Tricuspid Atresia

Question 1

What is Tricuspid Atresia?

Answer 1

Tricuspid Atresia is a CHD in which the tricuspid valve doesn’t form, but instead a wall forms between the RA and RV.

Question 2

What are the consequences of Tricuspid Atresia? (3)

Answer 2

-Blood can’t flow correctly through the heart and to the rest of the body
-RV is underdeveloped because blood is unable to get from the RA to the RV
-MPA may be small and underdeveloped with very little blood flowing through it to the lungs

Question 3

What else may come with Tricuspid Atresia? (2)

Answer 3

ASD and/or VSD

Question 4

What may infants with Tricuspid Atresia need soon after birth?

Answer 4

Infants with Tricuspid Atresia may need surgery or other procedures soon after birth

Question 5

Is Tricuspid Atresia a critical CHD?

Answer 5

Yes

Question 6

When will infants with Tricuspid Atresia show symptoms?

Answer 6

Infants with Tricuspid Atresia will show symptoms at birth or very soon afterwards

Question 7

What are some signs and symptoms of Tricuspid Atresia? (3)

Answer 7

-Cyanosis
-Problems breathing
-Extreme sleepiness

Question 8

What is cyanosis?

Answer 8

Cyanosis is the condition of having bluish lips, skin, and nails because of low blood oxygen levels.

Question 9

What may Tricuspid Atresia also come with? (4) Why is this?

Answer 9

Tricuspid Atresia may also come with an ASD, VSD, PDA, and/or a d-TGA. These additional abnormalities form because blood needs to use other routes to bypass the unformed tricuspid valve.

Question 10

What is a PDA?

Question 11

What do the ASD, VSD, and PDA help with? What does this result in?

Answer 11

The ASD and VSD help with getting some of the deoxygenated blood to the left side (the LV needs deoxygenated blood to pump to the lungs. The PDA helps with getting (some) deoxygenated blood tot he lungs. This results in a partially normal circulation

Question 12

What does d-TGA help with? What is its disadvantage?

Answer 12

d-TGA helps getting blood to the lungs (PA is connected to the LV), but blood cannot be transported to the body properly (Ao is connected to the RV, which is underdeveloped and disconnected from the RA)

Question 13

What are the exact causes of Tricuspid Atresia?

Answer 13

The exact causes of Tricuspid Atresia among most babies are unknown

Question 14

Why do some infants have CHDs? (1) What are some other risk factors/possible causes of Tricuspid Atresia? (3) What is the conclusion? (2)

Answer 14

-Some infants have CHDs because of changes in their chromosomes and genomes

-What the mother eats or drinks
-What medicines the mother takes
-The mother’s environment and health conditions

Conclusion: A combination of changes in the infant’s genome and other risk factors (above) may increase the risk of the infant getting Tricuspid Atresia

Question 15

What are some treatment options for Tricuspid Atresia? (3)

Answer 15

-Medicines
-Nutrition
-Surgery

Question 16

What are the goals of medicines used to treat tricuspid atresia?

Answer 16

Strengthen the heart muscles. Lower the infant’s blood pressure. Help the body get rid of extra fluid.

Question 17

Why might infants with tricuspid atresia struggle with nutrition?

Answer 17

Infants with tricuspid atresia get tired while eating and may not consume enough nutrition, food, or fat.

Question 18

What is a possible treatment to ensure infants with tricuspid atresia get enough nutrition?

Answer 18

A special high-calorie formula might be prescribed to ensure adequate nutrition and fat intake.

Question 19

What additional assistance might infants with tricuspid atresia require to feed properly?

Answer 19

Infants may need feeding tubes to get enough nutrition.

Question 20

How does the need for surgery depend on tricuspid atresia?

Answer 20

Surgery depends on the severity of tricuspid atresia and the presence of other congenital heart defects (CHDs).

Question 21

What is the purpose of surgery for tricuspid atresia?

Answer 21

Surgeries help increase blood flow to the lungs and bypass the unformed tricuspid valve.

Question 22

Do surgeries for tricuspid atresia cure the condition?

Answer 22

No. These surgeries do not cure tricuspid atresia but they help restore heart function.

Question 23

What is a septostomy, and when is it performed?

Answer 23

A septostomy is a procedure done within the first few days or weeks of an infant’s life to enlarge the atrial septal defect (ASD).

Question 24

What is the purpose of banding in tricuspid atresia surgery?

Answer 24

Banding is used to control blood flow to the lungs if there is another CHD and too much blood is going to the lungs. It is a temporary procedure likely to be removed later.

Question 25

What does a shunt procedure do, and when is it performed?

Answer 25

A shunt procedure is done within the first 2 weeks of life and creates a bypass from the aorta (Ao) to the main pulmonary artery (MPA). It may enlarge the aorta and can still leave the infant with cyanosis due to mixed oxygenated and deoxygenated blood.

Question 26

What is the purpose of the bi-directional Glenn procedure?

Answer 26

The bi-directional Glenn procedure, usually done at 4 to 6 months of age, creates a direct connection between the main pulmonary artery (MPA) and the superior vena cava (SVC).

Question 27

What is the Fontan procedure, and when is it typically performed?

Answer 27

The Fontan procedure is usually performed around 2 years of age and connects the inferior vena cava (IVC) to the pulmonary artery (PA). If done correctly, it eliminates cyanosis.

Question 28

What is the long-term outcome for infants who undergo surgeries for tricuspid atresia?

Answer 28

Surgeries are not a cure. Infants may have lifelong complications.

Question 29

What may be needed if the tricuspid atresia is very complex or the heart becomes weak after the surgeries?

Answer 29

If the tricuspid atresia is very complex or the heart becomes weak after the surgeries, a heart transplant may be needed.

Question 30

What do victims (of tricuspid atresia) who has received a heart transplant need to do?

Answer 30

Victims (of tricuspid atresia) who has received a heart transplant will need medicines for the rest of their lives.

Question 31

What is the purpose of the medicines given to heart transplant recipients?

Answer 31

These medicines help prevent the rejection of the new heart.

Question 32

What kind of follow-up is needed after a heart transplant?

Answer 32

Victims will need regular follow-up visits with a cardiologist.

Question 33

What is the purpose of regular follow-up visits with a cardiologist?

Answer 33

These visits monitor their progress and check for other health conditions that may develop over time.

Question 34

What may adult victims of tricuspid atresia need?

Answer 34

For adult victims (of tricuspid atresia), they may need more surgeries or medical care to address for other possible problems.