Anomalies of the Tricuspid Valve: TV Atresia

Question 1

Tricuspid Valve Atresia is defined as:

Answer 1

• Absent tricuspid valve
• A cyanotic lesion
• A single ventricle

Question 2

Tricuspid atresia obstructs flow to the ____.

Answer 2

lungs

Question 3

With tricuspid valve atresia, the baby needs and ___, ____, and ____ in order to have adequate flow to the lungs.

Answer 3

ASD, PDA, VSD

Question 4

With tricuspid atresia, the ____ is small and underdeveloped.

Answer 4

RV

Question 5

The RV is considered ____ in tricuspid atresia.

Answer 5

hypoplastic

Question 6

Why is the Right Ventricle so small with Tricuspid Valve Atresia?

Answer 6

Blood flow forms the chambers of the heart, and with an atretic tricuspid valve there isn’t any flow going through the chamber so it is underdeveloped.

Question 7

The PA and AO can be ____ in tricuspid atresia.

Answer 7

transposed

Question 8

If the baby has tricuspid atresia and no VSD, how does blood get to the lungs?

Answer 8

PDA, connecting the AO and the Pulmonary Arteries

Question 9

Most common form of TV atresia?

Answer 9

No transposition of the great vessels

Small ASD and VSD

Question 10

Clinical findings of TV Atresia?

Answer 10

1. cyanosis
2. cyanosis increases with crying
3. rapid breathing
4. increased HR (150-180 bpm)
5. PHTN
6. Respiratory distress (baby is intubated)

Question 11

Treatment for TV Atresia:

Answer 11

1. Palliative measures taken to increase blood flow to the lungs
2. Blalock-Tausigg Shunt
3. Glen Shunt
4. Fontan Procedure

Question 12

Palliative measures for Tricuspid Valve Atresia:

Answer 12

1. Prostagladin E’ stops PDA from closing

2. Balloon Septostomy keeps the Fossa Ovalis open / give baby an ASD

Question 13

There are a total of ____ surgeries over a ____ year period for patients with TV Atresia.

Answer 13

3, 2

Question 14

The first actual surgical step in treating TV atresia is called the ____ ____.

Answer 14

BT Shunt

Question 15

Steps of BT Shunt procedure:

Answer 15

1. (right or left) subclavian attached to (right or left) pulmonary artery

• mimics the patent ductus arteriosus
• maintains volume supply to the lungs
• at this point, we don’t care about the O2 content of the blood, just the volume
• maintain open ASD

Question 16

The second surgical step in treating TV Atresia is called the ____ ____.

Answer 16

Glen Shunt

Question 17

Steps of the Glen Shunt procedure:

Answer 17

1. BT shunt is removed
2. Attach SVC to the RPA
3. PA is ligated from the RV

• slowly introducing the lungs to a higher pressure
• baby is given medication to control pressure in the lungs, because there needs to be lower pressure in the lungs for blood to flow passively (with no “pump” aka RV)

Question 18

The third surgical step in treating TV Atresia is called the ____ ____.

Answer 18

Fontane Procedure

Question 19

Steps of the Fontane procedure:

Answer 19

1. IVC is ligated from the RA and attached to the SVC
   OR
   Baffle is used to shunt flow from IVC into RPA
2. A baffle is introduced to the RA near the IVC to shunt flow from the IVC to the RPA
3. ASD and VSD are closed
4. PA banding if necessary (decreases diameter, protects lung tissue)

Question 20

The Fontane Procedure is done at age ___ - ___ years because it gives the baby time to grow and develop lung tissue.

Answer 20

2, 3

Question 21

At the end of the TV Atresia treatment, the RV is left and serves what purpose:

Answer 21

None

Question 22

The three surgical stages of the TV Atresia treatment is called: _____ _____

Answer 22

Norwood Procedure