Congenital Heart Disease

Question 1

Atrial Septal Defects:
types
associations

Answer 1

secundum 75% @ fossa ovalis
primum 15-20%  cleft MV
sinus venous 5-10% near svc/ivc
coronary sinus <1%
anomalous venous return

Question 2

ASD sig features as adult

Answer 2

RV enlargement due to excessive pul flow

rv failure with pul infarctions, arrhythmias, pul htn

Question 3

ASD ecg

Answer 3

secundum: right axis deviation
primum: left axis deviation

Question 4

surg vs device for asd

Answer 4

too large for device
septum primum
coronary sinus
primum defect
contrindicated if severe pul htn

Question 5

asd prophylaxis

Answer 5

not indicated for isolated asd

indicated for 6 mths after surgery for asd

Question 6

congenital hd offspring

Answer 6

maternal and paternal
4-10% offspring
general population incidence: 6/1000
fetal echo @22 wks

Question 7

Gerbodie defect

Answer 7

AV canal repair- late complication of lv to la fistulae

Question 8

differential of PDA on echo:

Answer 8

aortopulmonary collaterals
coronary av fistula
ruptured sinus vasalva

Question 9

indication for PDA closure:

Answer 9

lae
lvh
endoarteritis
contraindicated if pul art hypertension

Question 10

subaortic stenosis

Answer 10

may recur post removal
associated: vsd, aortic insufficiency
surg if grad >50mmHg/ 30 mmHg mean

Question 11

supravalvular aortic stenosis:

Answer 11

associated:
Williams syndrome, coronary obstruction, aortic hypoplasia,
renal art stenosis
peripheral pul art stenosis

Question 12

Aortopathies:

Answer 12

Marfans
Loeys-dietz
Beale’s syndrome- ulnar deviation of fingers
Ehlors’ Danlos

Question 13

coarctation associations:

Answer 13

bicuspid aov
subaortic stenosis,vsd
aortic arch
mitral valve
circle of willis cerebral aneurysm

Question 14

coarctation intervention:

Answer 14

> 20 mmhg

look for rib notching on cxr

Question 15

pulmonary valve stenosis:

Answer 15

Noonan’s syndrome
intervention if: grad >60mmHg/ 40mmHg mean
if regurg may need replacement

Question 16

tetralogy of fallot

Answer 16

sub pul stenosis, rvh, vsd, overiding aorta
blalock taussig: sub artery to pul art
waterston cooley - pul art to ao (ruined pul art)

Question 17

associated cardiac defects with tetralogy of fallot

Answer 17

pul art anomalies
asd secundum (pentology of fallot)
av canal defect
right ao arch, cor anomalies
22q 11 deletion (degeorge syndrome)

Question 18

d transposition of great arteries

Answer 18

aorta connected to rv, pul art connected to lv
pda permits mixing oxygenated blood-
raskin procedure- create asd
procedure: mustard- vena cava flow tubed to “la”
newer: switch ao/pa,

Question 19

post Rastelli complication

Answer 19

rv to pa- with asd
conduit obstruction
branch pul stenosis

Question 20

L transposition great arteries

Answer 20

aorta on left, congenital corrected
rv systemic and may fail eventually
watch for complete heart block

Question 21

Ebstein anolmaly

Answer 21

apical displacement of tricuspid valve
“atrialization” of rv
asd/pfo 50%
25% wpw

Question 22

single ventricle

Answer 22

tri, mitral atresia, double inlet lv, hypoplastic lv
surgery to commit single ventricle to systemic circ and seperate pul inflow:
bidirect Glenn- svc to pul art, ivc to pul art
Fontan: ivc to pul art

Question 23

Risk factors for fetal congenital heart disease

Answer 23

Dm, moderate etoh, rubella,maternal hx cong defect, anti convulsive, lithium, warfarin,

Question 24

Trisomy 13

Answer 24

Inslet vsd, hypoplastic left heart

Trisomy 21 primum asd, inlet vsd, tetralogy
Trisomy 18 inslet vsd, hypoplastic left heart

Question 25

Noonan’s

Answer 25

Pulmonic stenosis, coarctation hypertrophic cm

Question 26

Turner syndrome

Answer 26

Aortic stenosis, coarctation