Cardiac embryology and congenital heart disease II Flashcards

1
Q

which gender more likely to have severe congenital cardiovascular malformation

A

boys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

risk factor for congenital cardiovascular malformation

A

maternal diabetes

fhx of cardiac defect in parent/sibling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

if maternal diabetes how do you screen for congenital CV malformation

A

fetal echo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Types of ASD

A

1) secundum ASD

2) sinus venosus ASD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

embyrological basis of secundum ASD

A

either
) too large central hole (ostium secundum) in septum primum

or
1) inadequate development of septum secundum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is PAPVR

A

sinus venosus ASD with partial anomalous pulm veins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

magnitude and direction of shunt in ASD depends on (2)

A

1) size of defect

2) inflow resistance to RV vs LV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what type of shunt across an ASD

A

left to right

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

why left to right shunt across ASD

A

LA pressure higher than RA pressure

LA and RA pressure equalize if ASD large

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

when would ASD shunts be left to right

A

1) RV thinner and compliance greater than LV (normal)
or

1) systemic vascular resistance > pulm vascular resistance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

why does ASD rarely present in infancy

A

LV and RV myocardium similar after birth

similar inflow resistance, minimal atrial level shunt –> min sx

as get older, pulm vascular resistance decr, RV wall thin —> get left to right shunting incr

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

physical exam of ASD

small defect with no/minimal shunt OR NEONATE

A

1) depends on degree of shunting

normal exam

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

physical exam of ASD

large defect

A

1) rales
2) sweating with feed but maybe asymptomatic
3) liver 2-3 cm below right costal margin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

physical exam of ASD

large defect

murmur

A

2-3/6 systolic ejection murmur at left upper sternal border

and/or diastolic rumble at left lower sternal border

S2 widely split

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

murmur ____ related to blood flowing across defect

A

NOT b/c pressure differential too small

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

why do you have systolic murmur with ASD

A

XS blood flow across pulm valve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

why do you have diastolic rumble with ASD

A

XS blood flow in diastole across tricuspid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what causes physiologic S2 split

A

inspiration

negative intrathoracic pressure, incr right heart filling, delay RV empty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what causes S2 split in ASD

A

RV volume overload –> delayed RV empty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

ECG of ASD

A

Right axis deviation

RVH (rsR’ or qR in V1/V2)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

if you see

RAD
RVH

what abnormality

A

ASD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

how to diagnose ASD

A

1) CXR
large main pulm artery
prominent pulm vascular markings

2) echo (size, location, magnitude of shunt)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

progression of ASD

A

undetected in childhood

risk of hemodynamically significant ASD

  • pulm vasc disease
  • atrial arrhythmia
  • cardiac failure
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

describe pulm vascular disease (sx of ASD)

A

high pulm blood flow

more common and earlier age at higher altitudes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

where is pulm vascular diseases most common

A

higher altitudes

26
Q

describe atrial arrhythmias (sx of ASD)

A

older patients due to atrial enlargement

27
Q

describe cardiac failure (sx of ASD)

A

> 20 y/o with large shunts or with pulm vascular disease

RIGHT HEART FAILURE

  • HEPATOMEGALY
  • VENOUS CONGESTION
28
Q

medical therapy for ASD

A

1) diuresis for dyspnea

2) CLOSE THE HOLE FOR OLDER CHILDREN W/ ASD or symptomatic infants

29
Q

how to close hole in ASD

A

surgery

percutaneous device closure

30
Q

normal ventricular septation

A

post-loop
day 28-42

intraventricular septum grows toward base of heart as ventricular outpouch develop –> 4 endocardial cushions

31
Q

what is anatomic correlate of superior endocardial cushion

A

1) left surface of outlet of Interventricular septum

2) mitral valve

32
Q

what is anatomic correlate of inferior endocardial cushion

A

1) inlet of interventricular septum
2) membranous portion of interventricular septum
3) tricuspid and mitral

33
Q

what is anatomic correlate of right endocardial cushion

A

tricuspid valve

34
Q

what is anatomic correlate of left endocardial cushion

A

posterior leaflet of mitral valve

35
Q

what is considered a large VSD?

A

same diameter as aortic orifice

36
Q

large VSD are often ____

A

unrestrictive

equalization of LV and RV pressure

37
Q

magnitude of VSD depends on (3)

A

1) size of defect
2) systemic and pulm vascular resistance
3) right or left heart obstructive lesions (pulm or aortic stenosis)

38
Q

physiologic consequences of PVR < SVR

A

1) blood to lungs (L–> R shunt)
2) incr pulm blood flow to LA
3) incr LV EDV
4) incr muscle fiber length
5) incr LV contractility, incr LV output

39
Q

VSD clinical presentation

A

asymptomatic until PVR falls after birth

delayed at elev altitudes

40
Q

VSD clinical presentation

large VSD

A

1) respiratory distress

2) diaphoresis with feeds

41
Q

VSD clinical presentation

small VSD

A

1) tachypnea

2) mild or no diaphoresis

42
Q

VSD physical exam

large VSD murmur

A

1) accentuated S2
2) 2-3/6 harsh, holosystolic murmur at left lower sternal border but also throughout
3) diastolic murmur d/t incr flow across mitral valve

43
Q

where do you hear large VSD murmur

A

left lower sternal border

44
Q

VSD physical exam

small VSD murmur

A

1) normal S2
2) 2-4/6 early systolic murmur
3) no diastolic murmur

45
Q

what would it mean if

murmur gets larger`

A

1) closing/restrictive VSD

2) low PVR

46
Q

what would it mean if

murmur goes away

A

1) large VSD with equalization of RV and LV pressure

2) incr PVR

47
Q

how to diagnose VSD

A

1) echo - location, magnitude of shunt
can see assoc aortic insufficiency

2) ECG normal in small
R axis deviation and LVH and RVH

48
Q

ECG in VSD

A

for small defect, normal

for large defect,
Right axis deviation
LVH
RVH

49
Q

CXR with large VSD

A

1) incr lung vascularity
2) large right pulm artery
3) large main pulm artery
4) cardiomegaly

50
Q

symptoms with VSD

A

Heart failure
1) tachypnea

2) diaphoresis
3) pulm edema due to XS pulm blood flow

51
Q

how to treat large VSD

A

1) diuretics

52
Q

indications for surgical closure of VSD

A

1) develop pulm vascular changes
2) persistent symptoms or poor growth
3) secondary effects (aortic insuff, double chambered RV)

53
Q

___ closure for some muscular VSD

A

device closure

54
Q

progression of VSD

A

small defect –> resolve spotnaeously

2) large defects decrease in size but must treat

55
Q

describe eisenmenger’s syndrome

A

1) large left to right shunt
2) incr pulm blood flow
3) muscularization of pulm arterioles
4) pulm HTN
5) incr RV pressure
6) shunt reversal R–> L
7) cyanosis and clubbing

56
Q

what is murmur in VSD caused by

A

flow across defect-pressure differential btwn LV and RV

57
Q

VSD physical exam

small VSD murmur

A

1) normal S2
2) 2-4/6 early systolic murmur
3) no diastolic murmur

58
Q

what would it mean if

murmur gets larger`

A

1) closing/restrictive VSD

2) low PVR

59
Q

what would it mean if

murmur goes away

A

1) large VSD with equalization of RV and LV pressure

2) incr PVR

60
Q

how to diagnose VSD

A

1) echo - location, magnitude of shunt
can see assoc aortic insufficiency

2) ECG normal in small
R axis deviation and LVH and RVH

61
Q

ECG in VSD

A

for small defect, normal

for large defect,
Right axis deviation
LVH
RVH

62
Q

small murmurs can have ____ murmurs than large defcts

A

louder