Truncus Arteriosus / Interrupted aortic arch / A-P window

Question 1

Conotruncal Septation fails to separate the truncal artery into the Aorta & Pulmonary arteries during gestation.

Answer 1

Persistent Truncus Arteriosus

Question 2

The spectrum of severity for Truncus Arteriosus is based on what ?

Answer 2

The origin of the pulmonary arteries from the truncal artery.

Question 3

Type 1 Truncus Arteriosus

• VSD
• ASD vs. PFO
• Left Aortic Arch

Answer 3

Main PA arises from the Truncal Artery

Question 4

Type II Truncus Arteriosus

• VSD
• ASD
• Left Aortic Arch

Answer 4

• There is no Pulmonary Trunk. - Branch pulmonary arteries rise separately but in close proximity in posterior aspect of the Truncus Arteriosus.

Question 5

Type III Truncus Arteriosus

• VSD
• ASD
• Left Aortic Arch

Answer 5

• There is no Pulmonary Trunk.
• Branch pulmonary arteries rise widely separate from the lateral aspects of the Truncal Artery.

Question 6

Type IV Truncus Arteriosus

• VSD
• ASD
• Left Aortic Arch
• Separate Systemic Collaterals to each lung.

Answer 6

No communication identifiable between the heart and the native pulmonary arteries, and the lungs are perfused via
collateral arteries

Question 7

In Pseudo truncus there is

Answer 7

pulmonary atresia

and a VSD

Question 8

Describe the total surgical repair of Truncus Arteriosus ?

Answer 8

1. ) PA is separated from the Truncal Root, truncal root repaired.
2. ) Right Ventriculotomy in the right ventricular outflow tract.
3. ) Valved Homograft Conduit implanted to connect the RV to the Pulmonary Arteries.
4. ) VSD repaired

Question 9

Truncus Arteriosus with Interrupted Arch. Describe this congenital defect?

Answer 9

Truncal Artery gives rise to the Aorta & Pulmonary arteries, except now with an interrupted Arch which originates as a ductus arteriosus that now supplies the lower half of the body.

Question 10

Closure of the continuation of the ductus arteriosus in a newborn will result in what?

Answer 10

• CHF

- decreased perfusion of all areas supplied by the descending aorta.

Question 11

How can we prevent closure of the continuation of the ductus arteriosus in a newborn ?

Answer 11

Prostaglandins (PGE1)

Question 12

Surgical repair for the Interrupted Aortic Arch ?

Answer 12

• CBP
• Cross-clamp
• DHCA
• End-to-end Anastamosis

Question 13

Communication between the ascending Aorta & the Pulmonary Artery describes what defect ?

Answer 13

Aortopulmonary Window

which results in a large Left to Right shunt.

Question 14

The Left to Right Shunt through the Aortopulmonary window results in what physiological complications?

Answer 14

• Increased pulmonary artery blood flow.
• LA & LV volume overload
• CHF
• Decreased C.O.