Tricuspid atresia (TA) is a heart defect present at birth (congenital). It occurs when the tricuspid valve doesn’t form right during fetal heart development. This happens during the first 8 weeks of pregnancy. The tricuspid valve is located between the right upper chamber (atrium) and the right lower chamber (ventricle) of the heart. The defect keeps blood from flowing normally from the right atrium to the right ventricle.
In tricuspid atresia, the following occurs:
The fetal heart develops during the first 8 weeks of pregnancy. In tricuspid atresia, the tricuspid valve does not form as it should. Development of the ventricles is influenced by blood flowing through them. Since blood does not pass through the tricuspid valve, the right ventricle remains small.
Some congenital heart defects may be passed down through families (genetic defects). Most of the time, there is no known cause for tricuspid atresia.
Symptoms of tricuspid atresia occur shortly after birth. Common symptoms may include:
The symptoms of tricuspid atresia may look like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.
Tricuspid atresia may be diagnosed with a prenatal ultrasound exam. Otherwise, symptoms occur shortly after birth. A pediatric cardiologist or a neonatologist will help care for your child. These are doctors with special training to treat problems in children.
One or more healthcare providers will examine your baby. They will listen to your baby's heart and lungs with a stethoscope and note any abnormal heart sound (heart murmur).
Testing for congenital heart disease may include:
Your child will most likely be in the intensive care unit (ICU). Early medical treatment may include:
Other early treatment may include:
Three surgeries, in stages, may be done. They are:
Heart transplant is also a choice, but it is not usually done because it is difficult to find a donor heart. Also the surgeries to improve blood flow have become more and more successful.
Complications of tricuspid atresia may include:
Your child may need a series of repair procedures.
Before your child comes home, the nursing staff will explain medicines and treatments. For example, your child may need special formula and supplemental feedings to help him or her grow. The staff will also help to arrange home health care, if needed. You may get other instructions from your child's heart care team and the hospital staff.
Babies will have bluish skin (cyanosis) after the first 2 surgeries, or until the final Fontan procedure is done. Your child may grow and develop slowly because of decreased oxygen in the blood. After the Fontan procedure, when oxygen levels improve, you will likely see large improvements in your child's grow and development.
Your child will need to be followed by a pediatric cardiologist. The cardiologist may recommend:
Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout life.
Talk with your child's cardiologist about the specific outlook for your child.
Tips to help you get the most from a visit to your child’s healthcare provider: