Peds Test 7

Question 1

Transposition of the Great Arteries (TGA)

Answer 1

aorta comes from RV, and pulm from LV
-has to have L-R shunt (VSD, ASD, or PDA)
= hypoxia, hyperventilation, pulm edema, CHF, myocardial depression
-embryology= bulbs cords defect= improper spiraling day 29 (no twisting)
——bulbus cords give rise to GAs (trunks arteriosus= aorta and conus cordis= PA)
-5% cases of CHF and 3x more common in boys

Question 2

2 Types

Answer 2

1) D-transposition= aorta is anterior and right of PA (60%)
2) L-transposition = aorta is anterior and left of PA (“congenitally corrected TGA”), this one is asymptomatic-aorta gets O2 blood

Question 3

Palliative Treatment

Answer 3

• atrial septectomy

- prostaglandin E = to keep PDA (increase Pulm BF, L-R shunting)- important for LVOT obstruction

Question 4

2 Corrective Surgeries

Answer 4

1) physiological procudre= atrial switch
- Mustard (baffle with pericardium) Senning (with atrial tissue)= reverses the blood flow in the heart
- takes IVC/SCV return to left heart (which goes to pulm, back to right, then to aorta)-but Right heart not meant for systemic circulation
- complications= baffle stenosis, pulm HTN, AV regurge, decrease RV function
2) Anatomic repair= arterial switch operation (ASO)
- Jatene= switching the Ao and Pa trunks (and moving coronaries)
- Le Compte Maneuver= TGA with LVOT obstruction= make outflow bigger and switch trunks
- not good with intact VSDs and LVOT obstruction = may do with Rastelli
- CPB: cannulate aortic, single atrial or bicaval (24-28 degrees)
- CPG- antegrade, retrograde, ostial (be careful because you don’t want to blow out new stitches)
- leave chest open, may need ECMO, reduce myocardial edema, leave chest open until reduced swelling

Question 5

L-TGA

Answer 5

-ventricle transposition
-asymptomatic unless with CHD
= CHD if RV fails to pump for systemic circulation
-Treatment- double switch
–Mustard/Senning + Arterial Switch
–Mustard/Senning + Rastelli

Question 6

Truncus Arteriosus Defect

Answer 6

-single outflow tract with VSD and one big common valve
-cyanotic, pulm edema/HTN, clubbing
-embryology= failed separation of trunks arteriosus= conotruncal separation (Ao and Pa together)
Type 1) one pulm artery, two lateral PA off that one
Type 2) 2 PAs posterior or common trunk
Type 3) 2 PAs lateral off trunk (they are independent of each other)

Question 7

Palliative Correction

Answer 7

PA banding to decrease PA flow and increase systemic flow

Question 8

Surgical Repair

Answer 8

complete repair with Rastelli and close VSD

• seperate PA
• conduit from RV to PA with valve
• close VSD
• —-CPB- can aortic and bicaval, moderate temp, CPG- antegrade
• keep chest open until reduced swelling
• watch blood gases improve