Tricuspid atresia (TA) is a heart defect present at birth (congenital). It occurs when
the tricuspid valve doesn’t form. Normally, the tricuspid valve is located between
the right upper chamber (atrium) and the right lower chamber (ventricle) of the heart.
Instead of a valve, a piece of tissue forms which restricts blood flow between the right
atrium and right ventricle. The defect keeps low oygenated blood from flowing normally
into the right atrium to the right ventricle and to the lungs where the blood becomes
oxygenated. With tricuspid atresia, there is mixing of low oxygenated blood with
oxygenated blood. This means not enough oxygen in the blood is carried to the body. This
causes a a bluish coloring of the skin.
tricuspid atresia, the following occurs:
tricuspid valve does not form properly or at all.
right ventricle is often smaller than normal.
- There is
an opening between the 2 atria (atrial septal defect). This is usually an open
(patent) foramen ovale. The foramen ovale is a normal part of fetal circulation. It
usually closes shortly after birth.
is an opening between the ventricles (ventricular septal defect).
- An open
(patent) ductus arteriosus lets blood flow from the aorta to the pulmonary artery.
This lets blood flow to the lungs for oxygen. The ductus arteriosus is a normal part
of fetal circulation. It usually closes shortly after birth.
heart defects may also be present. These may include transposition of the great
vessels, pulmonary stenosis, or pulmonary atresia.
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 helped by
blood flowing through them. Because 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:
- Blue color of the skin, lips, and nail beds (cyanosis)
- Fast breathing
- Trouble breathing
- Fast heart rate
- Poor feeding
symptoms of tricuspid atresia may look like other health conditions. Have your child see
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:
oximetry. A simple test that uses a small probe to check the amount of oxygen
in the blood.
Blood and urine samples will be checked.
X-ray. A chest X-ray may show changes often seen with tricuspid atresia.
Electrocardiogram (ECG). An ECG records the electrical activity of the
heart. It shows abnormal rhythms, and detects heart muscle stress.
Echocardiogram (echo). An echo uses sound waves to make a moving picture of
the heart and heart valves. The diagnosis of tricuspid atresia will be made based on
finding the abnormal heart structures.
Heart cath (c
ardiac catheterization). This procedure gives very
detailed information about the structures inside the heart. Your baby will be given
medicine to relax (sedation). Then a thin, flexible tube (catheter) is put into a
blood vessel in your baby's groin. It’s guided to the heart. Blood pressure and
oxygen measurements are taken in the heart chambers, the pulmonary artery, and the
aorta. Contrast dye is also injected to more clearly see the structures inside the
heart. A heart cath is not often done to diagnose this condition but may be done
before surgery and in the initial treatment of the defect.
Your child will most likely be in the intensive care unit (ICU). Early medical treatment may include:
- Supplemental oxygen or possibly a ventilator, to help with breathing
- Medicines may be given to help the heart and lungs work better. Prostaglandin E1 may
be given to keep the ductus arteriosus open.
Other early treatment may include:
cath. In some cases, cardiac cath may be used in the early period. If
pulmonary stenosis is present, a catheter with a balloon at the tip can be inflated
to widen the valve and let enough blood flow through. This may allow the child to
only need 2 surgical stages, rather than 3.
atrial septostomy. A special catheter with a balloon in the tip is used to
create or enlarge an opening in the wall between the left and right atria (atrial
septum). This helps with the flow of blood from the right side of the heart to the
left side of the heart.
Three surgeries, in stages, may be done. They include:
Blalock-Taussig shunt. This surgery is done on newborns. It varies,
depending on the exact defect present in your child. A connection (shunt) is created
to allow blood from the aorta to reach the lungs and pick up oxygen.
This surgery may be done on babies ages 3 to 6 months. A new connection (shunt)
replaces the first shunt. This shunt connects the large blood vessel from the top of
the body to the heart (superior vena cava) to the pulmonary artery. This allows blood
to get to the lungs for oxygen.
procedure. This surgery may be done from ages 2 to 3. A second connection
(shunt) is made. It allows blood from the large blood vessel from the lower part of
the body to the heart to flow to the pulmonary artery. This surgery varies depending
on the exact defect, as well as other factors.
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:
- Not enough oxygen getting to the body's tissues (hypoxemia)
- Enlarged right atrium
rhythm problems (arrhythmias)
ability to exercise
ability to absorb protein from intestines
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 healthcare, 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 get better, you will
likely see large improvements in your child's grow and development.
child will need to be seen often by a pediatric cardiologist. The cardiologist may
- Your child should get all recommended vaccines.
- Your child will probably need antibiotics before some types of surgery or dental procedures.
child may have to not take part in some sports.
- As your child grows and matures, he or she will need to work with a cardiologist and other healthcare providers to plan pregnancy or surgeries.
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.
When to Call a Healthcare Provider
Call your child's healthcare provider if your child's normal symptoms become worse or if new symptoms appear.
- Tricuspid atresia occurs when the tricuspid valve does not develop properly. This prevents blood flow from the right atrium to the right ventricle.
- Tricuspid atresia may be identified during a prenatal ultrasound or shortly after birth when the baby becomes blue (cyanotic).
- Your child will stay in the intensive care unit (ICU).
- Your child will need medicines and a series of surgical procedures to allow oxygen-rich (red) blood to be pumped to the body.
- Your child will need to be followed by a specialist in congenital heart conditions throughout life.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the
visit, write down the name of a new diagnosis, and any new medicines, treatments, or
tests. Also write down any new instructions your healthcare provider gives you for
- Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
how you can contact your child’s healthcare provider after office hours. This is
important if your child becomes ill and you have questions or need advice.