What is tricuspid atresia?

Tricuspid atresia is a congenital (present at birth) heart defect that occurs when the tricuspid valve of the heart is not properly formed. The tricuspid valve lies between two of the chambers of the heart (the right atrium and right ventricle). In tricuspid atresia, there is solid tissue in place of the tricuspid valve. This sheet of tissue blocks blood flow from the right atrium to the right ventricle. Because of this blockage, the right ventricle usually is small and underdeveloped.

Blood in a baby with this defect flows from the upper right chamber (right atrium) to the upper left chamber (left atrium) of the heart through a hole in the septum, the wall between the chambers. The hole can be a heart defect (atrial septal defect) or an enlarged natural opening (foramen ovale). The foramen ovale is a hole that should close soon after a baby is born. If there is no atrial septal defect, a surgeon may need to create a foramen ovale to allow blood flow.

In some babies with tricuspid atresia, there is a hole between the heart’s two lower chambers (ventricular septal defect). In babies with this defect, blood can flow through the hole and into the right ventricle, which will pump the blood into the lungs.

When tricuspid atresia is present, a sufficient amount of blood is not able to flow through the heart and into the lungs. As a result, a baby with this defect has blood that does not obtain oxygen as the blood in a normal heart would. The lungs are not able to provide the rest of the body with its required oxygen.

How is tricuspid atresia diagnosed?

In most cases, a doctor will use an echocardiogram to diagnose tricuspid atresia. An echocardiogram uses high-pitched sound waves that bounce off the child’s heart, producing moving images that the doctor can see on a video screen.

If your child has tricuspid atresia, the echocardiogram shows that the tricuspid valve is absent and that the right ventricle is smaller than normal. This test, which tracks the flow of blood, also can determine how much blood is moving through the holes in the septum. An echocardiogram also can show related defects of the heart, including a ventricular septal defect or an atrial septal defect.

What are the risk factors for tricuspid atresia?

Risk factors are traits that make a person more likely to develop a disease or condition. Risk factors do not mean that a person will definitely develop a condition, only that the person is more likely to do so. Risk factors for tricuspid atresia include:

  • Down syndrome
  • A parent who had a congenital heart defect
  • A mother who had a viral illness (including German measles) during early pregnancy
  • A mother who drank too much alcohol while pregnant
  • A mother whose diabetes is not properly controlled
  • A mother who used some kinds of medications, such as anti-seizure or acne medications, during pregnancy

What is the cause of tricuspid atresia?

Although there are known risk factors for tricuspid atresia, the exact causes are not known. Congenital heart diseases develop while the baby is in the womb and heart development is taking place.

What are the symptoms of tricuspid atresia?

In most cases, babies born with tricuspid atresia have symptoms within a week after being born. A baby with tricuspid atresia can show the following symptoms:

  • Becoming tired easily
  • Skin and lips that are bluish in color (cyanosis)
  • Shortness of breath
  • Slow growth
  • Heart murmur (unusual heart sounds)

In addition, some babies with this condition can develop heart failure symptoms. These include:

  • Rapid weight gain from retaining fluids
  • Swelling in the abdomen
  • Swelling in the legs, ankles, and feet
  • Weakness and fatigue
  • Irregular or fast heartbeat

How is tricuspid atresia treated?

The potentially fatal complications of this disease can be avoided with prompt treatment. These complications include:

  • Not getting enough oxygen to the body’s tissues
  • A high count of red blood cells, which may cause clots that can lead to stroke or heart attack

The standard surgery used to treat tricuspid atresia is known as the Fontan procedure. During this surgery, the surgeon forms a path that allows oxygen-poor blood that is going back to the heart to flow directly into the arteries that transport blood into the lungs. Most children with tricuspid atresia do not have the Fontan procedure until they are at least 2 years of age. Some children with this defect are not good candidates for this surgery.

What follow-up care is required after treatment?

Your baby will need lifelong follow-up care so that a cardiologist can monitor his or her health. The cardiologist can let you know whether your child has to continue taking preventive antibiotics before procedures such as dental care. Sometimes, the cardiologist will recommend that your child limit physical activity that is too vigorous.

What is the outlook for children with tricuspid atresia?

In general, the short-term and intermediate-term outlook for children who undergo a Fontan procedure is promising. The outlook usually is worse, however, for children who have surgery later in life. Should the Fontan procedure fail, a heart transplant may be needed. Your child’s doctor will evaluate the situation.


This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 6/8/2011…#14789

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