2 - Paeds - CVS - Coarctation of Aorta

Question 1

how does it happen?

Answer 1

when DA closes, aorta can also be constricted - severe LV outflow obstruction (and commonest cause of collapse)

Question 2

classical presentation of neonate

Answer 2

1st day usually normal

acute circulatory collapse and shock when duct closes on day 2

Question 3

physical signs

Answer 3

sick baby, severe HF, absent femoral pulses, severe metabolic acidosis
?murmur ULSE

Question 4

Ix

Answer 4

CXR -cardiomegaly from HF and shock

ECG - normal

Question 5

mgmt - 3 steps

Answer 5

resuscitate
prostaglandins
surgical repair

Question 6

surgical approaches? what about balloon angioplasty?

Answer 6

angioplasty =/- stent to correct the area

sometimes open surgery - resection, anastomeses, bypass graft etc

balloon angioplasty appears to only buy time

Question 7

3 associations with coarctation of aorta

Answer 7

bicuspid aortic valve
VSD
Turners syndrome (females, only one X chromosome)

Question 8

describe bicuspid aortic valve

Answer 8

most common congenital valve deformity - 1-2% of population
often aSx - can calcify later in life > varying stenosis and murmur, eventually > aortic regurg and need for surgery

these patients lack stamina and tire easily