2 - Paeds - CVS - Transposition of the Great Arteries

Question 1

What happens? blood flows? how is this compatible with life?

Answer 1

aorta and pulmonary artery are switched
RV > Aorta
LV > PA
blue blood >body
pink blood > lungs
!!!!!
2 parallel circulation only comp with life if mixing -> VSD, ASD, PDA - other anomalies

Question 2

Sx + presentation

Answer 2

cyanosis, may be profound/fatal
usually present when duct closes (day 2) -> less mixing
Cyanosis less severe/present later if assoc. anomalies eg ASD

Question 3

signs

Answer 3

cyanosis
loud single HS2
usually no murmur unless inc flow/stenosis in left outflow (systolic)

Question 4

Ix - CXR?

Answer 4

CXR - narrow upper mediastinum with ‘egg on side’ appearance of cardiac shadow, marked pulm vasc due to inc pulm bf

Question 5

Ix ECG + ECHO

Answer 5

ECG normal

Echo shows anatomy

Question 6

MGMT - if sick/cyanosed neonate? how to allow mixing if no communication?

Answer 6

improve mixing - prostaglandin - maintain DA patency

balloon atrial septostomy (life saving, 20% cases) to open foramen ovale

Question 7

MGMT - all pts need?? what is done and when?

Answer 7

surgery

neonatal period - arterial switch - coronary arteries transferred to new aorta

Question 8

Eisenmenger syndrome - what is it

Answer 8

chronic untreated high pulm bf due t L->R shunt/common mixing, causes pulm arteries to become thick walled and more resistant
Shunt decreases - reduces Sx - until 10-15y, shunt reverses -> blue teenager = eisenmenger

Question 9

Eisenmenger syndrome - prognosis and treatment

Answer 9

Tx - prevention or > palliation or transplant

progressive, adult will die in Right HF in 4/5th decade