CVPR 326 Pediatric Perfusion / Lecture 1 / Tricuspid Atresia

Question 1

Type 1 Tricuspid Atresia

Answer 1

• 70%
• No tricuspid valve
• Hypoplastic RV
• Normally, the great arteries
  are positioned correctly.

Question 2

Type 1A Tricuspid Atresia

Answer 2

• Pulmonary atresia
• Virtual abscence of the right
  ventricle.

Question 3

Type 1B Tricuspid Atresia

Answer 3

• Pulmonary Stenosis

- Small VSD

Question 4

Type 1C Tricuspid Atresia

Answer 4

• Normal pulmonary valve

- Large VSD

Question 5

Type II Tricuspid Atresia

Answer 5

30% of the cases have

transposed great arteries.

Question 6

Type IIA Tricuspid Atresia

Answer 6

• transposed great arteries

- Pulmonary Atresia

Question 7

Type IIB Tricuspid Atresia

Answer 7

• transposed great arteries
• pulmonary or subpulmonary
  stenosis

Question 8

Type IIC Tricuspid Atresia

Answer 8

• transposed great arteries
• normal or enlarged
  pulmonary valve and artery without subpulmonary stenosis.

Question 9

Stenosis

Answer 9

the abnormal narrowing of a passage in the body.

Question 10

4 Tricuspid Atresia Options for Repair

Answer 10

• BT Shunt
• Glenn/Bidirectional Glenn
• Fontan
• TCPC

Question 11

What happens if Tricuspid Atresia is not corrected ?

Answer 11

increase risk for
Eisenmenger’s Physiology
– Left to right shunt switches to right to left shunt
– No surgical procedures to correct this.

Question 12

Blalock-Taussig Shunt for Tricuspid Atresia.

Answer 12

Palliative care for Tricuspid Atresia by placing a shunt that connects the brachiocephalic artery to the right pulmonary artery.

Question 13

Eisenmenger’s Physiology

Answer 13

Way to much circulation to the lungs which causes a right to left blood shunt. This leads to the PVR to become SUPER SYSTEMIC which is irreversible.

Question 14

Glenn Anastomosis for Tricuspid
Atresia 
Restrictive ventricular septal defect 
Small atrial septal defect 
(vs. patent foramen ovale)

Answer 14

SVC to the right pulmonary artery only. This means that the right Pulmonary artery was divided, SVC was also divided from the RA, and then the SVC was connected to the Right pulmonary artery. Flow from the SVC > Right PA > Lung >PV >LA > LV & RV via VSD > from RV > PA > Left Lung

Question 15

Bi-dirrectional Glenn

Answer 15

Same as the original glen procedure, except the right pulmonary artery was not divided from the left, it therefore flow into both lungs.

Question 16

What anatomy is removed during Palliative Bidirectional Glenn procedure?

Answer 16

• BT Shunt

- Pulmonary Root

Question 17

Fontan Operation for Tricuspid

Atresia (AKA: TCPC)

Answer 17

• RA anastomosed to the PA
• VSD if present is closed
• ASD is closed
• Passive Filling of the lungs
• ↑ CVP (20-30 mmHg)
  throughout pts life
• Edametous

Question 18

TCPC

Answer 18

TOTAL CAVALPULMONARY CONNECTION
- Same Physiology as the Fontan Procedure but done differently.
1.) Bi-Directional Glenn performed at 4-6 months old
(SVC > Intact Right PA )
2.) Intracardiac baffle or Extracardiac conduit
(IVC > Baffle or Conduit > Intact Right PA )

Question 19

Do you need CPB to perform a GLENN procedure?

Answer 19

NO

Question 20

Rastelli

Answer 20

DON’T have Tricuspid Atresia
They DO have a ventricle, and therefore this is not FONTAN physiology.

Rastelli procedure is utilized under some or all of the following conditions.

• Pulmonary Atresia
• Pulmonary stenosis
• Supravalvular Pulmonary
  Stenosis
• Subvalvular Pulmonary
  Stenosis
• VSD is closed

Procudure:
- CPB, Cross-Clamp required. - – Right ventriculotomy is
performed, obstructions are
excised, and VSD is closed.
- Valved homograft is sutured onto the Right Ventricle > Pulmonary Artery

Question 21

How would you repair Truncus Arteriosus?

Answer 21

1. ) Repair the VSD

2. ) Perform a Rastelli Procedure