Cyanotic Congenital Heart Disease

Question 1

pathophysiology?

Answer 1

R–>L shunt

Question 2

causes?

Answer 2

• Transposition of great arteries
• Tetralogy of Fallot
• Eissenmenger’s Syndrome

Question 3

pathology of Tetralogy of Fallot

Answer 3

1. Pul stenosis
2. RV hypertrophy
3. overriding aorta
4. VSD

Question 4

murmur heard in ToF?

Answer 4

ejection systolic murmur loudest at pulmonary area

• 2nd IC space, left sternal border

Question 5

presentation of ToF?

Answer 5

• cyanosis
  
  • depends how severe the pul stenosis is- if not that bad baby will be pink
• poor feeding & weight gain
• “tet spells”

Question 6

Ix of ToF?

Answer 6

• Echo
• CXR
  
  • small boot shaped heart

Question 7

what are tet spells?

Answer 7

symptomatic episodes precipitated by crying/exercise etc where the R–>L shunt worsens and causes a cyanotic episode

Question 8

presentation of tet spells?

Answer 8

child will be irritable, SOB & cyanosed

severe spells can lead to loss of consciousness, seizures & death

Question 9

Mx of a tet spell?

Answer 9

• positioning
  
  • older kids- squat
  • younger kids- knees to chest
• oxygen
• beta blockers
• morphine
• IV fluids

Question 10

Rx of ToF?

Answer 10

• definitive
  
  • total surgical repair at 6 months
• temporary
  
  • prostaglandin infusion to keep ductus arteriosus open (allows blood flow back to PA)
  • BT shunt

Question 11

in what direction does the VSD shunt blood in ToF?

Answer 11

R–>L, as higher pressure in right heart because of increased resitance to flow due to pul stenosis

Question 12

what is the pathology behind Transposition of great arteries?

Answer 12

aorta comes off RV, pul artery comes off LV

Question 13

presentation of TGA?

Answer 13

cyanosis at birth or presentation when duct closes

Question 14

what does immediate survival depend on in TGA?

Answer 14

PDA / ASD/VSD in order to allow for oxygenation

Question 15

Mx of TGA?

Answer 15

• temporary
  
  • prostaglandin infusion to keep PDA open
  • balloon septostomy
    
    • enlarging existing ASD
• definitive
  
  • surgical arterial switch repair

Question 16

Pathophysiology of Eisenmenger Syndrome?

Answer 16

occurs due to an underlying structural heart lesion (PDA/VSD/ASD)

• ASD or VSD allows L–>R shunt
  
  • much higher pressure in left side of heart
• pul HT
• when Pulmonary pressure > systemic pressure: R–>L shunt = cyanosis

Question 17

Mx of Eisenmenger?

Answer 17

• correct underlying condition to prevent development
• once syndrome established, it is irreversible

Question 18

Findings O/E relating to R–>L shunt?

Answer 18

• cyanosis
• clubbing
• SOB
• plethoric complexion
  
  • red complexion due to polycythaemia

Question 19

who should be carefully monitored for development of Eisenmenger?

Answer 19

pregnant women with ASD/VSD as it can occur more rapidly during pregnancy

echo for close monitoring