Persistent Truncus Arteriosus & TAPVR

Question 1

What is persistent truncus arteriosus?

Answer 1

In this condition, the embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta. There is one large trunk with one large truncal valve.

Question 2

What goes wrong in fetal development to cause persistent truncus arteriosus?

Answer 2

There is failure of truncal septation & EC defect.

Question 3

What is Type I?

Persistent Truncus Arteriosus

Answer 3

Single PA trunk rises from one large main trunk.

Question 4

What is Type II?

Persistent Truncus Arteriosus

Answer 4

The RPA & LPA come off the main trunk posteriorly, not off the PA. There is no true PA.

Question 5

What is Type III?

Persistent Truncus Arteriosus

Answer 5

The RPA & LPA come off laterally from the aortic arch.

Question 6

What is Type IV?

Persistent Truncus Arteriosus

Answer 6

The RPA & LPA come off the descending AO.

Question 7

True or False: There are is no RVOT or LVOT in persistent truncus arterosus?

Answer 7

True

Question 8

Is persistent truncus arteriosus compatible with life?

Answer 8

No, you will only see it in babies not in adults.

Question 9

What are some additional characteristics of persistent truncus arteriosus?

Answer 9

• Large VSD
• Large truncal valve (may have 4-5 cusps)
• Abnormal coronaries

Question 10

What effect does one trunk supplying both the systemic and pulmonary circulation have on lungs?

Answer 10

Pressure in the lungs increases due to increased flow rate from both ventricles contributing to stroke volume. This will cause pulmonary HTN.

Question 11

What are the treatments for persistent truncus arteriosus?

Answer 11

• Close ASD & VSD
• Separate the PA from the arterial trunk which will be used for the AO.
• If there is no PA they will make one with a gortex graft
• Valve replacements

Question 12

What is TAPVR?

Answer 12

It stands for Total Anomalous Pulmonary Venous Return: it is abnormal placement of the pulmonary veins.

Question 13

What is partial TAPVR?

Answer 13

One pulmonary vein connects to the RA, only 3 connect to the LA. It can go undetected because it doesn’t cause too much of an impact.

Question 14

What is total TAPVR?

Answer 14

All oxygenated blood goes from pulmonary veins into the right heart. It is not compatible with life (you hope the Pt has a ASD).

Question 15

What are the 3 types of TAPVR?

Answer 15

1. Supra cardiac
2. Intra cardiac
3. Infra cardiac

Question 16

What is Supra cardiac TAPVR?

Answer 16

Pulmonary vein flow to an innominate vein. Oxygenated blood goes through an innominate vein into the SVC to the RA and through the ASD to the LA.

Question 17

What is Intra cardiac TAPVR?

Answer 17

Pulmonary vein flow goes to the coronary sinus to the RA through the ASD to the LA.

Question 18

What is Infra cardiac TAPVR?

Answer 18

Pulmonary veins are not attached to the LA. Oxygenated blood goes through another descending vein to the IVC. Left heart does not receive oxygenated blood. Baby needs to have ASD, VSD, and PDA. Only way for blood to go systemically is through the PDA. Otherwise it is not compatible with life.

Question 19

What are the treatments and surgeries for TAPVR?

Answer 19

It is dependent on the number of pulmonary veins with abnormal attachment and surgery will be performed in 1-2 months.

• ASD, VSD & PDA closed
• Pulmonary veins reattached to LA
• May use a baffle to redirect flow towards the MV
• If there are any defects in the PVs may be corrected with gortex grafts.