes out to the rest of the body.
Tricuspid atresia is when the tricuspid valve (located on the right side of the heart) does not develop. Also, the right ventricle is abnormally small. When this occurs, blood cannot move from the right atrium to the right ventricle. The defect can be mild to severe. There may also be other defects, like holes in the walls of the heart chambers that allow blood to move back and forth.
Heart Chambers and Valves
heart anatomy
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Blood Flow Through the Heart
IMAGE
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Causes

This condition is a congenital defect. This means that the heart forms incorrectly while the baby is in the womb. It is not known exactly why some babies’ hearts develop this way.

Risk Factors

Risk factors for congenital heart defects like tricuspid atresia may include:
  • Family history of congenital heart defect
  • Chromosomal disorder (eg, Down syndrome )
  • Previous pregnancy with fetal heart abnormalities or miscarriage
  • Conditions during pregnancy, such as:
    • Being infected with a virus
    • Having poorly controlled diabetes
    • Drinking alcohol
    • Taking certain medicines

Symptoms

Symptoms may include:
  • Blue or pale grayish skin color
  • Fast breathing
  • Sweating
  • Poor feeding/poor weight gain
The doctor may also detect a heart murmur during the exam.
This condition can lead to heart failure . If your child has any of these symptoms, get medical care right away.

Diagnosis

Your doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:
  • 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 low amounts of radiation to create an image of the chest
  • Electrocardiogram —a test that measures the electrical activity of the heart

Treatment

Talk with your doctor about the best treatment plan for your child. Often, surgery is needed right away with follow-up surgeries at ages 3-6 months and 2-5 years. Treatment options include:

Medication

Newborn babies are often given a medicine called prostaglandin. This medicine helps keep pathways open so blood can get to the lungs for oxygenation.

Surgery

The goal of surgery is to:
  • Improve blood flow within the heart
  • Restore connections between the heart, lungs, and body
Depending on the severity of the defect, there are several options. For example, a shunt may be placed to increase blood flow to the lungs. A Fontan procedure is another type of surgery. This involves a series of surgeries with a goal to reroute blood travel through the heart, lungs and body.

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 a heart infection.

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. Tricuspid atresia. American Heart Association website. Available at: http://americanheart.org/presenter.jhtml?identifier=1310 . Accessed July 6, 2010.

Cincinnati Children’s Hospital. Tricuspid Atresia. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/tricuspid.htm . Accessed July 6, 2010.

DynaMed Editorial Team. Tricuspid atresia. EBSCO DynaMed website. Available at:  http://www.ebscohost.com/dynamed/what.php . Updated February 11, 2010. Accessed July 6, 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: 00/60/2012 -

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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