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
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Blood Flow Through the Heart
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Tricuspid atresia develops when the baby is in the womb. It is not known exactly why some hearts develop this way.
Factors that increase the risk for congenital heart defects like tricuspid atresia include: Family history of congenital heart defect
Chromosomal disorder such as
Previous pregnancy with fetal heart abnormalities or
Conditions during pregnancy, such as:
Being infected with a virusHaving poorly controlled diabetesDrinking alcoholTaking certain medications
Symptoms may include: Blue or pale grayish skin colorFast breathingSweatingPoor feeding/poor weight gainFatigue
Tricuspid atresia may be diagnosed
After birth, a tricuspid atresia may be suspected if the baby has a blue-ish color or heart failure, and a
is detected during a physical exam.
Images may be taken of your baby's bodily structures. This can be done with: EchocardiogramChest x-ray
Your child's heart function may be tested. This can be done with: ElectrocardiogramCardiac catheterization
Treatment is important to prevent severe complications such as
heart failure. Treatments may include:
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 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.
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.
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 babyEating nutritious food and taking prenatal vitamins
smoking, and using drugs during pregnancy
Practicing good hygiene and staying away from people who are sick
Tricuspid atresia. Boston Children’s Hospital website. Available at:
http://www.childrenshospital.org/az/Site508/mainpageS508P0.html. Accessed November 10, 2014.
Tricuspid atresia. Children’s Hospital of Philadelphia website. Available at:
http://www.chop.edu/service/cardiac-center/heart-conditions/tricuspid-atresia.html. Accessed November 10, 2014.
Last reviewed December 2015 by Kari Kassir, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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