Cardiac embryology and congenital heart disease II Flashcards

1
Q

which gender more likely to have severe congenital cardiovascular malformation

A

boys

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2
Q

risk factor for congenital cardiovascular malformation

A

maternal diabetes

fhx of cardiac defect in parent/sibling

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3
Q

if maternal diabetes how do you screen for congenital CV malformation

A

fetal echo

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4
Q

Types of ASD

A

1) secundum ASD

2) sinus venosus ASD

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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

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6
Q

what is PAPVR

A

sinus venosus ASD with partial anomalous pulm veins

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7
Q

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

A

1) size of defect

2) inflow resistance to RV vs LV

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8
Q

what type of shunt across an ASD

A

left to right

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9
Q

why left to right shunt across ASD

A

LA pressure higher than RA pressure

LA and RA pressure equalize if ASD large

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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

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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

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12
Q

physical exam of ASD

small defect with no/minimal shunt OR NEONATE

A

1) depends on degree of shunting

normal exam

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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

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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

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15
Q

murmur ____ related to blood flowing across defect

A

NOT b/c pressure differential too small

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16
Q

why do you have systolic murmur with ASD

A

XS blood flow across pulm valve

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17
Q

why do you have diastolic rumble with ASD

A

XS blood flow in diastole across tricuspid

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18
Q

what causes physiologic S2 split

A

inspiration

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

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19
Q

what causes S2 split in ASD

A

RV volume overload –> delayed RV empty

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20
Q

ECG of ASD

A

Right axis deviation

RVH (rsR’ or qR in V1/V2)

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21
Q

if you see

RAD
RVH

what abnormality

A

ASD

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22
Q

how to diagnose ASD

A

1) CXR
large main pulm artery
prominent pulm vascular markings

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

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23
Q

progression of ASD

A

undetected in childhood

risk of hemodynamically significant ASD

  • pulm vasc disease
  • atrial arrhythmia
  • cardiac failure
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24
Q

describe pulm vascular disease (sx of ASD)

A

high pulm blood flow

more common and earlier age at higher altitudes

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25
where is pulm vascular diseases most common
higher altitudes
26
describe atrial arrhythmias (sx of ASD)
older patients due to atrial enlargement
27
describe cardiac failure (sx of ASD)
> 20 y/o with large shunts or with pulm vascular disease RIGHT HEART FAILURE - HEPATOMEGALY - VENOUS CONGESTION
28
medical therapy for ASD
1) diuresis for dyspnea | 2) CLOSE THE HOLE FOR OLDER CHILDREN W/ ASD or symptomatic infants
29
how to close hole in ASD
surgery percutaneous device closure
30
normal ventricular septation
post-loop day 28-42 intraventricular septum grows toward base of heart as ventricular outpouch develop --> 4 endocardial cushions
31
what is anatomic correlate of superior endocardial cushion
1) left surface of outlet of Interventricular septum | 2) mitral valve
32
what is anatomic correlate of inferior endocardial cushion
1) inlet of interventricular septum 2) membranous portion of interventricular septum 3) tricuspid and mitral
33
what is anatomic correlate of right endocardial cushion
tricuspid valve
34
what is anatomic correlate of left endocardial cushion
posterior leaflet of mitral valve
35
what is considered a large VSD?
same diameter as aortic orifice
36
large VSD are often ____
unrestrictive equalization of LV and RV pressure
37
magnitude of VSD depends on (3)
1) size of defect 2) systemic and pulm vascular resistance 3) right or left heart obstructive lesions (pulm or aortic stenosis)
38
physiologic consequences of PVR < SVR
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
VSD clinical presentation
asymptomatic until PVR falls after birth | delayed at elev altitudes
40
VSD clinical presentation large VSD
1) respiratory distress | 2) diaphoresis with feeds
41
VSD clinical presentation small VSD
1) tachypnea | 2) mild or no diaphoresis
42
VSD physical exam large VSD murmur
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
where do you hear large VSD murmur
left lower sternal border
44
VSD physical exam small VSD murmur
1) normal S2 2) 2-4/6 early systolic murmur 3) no diastolic murmur
45
what would it mean if murmur gets larger`
1) closing/restrictive VSD | 2) low PVR
46
what would it mean if murmur goes away
1) large VSD with equalization of RV and LV pressure | 2) incr PVR
47
how to diagnose VSD
1) echo - location, magnitude of shunt can see assoc aortic insufficiency 2) ECG normal in small R axis deviation and LVH and RVH
48
ECG in VSD
for small defect, normal for large defect, Right axis deviation LVH RVH
49
CXR with large VSD
1) incr lung vascularity 2) large right pulm artery 3) large main pulm artery 4) cardiomegaly
50
symptoms with VSD
Heart failure 1) tachypnea 2) diaphoresis 3) pulm edema due to XS pulm blood flow
51
how to treat large VSD
1) diuretics
52
indications for surgical closure of VSD
1) develop pulm vascular changes 2) persistent symptoms or poor growth 3) secondary effects (aortic insuff, double chambered RV)
53
___ closure for some muscular VSD
device closure
54
progression of VSD
small defect --> resolve spotnaeously 2) large defects decrease in size but must treat
55
describe eisenmenger's syndrome
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
what is murmur in VSD caused by
flow across defect-pressure differential btwn LV and RV
57
VSD physical exam small VSD murmur
1) normal S2 2) 2-4/6 early systolic murmur 3) no diastolic murmur
58
what would it mean if murmur gets larger`
1) closing/restrictive VSD | 2) low PVR
59
what would it mean if murmur goes away
1) large VSD with equalization of RV and LV pressure | 2) incr PVR
60
how to diagnose VSD
1) echo - location, magnitude of shunt can see assoc aortic insufficiency 2) ECG normal in small R axis deviation and LVH and RVH
61
ECG in VSD
for small defect, normal for large defect, Right axis deviation LVH RVH
62
small murmurs can have ____ murmurs than large defcts
louder