congenital heart disease

Question 1

syndromes with associated CHD sporadic

Answer 1

williams- supra-valvular AS, PS

Question 2

syndromes with associated CHD:

teratogenic:

1. rubella
2. alcohol
3. dilantin
4. lithium

Answer 2

1. rubella- PDA, PS, ASD
2. Alcohol- VSD
3. Dilantin- PS AS, coarctation, PDA
4. lithium- Ebstein’s tricupsid atresia

Question 3

syndromes with associated CHD

chromosomal

1. tri 21
2. tri 13
3. tri 18

Answer 3

1. tri 21: endocardial cushion, TOF
2. tri 13: VSD
3. tri 18: polyvalvular dysplasia

Question 4

cardiac circulation is in

Answer 4

series

Question 5

complex CHD may be in

Answer 5

parallel or mixing

Question 6

ductus arteriosus dependent (requiring protaglandin) (3)

Answer 6

1. pulmonary atresia
2. transposition of the great arteries
3. severe coarctation of the aorta

Question 7

non- duct dependent (3)

Answer 7

1. transposition of the great arteries with a large VSD
2. double outlet right ventricle
3. truncus arteriosus

Question 8

RVOT/obstruction

Answer 8

TOF

Question 9

parallel circulations

Answer 9

TGA

Question 10

common mixing

Answer 10

truncus arteriosus

Question 11

CHD with pulmonary HTN (Eisenmengers)

Answer 11

VSD, ASD and PDA

Question 12

cyanosis generally occurs when (2)

Answer 12

1. there is too little pulmonary blood flow

2. venous blood mixes with arterial blood

Question 13

flow is largely influenced by

Answer 13

resistance and compliance

Question 14

shunts cause

Answer 14

downstream chamber dilation

Question 15

_______ equalize across a large defect/ shunt

Answer 15

pressures

Question 16

obstruction to flow produces

Answer 16

hypoplasia

Question 17

obstruction elevates

Answer 17

upstream proximal pressure

Question 18

the blood flow through an organ can be quantitated if an indicator is either added to or removed from the blood during its flow through the organ

Answer 18

Fick principle

Question 19

Qp/Qs

Answer 19

AO - SVC / AO- PA

Question 20

ASD:

1. common in
2. women or male
3. types
4. volume overload on the
5. eisenmenger’s
   magnitude of shunt relative to

Answer 20

1. common defect in children and 2nd most common in adult
2. women 3:1
3. secundum, venosus and primum
4. right sided volume overload
5. eisenmenger’s rare- 5%
6. magnitude of shunt relative to diastolic distensibility and vascular resistance

Question 21

a shunt in the atrium will cause an

Answer 21

increase in pulmonary blood flow

Question 22

ASD Qp to Qs ratio

Answer 22

Qp > Qs

Question 23

a small VSD will _______ pulmonary blood flow and to a certain extent pressure

Answer 23

increase

Question 24

a large VSD will increase both

Answer 24

blood flow and pressure

Question 25

VSD Qp to Qs ratio

Answer 25

Qp>Qs

Question 26

ASD vs VSD if the amount of pulm. blood flow in both pt. is the same, who will be sicker

Answer 26

ASD- volume overload VSD- volume and pressure overload

Question 27

reversed shunt

Answer 27

eisenmenger's syndrome

Question 28

Eisenmenger's syndrome: 1. what happens over time? 2. as the pulmonary vascular resistance increases the Qp/Qs 3. When ______ blood will shunt from the right heart to the left heart leading to the development of systemic cyanosis 4. associated with

Answer 28

1. increased blood flow/pressure damages the pulmonary arteriole muscular walls causing hypertrophy, leading to elevation of the PVR and a reduction in PBF 2. decreases 3. Qp

Question 29

overall effect in eisenmenger's

Answer 29

resistance to flow in pulmonary circuit now higher than systemic

Question 30

PDA connects the

Answer 30

descending aorta to left pulmonary artery

Question 31

what closes the PDA

Answer 31

after birth - up to 2 weeks with the increase in O2 and PGE

Question 32

PDA becomes the

Answer 32

ligamentum arteriosus

Question 33

aorta constriction typically distal to subclavian artery

Answer 33

coarctation of the aorta

Question 34

COA: 1. causes 2. men or women 3. associated with 4. consider intervention when

Answer 34

1. causes upper extremity HTN leading to cardiac hypertrophy from pressure overload 2. males 2:1 3. BAV (60-80%) 4. consider intervention in persistent HTN and a gradient > 20-30 mmHg

Question 35

most common cyanotic CHD

Answer 35

TOF

Question 36

TOF: 1. has four issues 2. in 25% of the cases we see 3. if it is pentalogy of fallot what do they have in addition to

Answer 36

1. (i) RVOT obstruction (ii) RVH (iii) overriding aorta (iv) VSD 2. right aortic arch - 25% 3. pentalogy of fallot- ASD

Question 37

blue child

Answer 37

TOF

Question 38

TOF: 1. what do we see 2. Qp to Qs

Answer 38

1. output from the right and left ventricles is different 2, Qp

Question 39

rashkind septostomy

Answer 39

emergency procedure that creates an inter-atrial bi-directional shunt prior to performing an arterial switch operation in which the aorta and pulmonary artery are exchanged to connect LV to aorta and RV to pulmonary artery

Question 40

mustard procedure

Answer 40

atrial switch operation

Question 41

tricuspid atresia

Answer 41

single ventricle

Question 42

Qp>Qs

Answer 42

pulmonary blood flow too much

Question 43

Qp

Answer 43

pulmonary blood flow too little

Question 44

Qp=Qs

Answer 44

pulmonary blood flow just right