Devo + Clinical Flashcards

(78 cards)

1
Q

what is associated with ectopia cordis? and what is?

A

anterior thoracic wall fails to close properly. assoc w pentalogy of cantrell: EC, VSD, sternal cleft, diaphragmatic hernia, omphalocele

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2
Q

secondary heart field allows formation of?

A

primitive RV, outflow region, primordial atrium, AV canal, sinus venosus

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3
Q

what is the precursor tissue of the heart?

A

splanchinic mesoderm

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4
Q

what is precursor additions is looping driven by?

A

secondary heart field

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5
Q

what are the tissues used for heart partitioning?

A

cardiac muscle, cardiac mesenchyme, extracardiac mesenchyme, neural crest mesenchyme

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6
Q

structure: coronary sinus

derived from?

A

left horn of sinus venosus

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7
Q

structure: crista terminalis

derived from?

A

right valve of sinus venosus

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8
Q

structure: valve of IVC

derived from?

A

right valve of sinus venosus

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9
Q

structure: valve of CS

derived from?

A

right valve of sinus venosus

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10
Q

structure: smooth area of RA

derived from?

A

absorption of sinus venosus, primarily the right horn

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11
Q

structure: IVC (terminal portion)

derived from?

A

right vitelline vein

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12
Q

structure: valve of SVC

derived from?

A

right common cardinal & proximal anterior cardinal vein

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13
Q

structure: valve of appendage (auricle) (L and R)

derived from?

A

primordial atrium

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14
Q

structure: definitive LA

derived from?

A

absorption of pulmonary veins and primitive left atrium

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15
Q

structure: smooth part of LA

derived from?

A

absorption of the pulmonary veins

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16
Q

how is common AV canal expanded?

A

myocardialization: cushion tissue into cardiac muscle

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17
Q

what does division of common AV canal depend on?

A

formation of cardiac mesenchyme (cushion tissue). epithelium to mesenchyme transformation (endocardial cells transform into cardiac mesenchyme cells which move into the cardiac jelly).

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18
Q

what structures are formed from cardiac mesenchyme?

A

valve leaflets, chordae tendinae and fibrous skeleton of the heart

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19
Q

embryonic primordia: proximal outflow region

adult derivative: ?

A

ventricular outlets (R & L)

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20
Q

embryonic primordia: distal outflow region

adult derivative: ?

A

pulmonary and Ao valves, and pulmonary and Ao root

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21
Q

embryonic primordia: aortic sac

adult derivative: ?

A

pulmonary and aortic root

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22
Q

aortic sac partitioning occurs due to what tissue type?

A

neural crest-derived mesenchyme that forms the aorticopulmonary septum

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23
Q

what components form the membranous IVS?

A

conotruncal septum + endocardial cushion, fuse with muscular IVS

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24
Q

structure: inlet of RV

derived from?

