Congenital Defects

Question 1

Congenital heart defects usually arise during this embryological time period

Answer 1

3-8 weeks

after 8 weeks, the heart has its basic structure

Question 2

What type of shunt leads to cyanosis at birth?

Answer 2

right-to-left shunt

pumping blue blood out into systemic circulation

Question 3

What type of shunt leads to cyanosis at some point, but not right after birth?

Answer 3

left-to-right shunt

pumping red blood into lungs again isn’t that bad

until it reverses (Eisenmenger syndrome) due to hypertrophy on right side

now it’s a right-to-left shunt and you’re pumping blue blood into circulation

cianosis

Question 4

Ventricular septal defect

associated condition

Answer 4

fetal alcohol syndrome

Question 5

Atrial septal defect

associated condition

Answer 5

trisomy 21/Down syndrome

persistant primary atrial foramen espeically

Question 6

Patent ductus arteriosis

condition association

Answer 6

congenital rubella

Question 7

Transposition of the Great Vessels

condition association

Answer 7

materal diabetes

not gestational, since sets in after 8+ weeks

heart is fully developed by 8 weeks

Question 8

Truncus Arteriosis

condition association

Answer 8

22q11

DiGeorge Syndrome

Question 9

Coarcation of the Aorta

condition association

Answer 9

Turner syndrome

(XO instead of XX or XY)

Question 10

Ventricular septal defect

pathology

Answer 10

left -> right shunt

Eisenmenger syndrome later in life

Question 11

Atrial septal defect

pathology

Answer 11

most common: persistant 2˚ atrial foramen

left-right shunt

paradoxical emboli are important complication

(DVT can cause stroke because it crosses from right atrium into left atrium and out to the brain)

Question 12

22q11

congenital heart disease

associations

Answer 12

truncus arteriosis

tetralogy of Fallot

Question 13

Most common congenital heart condition

Answer 13

ventricular septal defect

Question 14

Atrial septal defect on auscultation

Answer 14

split S₂

increased blood in right heart delays closure of pulmonary valve

Question 15

Murmur in patent ductus arteroisis

Answer 15

constant, machine-like murmur

Question 16

4 characteristics of tetralogy of fallot

Answer 16

stenosis of right ventricular outflow tract

right ventricular hypertrophy

ventricular septal defect

overriding aorta

Question 17

What substance maintains patency of PDA

Answer 17

PGE

NSAIDs given to close PDA by decreasing PGE syntesis

PGE2 given to maintain PDA patency in case of transposition of the great vessels

Question 18

Important complication of atrial septal defect

Answer 18

paradoxical emobli

DVT –> right atrium –> through atrial septal defect –> left atrium –> left ventrical –> embolus in systemic circulation, such as brain

Question 19

How do patients learn to compensate with tetralogy of Fallot?

Answer 19

squat

increases peripheral arterial resistance, increases flow to lungs

Question 20

‘Boot-shaped’ heart on X-ray is a finding in which heart condition?

Answer 20

tetralogy of Fallot

apparently the switched arteries make it look like that ?

Question 21

How can someone survive at all with transposition of the great vessels?

Answer 21

they need to keep the ductus arteriosis open

PGE administration

may also have atrial septal defect

Question 22

Truncus arteriosis pathology

Answer 22

single large vessel arising from both ventricles

failure of truncus arteriosis to divide

associated with 22q11

Question 23

Tricuspid atresia pathology

Answer 23

atrioventricular orifice fails to develop

often associated with atrial septal defect

Question 24

Coarcation of the aorta

findings

Answer 24

low ankle:brachial index

lower extremity cyanosis

rib notching on X-ray

(from erosion)

Question 25

Infantile form

coarcation of the aorta

Answer 25

distal to aortic arch

proximal to PDA

Question 26

Adult form of coarcation of the aorta

Answer 26

not associated with PDA

occurs distal to aortic arch