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Lahey Health is now part of Beth Israel Lahey Health

by EBSCO Medical Review Board
(Single Ventricle Defect—Child)

Definition

Tricuspid atresia is when the tricuspid valve that controls blood flow between the right upper and right lower chamber is missing. This makes it hard for the heart to pump blood to the lungs to get oxygen. There may also be a hole between the left and right side of the heart that causes oxygen-rich and oxygen-poor blood to mix. A child may also have 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
IMAGE
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Causes

This problem is caused by a congenital defect. This means that the problem develops in the womb and a baby is born with it. It is not known exactly why this happens.

Risk Factors

Things that may raise the risk of this problem are:

  • A family history of congenital heart defects
  • Chromosomal disorders, such as Down syndrome
  • Prior pregnancy with fetal heart abnormalities or miscarriage
  • Factors in the mother, such as:
    • Being infected with a virus
    • Having poorly controlled diabetes
    • Drinking alcohol
    • Taking certain medicines

Symptoms

Problems may be:

  • Blue or pale grayish skin color
  • Fast breathing
  • Sweating
  • Poor feeding
  • Poor weight gain
  • Lack of energy

Diagnosis

This problem is often diagnosed before birth.

In others, the doctor will ask about your child’s symptoms and health history. A physical exam will be done. It will focus on the heart. A heart murmur may be detected.

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

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

Treatment

The goal of treatment is to restore normal blood flow and to prevent severe problems. Supportive care, such as oxygen and nutrition therapy, will be needed.

Treatment may include medicines to:

  • Strengthen the heart muscle
  • Lower blood pressure
  • Help the body get rid of extra fluids

Surgery will be done right away. The surgery chosen depends on a child's symptoms and heart defects. More than one surgery may be needed. The goal of surgery is to:

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

Lifelong heart monitoring will also be needed.

Prevention

There are no known guidelines to prevent this heart problem.

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

Rao PS. Consensus on timing of intervention for common congenital heart diseases: part II - cyanotic heart defects. Indian J Pediatr. 2013 Aug;80(8):663-674.

Tricuspid atresia. Boston Children’s Hospital website. Available at: https://www.childrenshospital.org/conditions-and-treatments/conditions/t/tricuspid-atresia. Accessed March 11, 2021.

Tricuspid atresia. Children’s Hospital of Philadelphia website. Available at: http://www.chop.edu/service/cardiac-center/heart-conditions/tricuspid-atresia.html. Accessed March 11, 2021.

Tricuspid atresia. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/tricuspid-atresia . Accessed March 11, 2021.

Revision Information

  • Reviewer: EBSCO Medical Review Board Kari Kassir, MD
  • Review Date: 12/2020
  • Update Date: 03/11/2021