Overview

Tricuspid atresia is a congenital (present at birth) heart defect that occurs when the tricuspid valve of the heart doesn’t form. The tricuspid valve is normally between two chambers on the right side of your heart, the right atrium (upper chamber) and right ventricle (lower chamber).

In tricuspid atresia, you have solid tissue instead of the tricuspid valve. This sheet of tissue blocks blood flow from the right atrium to the right ventricle, where blood would normally go. Because of this blockage, the right ventricle usually is small and underdeveloped. The other valve on the right side between the right ventricle and the lungs can also be underdeveloped (pulmonary valve).

Tricuspid atresia types

Healthcare providers put cases of tricuspid atresia into different categories. Up to 80% of people have Type I. Types I and II also have three subcategories based on other heart defects present.

  • Type I: Your baby’s pulmonary artery and aorta (or “great arteries”) are in the correct place. However, they may have a hole in their ventricle wall (ventricular septal defect) or a problem with their pulmonary valve.
  • Type II: Your baby’s pulmonary artery and aorta are in each other’s places. Also, there’s a ventricular septal defect and possibly a problem with your baby’s pulmonary valve.
  • Type III (the rarest type): There are different problems with the position of your baby’s pulmonary artery and aorta and the right and left ventricles.

How does tricuspid atresia affect my body?

Someone with tricuspid atresia can’t get enough blood flowing through their heart and into their lungs, where it would get oxygen. Because of this, their lungs can’t provide enough oxygen to the rest of their body.

In a baby born with tricuspid atresia, blood 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. This hole is always present during fetal life (foramen ovale) but sometimes the hole is big and becomes a heart defect (atrial septal defect).

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

When blood is flowing through these unnatural routes, blood high in oxygen blends with blood low in oxygen. In a normal heart, the two types of blood don’t mix.

Is tricuspid atresia life threatening?

Yes. Tricuspid atresia is one of the serious heart defects that healthcare providers consider critical congenital heart defects. This type of heart disease usually requires care in an intensive care unit with experience in complex congenital heart disease at birth. Frequently, a heart surgery is needed before the baby goes home. Fatal complications occur without diagnosis and treatment.

The difference between tricuspid atresia and Ebstein’s anomaly

Both of these heart valve issues have to do with the tricuspid valve. With tricuspid atresia, there’s no tricuspid valve. With Ebstein’s anomaly, the tricuspid valve is there but doesn’t work right.

Who does tricuspid valve atresia affect?

Tricuspid atresia is rare but is one of the more common complex congenital (present at birth) heart diseases. About 1 out of 10,000 babies born has tricuspid atresia, regardless of gender.

People are more likely to get tricuspid atresia or another congenital heart disease if they have Down syndrome or a parent who had a congenital heart defect.

Other risk factors include having a birthing parent who:

  • Had a viral illness (including German measles) during early pregnancy.
  • Drank too much alcohol while pregnant.
  • Has diabetes that isn’t properly managed.
  • Used certain kinds of medications, such as anti-seizure or acne medicines, during pregnancy.
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Symptoms

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Complications

Blood clots are a dangerous complication of atrial fibrillation (AFib). Blood clots can lead to stroke.
The risk of stroke from AFib increases as you grow older. Other health conditions also may increase the risk of a stroke due to AFib. These conditions include:
  • High blood pressure.
  • Diabetes.
  • Heart failure.
  • Some types of heart valve disease.
Blood thinners are commonly prescribed to prevent blood clots and strokes in people with atrial fibrillation.

Prevention

Healthy lifestyle choices can reduce the risk of heart disease and may prevent atrial fibrillation (AFib). Here are some basic heart-healthy tips:
  • Control high blood pressure, high cholesterol and diabetes.
  • Don't smoke or use tobacco.
  • Eat a diet that's low in salt and saturated fat.
  • Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
  • Get good sleep. Adults should aim for 7 to 9 hours daily.
  • Maintain a healthy weight.
  • Reduce and manage stress.


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