Heart Development Flashcards

1
Q

About how often do congenital cardiac anomalies occur?

How many are severe?

A

5:1000 to 8:1000 live births

1/3 are severe

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2
Q

What is the order of foramen in atrium septation?

A

foramen primum at 33 days –> foramen secudum 6th week –> foramen ovale 7th week

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3
Q

What happens during lateral folding?

A

endocardial heart tubes approach each other and fuse

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4
Q

What are the left and right portions of the incorporated bulbus cordis?

A
right = conus arteriosus (infundibulum
left = aortic vestibule
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5
Q

What occurs in primitive heart folding?

A

bulbus cordis and ventricles grow quickly –> go anterior and inferior –> atrium and sinus venosus fold up and come to lie dorsally

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6
Q

How do you correct transposition of the great vessels?

A

put on prostaglandins to keep septal defect open until surgery

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7
Q

What is a transitional AV septal defect?

A

large primum defect
cleft mitral valve
VSD

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8
Q

When does the fetal heart first beat?

A

4th week (22 days)

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9
Q

What are the 4 main primitive divisions of the heart?

A

truncus arteriosus = outflow tube
bulbus cordis
primitive ventricle
primitive atrium

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10
Q

What is the transverse pericardial sinus?

A

in embryo - where dorsal mesocardium once was between the heart and the posterior body wall
In an adult - btw the aorta and pulmonary artery; put fingers through to clamp aorta

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11
Q

Where are right and left bulbar ridges?

A

more inferior –> R and L bulbar ridgesend up fusing w/ endocardial cusion to form the interventricular septum and aorticopulmonary septum

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12
Q

What do you normally see before and after indomethacin therapy for PDA?

A

enlarged heart and opacificed lungs –> treatment –> clear lungs and heart smaller

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13
Q

What are the endocardial/ AV cushions?

When do they form?

A

derived from mesoderm from dorsal and ventral walls –> grow together –> right and left AV canals and separates atria from ventricle
mesenchyme invades in 5th week

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14
Q

Where is the truncus arteriosus?

A

ridges are more superior = outflow tract

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15
Q

In general, are atrial septal defects cyanotic or acyanotic?

A

acyanotic

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16
Q

What are defects in the interventricular septum due to?

A

membranous part of the septum

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17
Q

What is dextrocardia?

A

heart folds the exact opposite –> mirror image forms

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18
Q

What is patent ductus arteriosus?

A

ductus arteriosus is still open = L to R shunt pushing blood from aorta into pulmonary A = acyanotic
can cause pulmonary HTN if untreated

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19
Q

What foramen arise due to the septum primum?

A

foramen primum

foramen secundum

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20
Q

What are bulbus cordis and truncus arteriosus derived from?

A

neural crest cells

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21
Q

What type of heart defects are cyanotic?

A

right to left shunt

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22
Q

What is truncus arteriosus communis?

A

one great vessel = cyanotic defect

NC migration screwed up –> truncal ridges don’t form correctly

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23
Q

What genes/molecules regulate neural crest migration to the heart?

A

retinoic acid (vit A)
hox genes
Nf-1
pax 3

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24
Q

What is the aorticopulmonary septum?

A

divides bulbus cordis and truncus arteriosus into ascending aorta and pulmonary trunk –> fuses w/ endocardial cushion

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25
Q

What type of heart defects are acyanotic?

A

no shunt or L to R shunt

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26
Q

What does the smooth muscle of the atria arise from?

A

the sinus venosus

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27
Q

What is transposition of the great vessels?

A

R ventricle now goes to aorta and L ventricle now goes to pulmonary trunk; also have septal defect
acyanotic
caused by NC cells that don’t migrate properly –> bulbar and truncal folds dont’ twist right

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28
Q

What are the heart fields developed from?

A

splanchnic mesoderm

established in cranial end of embryo

29
Q

What happens in primum ASD?

A

septum primum doesn’t fuse w/ endocardial cushions

associated with mitral valve cleft

30
Q

How does the bulbar and truncal ridge twisting orient the pulmonary trunk and aorta?

A

aorta starts anterior –> twist –> posterior at the end

31
Q

What are angioblastic cords?

A

masses of splanchnic mesoderm that are the early heart tubes

32
Q

When does the foramen primum disappear?

A

when it fuses with endocardial cushions in 6th week

33
Q

What are the signs of critical aortic stenosis?

Is it cyanotic?

A

tachypnea, poor feeding and perfusion, may lead to hypoplastic L heart syndrome
yes - is cyanotic bc decreased systemic blood flow

34
Q

Why can’t pregnant mothers not take accutane?

A

it is a Vit A derivative –> stops neural crest cells from migrating to the heart –> fetal heart defects

35
Q

What is hypoplastic left heart syndrome?

