M1: Basic Embryology and Septal Defects Flashcards

1
Q

when in wks in the heart formed

A

3-7 wks gestation, first organ to develop

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2
Q

in what wk does the echogenic area of the heart appear and what is it called

A

wk 3

cardiogenic area

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3
Q

when the heart is just an endocardial tube, what direction does blood flow

A

unidirectional

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4
Q

how do the two heart tubes form the heart

A

they fuse into one tube and then twist/loop

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5
Q

what does looping of the heart accomplish

A

transforms the single heart tube into a more complex structure with 2 atria and 2 vent

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6
Q

how does the heart normally loop (which direction)

what direction is abnormal heart looping and what does it lead to

A

normal looping is rightwards… heart tube folds to the right

left - left transposition of the great vessels

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7
Q

what is the trunks arteriosus and what does it form

A

structure near the superior aspect of the heart

forms the semilunar valves, the AO root and PA form as the trunk separates

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8
Q

order of formation of the atrial septum and structures

A

septum primum
foramen secundum
septum secundum
foramen ovale valve

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9
Q

describe the septum primum and how it forms

A

divides the atria into L and R

extends down from the roof of the common atria towards the endocardial cushions… creates the foramen primum

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10
Q

what is the the foramen primum

A

opening b/w atria formed by the septum primum and located at its lower margin

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11
Q

describe the foramen secundum and how it forms

A

tissue that grows inferiorly towards the endocardial cushions and closes the foramen primum

perforations appear in the upper portion of the septum primum which form the foramen secundum

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12
Q

describe the septum secundum and how it forms

A

tissue that grows inferiorly to the right of the septum primum and partially overlaps the foramen secundum which forms the foramen ovale

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13
Q

after the foramen ovale is initially formed, what keep the flap patent

A

increased RAP

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14
Q

describe the foramen ovale valve and how it forms

A

the upper septum primum disappears and the lower part of the septum primum becomes the valve of foramen ovale and the septum secundum starts growing superiorly for the AV cushion

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15
Q

what happens to the foramen ovale at birth

A

increased systemic and vascular resistance paired with decreased RAP causes LAP to rise over the RAP

this pushes the valves of the foramen ovale against the septum secundum and closes the hole (they should eventually fuse after a few weeks)

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16
Q

5 types of ASDs

A
ostium primum
ostium secundum
sinus venosus
coronary sinus
PFO
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17
Q

describe an ostium primum ASD

what is it associated with

A

ASD located in lower atrial septum, 15 % of cases

cleft anterior MVL
atrioventricular canal defect
T21

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18
Q

describe an ostium secundum ASD

what is it associated with

A

ASD located mid atrial septum, 80% of cases

usually isolated
MVP
pulmonary stenosis

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19
Q

describe a sinus venosus ASD

what is it associated with

A

can be superior or inferior, where the IVC and SVC enter the RA, 6% of cases

anomalous pulmonary venous drainage (into RA instead of LA)

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20
Q

describe an coronary sinus ASD

what is it associated with

A

when the root of a CS is missing in both the LA and RA, creating a hole in the IAS, <1 %

persistent left SVC
total anomalous pulmonary venous return

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21
Q

3 steps in ventricular septal formation

A

1 trabecular IVS formation
2 TV orifice and MV orifice formation
3 truncus formation
4 membranous IVS

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22
Q

describe trabecular IVS formation

how does it grow

A

the bulbs cords becomes the RV and the primitive ventricle becomes the LV…

grows from apex to base but stops part way

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23
Q

what does the trabecular IVS stop growing part way

A

to allow blood from both ventricles to exit the truncus arteriosus through the inter ventricular foramen (IVF

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24
Q

is the trabecular IVS the membranous or muscular portion

A

muscular

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25
Q

describe TV orifice and MV orifice formation formation

how does it grow

A

the truncus arteriosus and AV canal move more centrally and the orifices are formed through the growth of the inlet portion of the IVS

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26
Q

the fusion of which two structures forms most of the IVS

A

the inlet and trabecular IVS

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27
Q

describe the truncus formation

A

this structure divides with the formation of the conotruncal septum to form the prox AO and prox pulmonary A…. forms in a spiral fashion

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28
Q

more specifically, how do the AO and PA roots form

A

the conus cordis divides the outflow tract into R and L

two swellings in the truncus arteriosus begin to grow which form a septum and divides the truncus into AO and PA

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29
Q

what is the conus cordis

A

superior part of the RV

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30
Q

describe the membranous IVS formation

A

after the TA is divided, the membranous septum forms to complete the R and L heart septation sequence

31
Q

4 locations for VSD and their prevalence

A

muscular 10-20 %
membranous/paramembranous 70-80%
inlet septum 5%
outlet septum 5%

32
Q

another name for outlet VSD

another name for inlet VSD

A

-infundibular VSD
….infundibulum is the ridge of muscle b/w the LVOT and RVOT

-atrioventricular canal

33
Q

2 types of outlet VSDs

explain their location

A

supracristal and infracristal

there’s a ridge of smooth muscle in the RVOt called the crista supraventricularis

  • supracristal VSDs are above the ridge
  • infracristal VSDs are below the ridge
34
Q

describe where you would see supracristal, infracristal and paramembranous VSDs in PSAX

A

AO zoom,
supra- coming straight up
infra - going to the right of the image toward the PA
paramembranous - going to the left of the image towards the TV

35
Q

what type of congenital heart defect is a VSD

what does this mean

A

normally it is an acyanotic congenital defect

means it not bad enough to cause serious hypoxia

36
Q

in which direction does blood travel with a VSD and ASD

A

from left to right due to high left heart pressure… in fetus is goes from right to left.