A

AV canal

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25
structure: apical trabeculated component of RV | derived from?
primitive RV
26
structure: outlet of RV | derived from?
proximal outflow region
27
structure: inlet of LV | derived from?
AV canal
28
structure: apical trabeculated component of LV | derived from?
primitive LV
29
structure: outlet of LV | derived from?
proximal outflow region
30
what cell types come from cardiogenic mesoderm (3)
endocardial endothelial cell, atrial myocyte, ventricular myocyte
31
what other cell type is derived from endocardial endothelial cells?
cushion cell
32
what other cell type is derived from ventricular myocytes?
purkinje fibers
33
what cell types are derived from cardiac neural crest cells?
aortic smooth muscle (and poss pulmonary vessels), and neurons
34
what cell types are derived from proepicardium?
coronary smooth muscle, endothelial cells, fibroblasts
35
pharyngeal arch artery 3 developmental fate?
proximal: common carotid arteries distal: internal carotid arteries
36
pharyngeal arch artery 4 developmental fate?
left: part of aortic arch right: proximal right subclavian artery
37
pharyngeal arch artery 6 developmental fate?
left, proximal: proximal left pulmonary artery left, distal: ductus arteriosus right, proximal: proximal right pulmonary artery right, distal: degenerates
38
what are the 3 fetal shunts? where to where?
ductus venosus: O2 rich blood from placenta around liver foramen ovale: blood in IVC from R atrium to L atrium ductus arteriosus: blood from RV into aorta (bypassing left heart)
39
what types of gene products are affected with CHD?
transcription factors, signaling proteins & receptors, helicase-binding protein
40
what are the L to R shunts? are they cyanotic?
ASD, VSD, AVSD, PDA. late cyanotic
41
what are the obstructions? are they cyanotic?
pulmonary stenosis, aortic stenosis, coarctation. acyanotic.
42
what are the R to L shunts? are they cyanotic?
Tetralogy of Fallot, Transposition of the Great Arteries, Truncus arteriosus, TV atresia, TAPVR
43
what is the valvular regurgitation associated with CHD?
ebstein
44
clinical consequence of ASD?
potential pulmonary hypertension and right-sided failure
45
clinical consequence of PFO?
paradoxical emboli, decompression sickness, migraines possibly
46
long term effects of ASD?
RVH and dilation, RA and LA dilation
47
murmor with PDA?
harsh, continuous, machinery like murmur
48
what other anomalies are AVSDs associated with?
mitral and tricuspid valve anomalies
49
what is a partial AVSD?
primum ASD and cleft MV with MR
50
what is a complete AVSD?
AVSD and common AV valve
51
what genetic syndrome is associated with complete AVS?
down syndrome (40%)
52
clinical symptoms of R to L shunts
early cyanosis. paradoxical emboli and decompression sickness (clots or gas bubbles not filtered by lungs). digital clubbing. polycythemia.
53
findings of tetralogy?
VSD, subpulmonary stenosis, overriding aorta, RVH
54
CXR of tetralogy?
boot shaped heart d/t RVH
55
gross findings of tetralogy?
small PA
56
definition of transposition of the great arteries?
aorta arises from RV. pulmonary artery arises from LV.
57
sequelae of TGA?
aorta anterior and to R of PA. separate pulmonary and systemic circulations. RVH. pulmonary hypertension develops unless pulmonary stenosis is present.
58
2 types of TGA?
stable: with VSD unstable: with intact VS (need surgery)
59
truncus arteriosus definition?
origin of aorta and pulmonary artery from truncal artery. + most have large VSD
60
etiology of truncus?
developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery
61
sequelae of truncus?
mixing of blood, increased pulmonary blood flow, pulmonary HTN, cyanosis
62
what other syndrome is truncus associated with?
DiGeorge
63
what does tricuspid atresia result from?
unequal division of the AV canal (MV enlarged)
64
what defects must be present also?
ASD/PFO and VSD
65
what does tricuspid atresia cause?
RVH
66
what is TAPVR (total anomalous pulmonary venous return). why does it occur?
pulmonary veins do not directly drain into LA; left atrial hypoplasia. d/t common pulmonary vein failure of development
67
how does ASD/PFO help?
allows oxygenated blood to enter systemic circulation
68
what are 2 types of aortic coarctation?
preductal/infantile (tubular hypoplasia with PDA); postductal/adult (ridgelike infolding at ligament without PDA)
69
what is the definition of pulmonary stenosis?
PV obstruction d/t hypoplasia, dysplasia, or abnormal number of cusps
70
results of isolated PV stenosis?
RV dilation and hypertrophy. post-stenotic injury to PA.
71
what occurs with PV atresia with intact VS?
hypoplastic RV and TV. PDA needed to get blood to lungs.
72
long term changes d/t PS?
pulm artery dilation, RVH & RA dilation
73
isolated aortic valve stenosis results in?
LVH and LA dilation
74
AS murmur type?
systolic murmur
75
what is hypoplastic left heart syndrome due to?
aortic valve atresia with intact VS. need PDA for survival.
76
what is an ebstein anomaly?
inferiorly displaced and adherent septal and posterior leaflets of tricuspid valve. redundant anterior leaflet. dilated annulus with tricuspid regurgitation.
77
secondary effects of ebstein anomaly?
RV and RA dilation
78
other features of ebstein anomaly?
arrhythmias (including WPW). may be asymptomatic until adulthood.