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Tricuspid Atresia—Child

(Single Ventricle Defect—Child)

Definition

Tricuspid atresia is an abnormal development of the right side of the heart. It includes a missing valve between the upper and lower chamber. Tricuspid atresia makes it difficult for your heart to efficiently pump blood to the lungs to get oxygen. Holes will be present in the walls between the left and right side of the heart that cause oxygen-rich and oxygen-poor blood to mix. These holes are necessary to allow blood to be brought to the lungs before a repair can be done. It is often associated with a smaller than normal right lower chamber and abnormalities of the pulmonary artery and aorta.

Heart Chambers and Valves

heart anatomy
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Blood Flow Through the Heart

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Causes    TOP

Tricuspid atresia develops when the baby is in the womb. It is not known exactly why some hearts develop this way.

Risk Factors    TOP

Factors that increase the risk for congenital heart defects like tricuspid atresia include:

  • Family history of congenital heart defect
  • Chromosomal disorder such as 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 medications

Symptoms    TOP

Symptoms may include:

  • Blue or pale grayish skin color
  • Fast breathing
  • Sweating
  • Poor feeding/poor weight gain
  • Fatigue

Diagnosis    TOP

Tricuspid atresia may be diagnosed before birth.

After birth, a tricuspid atresia may be suspected if the baby has a blue-ish color or heart failure, and a heart murmur is detected during a physical exam.

Images may be taken of your baby's bodily structures. This can be done with:

Your child's heart function may be tested. This can be done with:

Treatment    TOP

Treatment is important to prevent severe complications such as heart failure. Treatments may include:

Medication

Medication may be given to:

  • Improve the levels of oxygen in the blood.
  • Manage the level of fluids in the body to decrease workload on the heart.
  • Treat heart failure.
  • Prevent a respiratory infection called bronchiolitis.

Oxygen may also be given to increase the amount of oxygen in the blood.

Surgery

Surgery is often needed right away to restore normal blood flow to the lungs. Follow-up surgeries at needed at ages 3-6 months and 2-5 years.

The goal of surgery is to:

  • Improve blood flow within the lungs and heart.
  • Restore connections between the heart, lungs, and body

The exact surgery will depend on the type and severity of defects that are present. For example, a shunt may be placed to increase blood flow between the sides og the heart. Later, a series of surgeries will be done to further reroute blood flow through the heart, lungs, and body.

Lifelong Monitoring    TOP

Your child will have regular exams from a heart specialist. Your child may also need antibiotics before certain medical or dental procedures to prevent a heart infection.

Prevention    TOP

Preventing fetal heart defects may not always be possible. Good prenatal care may reduce some type of congenital heart defects. Prenatal care includes:

  • Visiting the doctor regularly to monitor your health and the health of the baby
  • Eating nutritious food and taking prenatal vitamins
  • Avoiding alcohol, smoking, and using drugs during pregnancy
  • Practicing good hygiene and staying away from people who are sick

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.com

References:

Tricuspid atresia. Boston Children’s Hospital website. Available at:
...(Click grey area to select URL)
Accessed November 10, 2014.
Tricuspid atresia. Children’s Hospital of Philadelphia website. Available at:
...(Click grey area to select URL)
Accessed November 10, 2014.
Tricuspid atresia. EBSCO DynaMed website. Available at:  http://www.dynamed.com/topics/dmp~AN~T113849/Tricuspid-atresia. Updated January 25, 2016. Accessed September 29, 2016.
Last reviewed December 2015 by Kari Kassir, MD
Last Updated: 12/20/2014

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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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