Cyanotic Heart Defects

Question 1

tetralogy of fallot - what is it?

Answer 1

right ventricular tract outflow obstruction/pulmonary stenosis
VSD
aortic over-ride
RVH

Question 2

most common cyanotic lesion in children who survive infancy

Answer 2

tetralogy of fallot

Question 3

ToF - risk factors

Answer 3

maternal rubella / viral disease

Question 4

ToF pathophys

Answer 4

right ventricular outflow tract obstruction dictates degree of shunting

• minimal obstruction = CHF
• mild obstruction = equal L/V pressures = pink Tet (not a lot of shunting)
• severe obstruction = cyanosis = no pulm blood flow

Question 5

what is a tet spell?

Answer 5

• context: occur in the AM when SVR is low

- precipitating factors: hot baths, fever, drugs that lower SVR

Question 6

what happens during a tet spell?

Answer 6

cyanosis (due to R to L shunting)
syncope/seizures
cardiac arrest

Question 7

tetralogy of fallot X-ray

Answer 7

boot-shaped, low pulm markings, right aortic arch

Question 8

treatment for tetralogy of fallot

Answer 8

• complete repair surgically:
• VSD
• relief of RTOT
• ligation of shunts
• ASD/PFO closure

Question 9

mortality w/o repair of ToF

Answer 9

50% by 3
90% by 20
95% by 30

Question 10

most common cyanotic lesion of the newborn period

Answer 10

transposition of the great vessels

Question 11

what is transposition of the great vessels

Answer 11

pulmonary arteries + aorta switched

no oxygenation of blood

Question 12

how do these babies survive?

Answer 12

• ASD, VSD, or PDA - to survive

- PDA alone not sufficient to survive

Question 13

transposition of the great vessels presentation

Answer 13

CHF in 1st week of life

Question 14

transposition of the great vessels CXR

Answer 14

cardiomegaly

egg-shaped heart + narrow mediastinum

Question 15

signs/symptoms of transposition

Answer 15

no murmur
single S2
early cyanosis

Question 16

treatment of transposition

Answer 16

prostaglandin - keep PDA open
balloon atrial septostomy
arterial switch procedure

Question 17

truncus arteriosus

Answer 17

bottom line: mixing o2 blood + non-O2 blood

single arterial trunk emerging from the ventricles - pulmonary + systemic circulation (co-existing VSD)

Question 18

what is truncus arteriosus associated w/

Answer 18

DiGeorge’s syndrome

Question 19

signs/symptoms of truncus arteriosus

Answer 19

CHF / cyanosis w/in 1st wk

L–> R shunt - dyspnea, respiratory infxn, failure to thrive

Question 20

heart sounds of truncus arteriosus

Answer 20

S2 louder + prominent (only 1 valve closing)

Question 21

chest x-ray truncus arteriosus

Answer 21

cardiomegaly + incr. lung markings

Question 22

treatment

Answer 22

closure of the VSD + pulm artery reconnect

Question 23

hypoplastic left heart syndrome

Answer 23

aortic valve stenosis or atresia +/- mitral valve stenosis/atresia

Question 24

which heart conditions present w/ cyanosis in the 1st wk of life?

Answer 24

hypoplastic left heart syndrome
truncus arteriosus
transposition of the great vessels

Question 25

treatment of hypoplastic left heart syndrome

Answer 25

-nothing. you’re just gonna die.