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Tricuspid Atresia

Tricuspid atresia is a rare congenital heart disease. It is a birth defect in which the valve (tricuspid valve) between two of the heart’s chambers isn’t formed.

What is tricuspid atresia?

The heart is divided into four chambers: the right atrium, right ventricle, left atrium, and left ventricle. The right side of your heart circulates blood to your lungs, where the blood is oxygenated before it circulates to the heart’s left side.

Valves control the blood flow into and out of the heart. These valves open allowing your blood to move to the next chamber or one of your arteries, and close to keep the blood from flowing backward.

In tricuspid atresia, the right side of your heart cannot pump enough blood to your lungs as the tricuspid valve is missing. There is blockage of blood flow from the right atrium to the right ventricle of the heart. Consequently, the right ventricle is generally small and underdeveloped (hypo plastic). Due to this, blood fails to reach the lungs to get oxygenated, which causes low oxygen levels in the body. 

Blood flows from the right to the left atrium through a hole situated in the wall between them (septum). This hole is either a defect of the heart (atrial septal defect) or an enlarged natural opening which is supposed to get closed soon after the birth (patent foramen ovale). 

After blood flows to the left atrium, it goes into the left ventricle and then is pumped to the aorta. To get into the lungs, the blood flows through a vessel which connects the aorta to the pulmonary artery (ductus arteriosus) Many infants born with tricuspid atresia also have a hole between ventricles (ventricular septal defect). 

The different types of tricuspid atresia are:

  • Large VSD: A large ventricular septal defect means that the hole in your baby’s heart is too big. This may cause too much of  blood to flow into the lungs and lead to congestive heart failure.
  • Moderate VSD: In this type, the hole in your baby’s heart is medium-sized. There is some flow of blood to the lungs and the baby will be in less distress compared to the ones having a large or small VSD.
  • Small VSD: In this type, the hole is too small and does not allow the blood to flow to the lungs and pick up oxygen. A lack of oxygenated blood may cause a newborn’s skin to turn blue.

What are the symptoms of tricuspid atresia?

If your baby is born with tricuspid atresia, he or she may experience symptoms such as:

  • Slow growth
  • Shortness of breath or breathing difficulty
  • Tiring easily
  • Cyanosis — a blue tinge on the skin and lips
  • Poor weight gain

Some babies born with this condition may also develop symptoms similar to heart failure such as:

  • Fatigue
  • Weakness
  • Swelling on legs and feet
  • Fluid retention
  • Swelling of abdomen
  • Sudden weight gain

These symptoms will become evident soon after birth and may require immediate medical treatment.

What causes tricuspid atresia?

Tricuspid atresia is a congenital birth defect. This condition is generally seen in patients who have family histories of congenital heart defects. However, several other factors may also put your baby at risk of developing this condition. They are:

  • Illness during pregnancy: Suffering from German measles or other viral illness during pregnancy may increase the risk of tricuspid atresia in your baby.
  • Medications: Certain medications for controlling acne like isoretinoin  or seizures may also put your baby at risk.
  • Smoking or consumption of alcohol during pregnancy may increase the risk.
  • Poorly controlled diabetes during pregnancy may also cause developmental issues in the baby.
  • The infant is born with Down syndrome, a genetic disorder which results from an extra 21st chromosome

When do you need to see a doctor?

If you or any of your relatives suffers from a congenital heart defect, make sure you visit your doctor regularly while pregnant. After your child’s birth, if you notice any of the signs for tricuspid atresia mentioned above, consult your doctor immediately. 

Request an appointment at Apollo Hospitals. 

Call 1860-500-1066 to book an appointment.

What are the treatment options for tricuspid atresia?

If your baby is born with this health condition, the doctor may recommend surgery options. 

There may be a need for multiple surgeries along with medications to control blood pressure. 

Some of the common surgical procedures used to treat tricuspid atresia are as follows:

Medications

Before performing any surgery, the cardiologist of your child may recommend your child to take hormone prostaglandin to help dilate (widen) and keep the ductus arteriosus open.

Surgeries or other procedures

A few procedures used for treatment of tricuspid atresia are just a temporary fix to increase blood flow (palliative surgeries). Surgeries that may be needed include:

1. Atrial septostomy: It is a rare procedure in which a balloon is used to create an enlargement of the opening between the heart’s upper chambers for easy blood flow 

2. Shunting: This procedure is like bypass surgery. It involves the creation of a bypass from a main blood vessel exiting the heart to the pulmonary artery which leads to the lungs. It helps with the flow of blood from the heart to the lungs for proper oxygenation..

3. Glenn operation: The age group for this surgery is 3 to 6 months. When the babies outgrow first shunt, they often need this surgery that sets a stage for more permanent corrective surgery known as the Fontan procedure 

4. Fontan procedure: A path is created for the blood that returns to the heart in order to flow directly into the pulmonary arteries, which later transports the blood into lungs

5. Pulmonary artery band placement: If the baby has a ventricular septal defect and too much blood is flowing into the lungs from heart, the surgeon may place a band around pulmonary artery to reduce the blood flow.

What are the complications?

A life threatening condition of tricuspid atresia for your baby is lack of oxygen his/her tissues (hypoxemia).

Later in life , it may lead to serious health complications such as:

  • Irregular heartbeat
  • Kidney problems
  • Liver problems
  • Blood clots
  • Stroke

What are the preventive measures you can take?

You can take some preventive measures to reduce your infant’s risk of developing birth defects. Some of them are:

  1. Consume 400 micrograms of folic acid daily. It is beneficial for the development of the fetal brain as well as helps cut down the risk of developing heart defects. 
  2. Do not smoke or drink during pregnancy.
  3. Avoid exposure to toxic chemicals present in the cleaning agents or paints.

Check with your doctor about any medication use in pregnancy . 

Conclusion

Tricuspid atresia is a rare and severe medical condition. However, by opting for surgery, this condition can be treated. Manage your health and consult your doctor regularly during pregnancy to avoid the development of this condition in your baby.

Frequently Asked Questions (FAQs)

Is tricuspid atresia curable?

There is no cure for this condition. Your infant can improve life quality after undergoing necessary surgeries. 

Is tricuspid atresia genetic?

Studies suggest that tricuspid atresia may be genetic. If one of the partners has a family history of tricuspid atresia, an infant is most likely to develop this birth defect.

Will my baby require another surgery as he grows up?

Yes, your baby may have to undergo another surgery to ensure proper flow of blood through the heart as he grows up.

How is tricuspid atresia diagnosed?

Your doctor may perform several tests like ultrasound, electrocardiogram and MRI to diagnose this condition in your baby.

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