37
Q

what tool is used to assess a shunt of any kind (PDA, ASD, VSD)

A

Qp/Qs

38
Q

study photos in notes for locations of ASDs/VSDs in various echo views

A

/

39
Q

what does Qp/Qs represent

A

Q= volume of flow…

volume of flow in the pulmonary circulation / volume of flow in the systemic circulation

40
Q

how is Q/the volume of flow determined

A

by measuring the stroke volume at two intracardiac sites…

41
Q

how to do Qp/Qs for a ASD and VSD

A

find the stroke volume of the pulmonary artery using RVOT diameter and VTI of the PW of the RVOT ( pie(r)^2 x VTI )

then do the same with the LVOT and divide these values

you can technically do this with any valve in the heart so could use the TV for Qp and MV for Qs

42
Q

what does Qp/Qs equal in a normal heart

A

1:1 because flow through the R and L sides are equal

43
Q

what Qp/Qs values indicates a hemodynamically significant shunt

A

Qp/Qs of > 1.5:1

44
Q

Qp/Qs ratio for small and large shunts

A

small: 1 or 1.5:1

large > 2:1

45
Q

treatment for small, med and lrg shunts

A

sm: none or septal occlude device
md: septal occluder device or septal patch
lrg: usually septal patch

46
Q

how are septal occlude devices deployed

A

intravenously

47
Q

how are septal patchs deployed

A

open heart surgery

48
Q

with a VSD, how can the increased flow effect the RV and LA

A

they may dilate

49
Q

how is doing Qp/Qs different for a PDA

why

A

for a PDA, the Qp is derived from the LVOT and the Qs is derived from the RVOT…. we want to measure flows after they have travelled through the pulmonary and systemic circulation.

after the blood exits the LVOT, it travels to the MPA through the PDA then to the lungs…. we switch the values because the flow in greater in the L heart so Qs will be larger than Qp in this case

50
Q

which side of the heart does a PDA cause increased volume

A

left heart ONLY (VSD and ASD are both the R and L)

51
Q

explain the flow of blood with a PDA

A

blood in the ascending Ao is shunted to the MPA through the PDA, then the extra volume goes through the lungs and the PVs to empty into the LA, LV and AO again.

then the cycle continues

52
Q

explain the flow of blood with a ASD

A

blood is shunted from the LA to the RS, into the RV, P valve, lungs, PVs and into the LA, then back to the R heart

53
Q

explain the flow of blood with a VSD

A

blood is shunted from the LV to the RV, then the P valve, lungs, PVs and LA, LV again and back to the R heart.

54
Q

how do you calculate the RVSP with a VSD

A

RVSP = Systolic BP of the arm - ( 4 x (max velocity of the VSD)^2) )

second half of the equation will give you the change in pressure b/w the ventricles

55
Q

are VSDs in adult usually small

avg velocity

A

yes

5-6 m/sec

56
Q

what happens to the heart if the VSD is large

A

Eisenmengers will occur

57
Q

what is Eisenmengers syndrome

why does it happen

A

when the direction of a shunt is switched to go right to left

due to a large shunt in the heart that has high volume passing through it going to the lungs, causing lung damage and scarring over time…
… which causes irreversibly high pulmonary vascular resistance and severe pulmonary hypertension.

58
Q

6 complications of Eisenmengers

A
CHF
early death
Brain abscess
SBE - bacterial endocarditis
pulmonary infarction
pregnancy is contraindicated w/ Eisenmengers, extra volume would be too much for the heart, mom would likely die
59
Q

how should you describe intracardiac shunts when reporting

A

segmental approach, then describe actual name of the defect

60
Q

do you have to interrogate every heart for a shunt

A

yes

61
Q

2 functions of the endocardial cushion

A

1 divides the AV canal into 2 atrioventricular orifices

2 assist in closure of ostium primum portion of IAS and membranous portion of IVS

62
Q

what are the 4 endocardial cushions

A

2 medial cushions

2 lateral cushions

63
Q

what do the 2 medial endocardial cushions form

how does they grow

A

form the: septal leaflet of the TV, anterior leaflet of the MV and part of the inflow perimembranous inter ventricular septum

grow from laterally from mid-line

64
Q

what do the 2 lateral endocardial cushions form

how does they grow

A

form the: anterior and posterior TV leaflets and the posterior MV leaflet

grow from medially from the sides

65
Q

how many sets of Ao arches originally for in the fetus

A

6 sets, most are absorbed into the body

66
Q

which AO arch in the fetus forms the adult AO

what do the others form into

A

4th Ao arch

carotid and ductus arteriosus

67
Q

what forms the AV valves

A

formed from the partition of the AV canal and the lateral endocardial cushions… AV valves made of connective tissue

68
Q

what forms the SL valves

A

the truncus arteriosus, swelling of tissue forms the valves

69
Q

normal route of fetal circulation stating at umbilical vein

A

umbilical vein > ductus venosus > IVC > RA, then the eustachian valve directs more of the blood through the foramen ovale but there are two other paths:

1) RV>PA>…LA>LV>AO
2) RV>PA>…DA>A

70
Q

function of the eustachian valve

A

directs fetal blood through the foramen ovale

71
Q

why does very little blood go through the pulmonary circulation in the fetus

A

fetal lungs have very high resistance vascular beds

72
Q

how does the fetal circulation pathway change at birth

A
  • umbilical vein closes
  • ductus arteriosus closes and become the ligamentum arteriosum
  • ductus venosus closes and becomes the ligamentum venosum
  • foramen ovale closes and becomes the fossa ovalis (indent where the hole was located)
  • RV and PA walls decrease in thickness and LV wall increase in thickness. because pressure in R heart goes down and L heart goes up
73
Q

2 most common locations for congenital heart defects

A

ductus arteriosus and foramen ovale