A
mitral valve stenosis or atresia
L ventricle hypoplasia
aortic valve stenosis or aortic arch hypoplasia
cyanotic!
50% die
36
Q

When does circulation begin?

A

4th week

37
Q

What type of pediatric patients are likely to have AV septal defects?

A

Down’s syndrome = 20% have it

38
Q

What signal is required for remodeling of AV cushions?

A

retinoic acid

39
Q

What is a partial AV septal defect?

A

primum ASD
single AV valve annulus w/ 2 separate valve orifices
(anterior leaflet of mitral valve is typically cleft)

40
Q

What type of heart defects cause no shunt?

A

anomalies of aortic arches

coarctation of aorta

41
Q

when do the semilunar valves start to develop?

A

once partition of truncus arteriosus is nearly complete –> valves swell btw bulbus cordis and truncus arteriosus –> blood pressure erodes tissue to cusps

42
Q

What do the endocardium, myocardium, and epicardium all arise from?

A

splanchnic mesoderm

43
Q

What is the clinical presentation of patent ductus arteriosus?

A

continuous murmor
large defect = poor eating, sweating w/ crying or eating, rapid heart rate, tachypnea
persistent: high pressure blood from aorta can destroy capillary beds in lungs

44
Q

What are AV septal defects caused by?

are they cyanotic or acyanotic?

A

failure of endocardial cushions to fuse

acyanotic

45
Q

What is a mesentery?

A

double layer of splanchnic mesoderm = route for nerves and vessels to get to organs

46
Q

Are ventricular septal defects acyanotic of cyanotic?

What is the basis for the defect?

A

acyanotic

interventricular septum never fuses w/ endocardial cushions

47
Q

Why doesn’t patent ductus arteriosus close?

How do you treat it?

A

low oxygen content
circulating prostaglandin E2 = vasodilator that keeps duct open; mediated by COX-2
treatment = COX-2 inhibitors (ibuprofen, indomethacin)

48
Q

What is the dorsal mesocardium?

A

bridge of mesoderm that connects the developing heart to the posterior wall of the embryo

49
Q

What do secondary heart fields develop into?

A

right ventricle
outflow tract
part of atria (venous pole)

50
Q

What is tetralogy of fallot?

A

common and serios defect
polmonary stenosis and interventricular septal defect –> over-riding aorta and R ventricle hypertrophy
cyanotic
caused by abnormal NC septation of outflow tract

51
Q

How do neural crest cells contribute to heart development?

A

originate from myelencephalon –> migrate through pharyngeal arches 3, 4, and 6 –> help form truncus arteriosus and aorticopulmonary septa

52
Q

Aortic or pulmonary valve defects can be due to disruption of what embryonic tissue?

A

neural crest (migration)

53
Q

How does ventricular septation occur?

A

interventricular septum grows superiorly to eventually fuse with the endocardial cushions
(still have interventricular foramen for most of development)

54
Q

What is the ductus arteriosus?

A

connection btw pulmonary artery and aorta in utero that should close when born
shunts blood from pulmonary A straight to aorta, I think

55
Q

What does the left horn of the sinus venosus give rise to?

A

orifice of the coronary sinus

56
Q

What happens in secundum ASD?

A

patent foramen ovale - most common type = incomplete adhesion of foramen ovale and septum secundum aftern birth
could also have inadequate dev of septum secundum; excessive cell death

57
Q

What does the right horn of the sinus venosus give rise to?

A

orifices of superior and inferior vena cava

sinus venarum

58
Q

What happens to the left and right sinuatrial valves?

A

cranially: both meet and fuse w/ septum spurium
right cranial –> crista terminalis
right caudal –> valve of coronary sinus and most of valve of inferior vena cava

59
Q

Which direction does the bulboventricular loop bend?

A

to the right

60
Q

What are the 2 types of atrial septal defects?

A

secundum ASD

primum ASD

61
Q

What is the heart derived from?

A

splanchnic mesoderm
mesenchyme
angioblastic tissue

62
Q

What do primary heart fields develop into?

A

left and right atria and the left ventricle

63
Q

What is critical pulmonary stenosis?

A

cusps of pulmonary valves are fused or thickened

cyanotic if called “critical”

64
Q

What is a complete AV septal defect?

A

primum ASD and VSD w/ a common AV valve

65
Q

Where are the septum primum and secundum, from R to L?

A
R = secundum
L = primum
66
Q

What is double outlet right ventricle?

A

cyanotic defect
both great vessels lead to R ventricle
ventricular septal defect usually
caused by abnormal migration of bulbar ridges

67
Q

When do the endocardial tubes begin to fuse/ when are they fully fused?

A

begin at day 21

finished at 22 days

68
Q

What does heart folding begin to occur?

A

day 23

69
Q

When is heart folding complete?

A

day 35