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Cardiovascular Unit > Devo + Clinical > Flashcards

Devo + Clinical Flashcards

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
Q

structure: apical trabeculated component of RV

derived from?

A

primitive RV

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26
Q

structure: outlet of RV

derived from?

A

proximal outflow region

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27
Q

structure: inlet of LV

derived from?

A

AV canal

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28
Q

structure: apical trabeculated component of LV

derived from?

A

primitive LV

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29
Q

structure: outlet of LV

derived from?

A

proximal outflow region

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30
Q

what cell types come from cardiogenic mesoderm (3)

A

endocardial endothelial cell, atrial myocyte, ventricular myocyte

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31
Q

what other cell type is derived from endocardial endothelial cells?

A

cushion cell

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32
Q

what other cell type is derived from ventricular myocytes?

A

purkinje fibers

33
Q

what cell types are derived from cardiac neural crest cells?

A

aortic smooth muscle (and poss pulmonary vessels), and neurons

34
Q

what cell types are derived from proepicardium?

A

coronary smooth muscle, endothelial cells, fibroblasts

35
Q

pharyngeal arch artery 3 developmental fate?

A

proximal: common carotid arteries
distal: internal carotid arteries

36
Q

pharyngeal arch artery 4 developmental fate?

A

left: part of aortic arch
right: proximal right subclavian artery

37
Q

pharyngeal arch artery 6 developmental fate?

A

left, proximal: proximal left pulmonary artery
left, distal: ductus arteriosus
right, proximal: proximal right pulmonary artery
right, distal: degenerates

38
Q

what are the 3 fetal shunts? where to where?

A

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
Q

what types of gene products are affected with CHD?

A

transcription factors, signaling proteins & receptors, helicase-binding protein

40
Q

what are the L to R shunts? are they cyanotic?

A

ASD, VSD, AVSD, PDA. late cyanotic

41
Q

what are the obstructions? are they cyanotic?

A

pulmonary stenosis, aortic stenosis, coarctation. acyanotic.

42
Q

what are the R to L shunts? are they cyanotic?

A

Tetralogy of Fallot, Transposition of the Great Arteries, Truncus arteriosus, TV atresia, TAPVR

43
Q

what is the valvular regurgitation associated with CHD?

A

ebstein

44
Q

clinical consequence of ASD?

A

potential pulmonary hypertension and right-sided failure

45
Q

clinical consequence of PFO?

A

paradoxical emboli, decompression sickness, migraines possibly

46
Q

long term effects of ASD?

A

RVH and dilation, RA and LA dilation

47
Q

murmor with PDA?

A

harsh, continuous, machinery like murmur

48
Q

what other anomalies are AVSDs associated with?

A

mitral and tricuspid valve anomalies

49
Q

what is a partial AVSD?

A

primum ASD and cleft MV with MR

50
Q

what is a complete AVSD?

A

AVSD and common AV valve

51
Q

what genetic syndrome is associated with complete AVS?

A

down syndrome (40%)

52
Q

clinical symptoms of R to L shunts

A

early cyanosis. paradoxical emboli and decompression sickness (clots or gas bubbles not filtered by lungs). digital clubbing. polycythemia.

53
Q

findings of tetralogy?

A

VSD, subpulmonary stenosis, overriding aorta, RVH

54
Q

CXR of tetralogy?

A

boot shaped heart d/t RVH

55
Q

gross findings of tetralogy?

A

small PA

56
Q

definition of transposition of the great arteries?

A

aorta arises from RV. pulmonary artery arises from LV.

57
Q

sequelae of TGA?

A

aorta anterior and to R of PA. separate pulmonary and systemic circulations. RVH. pulmonary hypertension develops unless pulmonary stenosis is present.

58
Q

2 types of TGA?

A

stable: with VSD
unstable: with intact VS (need surgery)

59
Q

truncus arteriosus definition?

A

origin of aorta and pulmonary artery from truncal artery. + most have large VSD

60
Q

etiology of truncus?

A

developmental failure of separation of the embryologic truncus into the aorta and pulmonary artery

61
Q

sequelae of truncus?

A

mixing of blood, increased pulmonary blood flow, pulmonary HTN, cyanosis

62
Q

what other syndrome is truncus associated with?

A

DiGeorge

63
Q

what does tricuspid atresia result from?

A

unequal division of the AV canal (MV enlarged)

64
Q

what defects must be present also?

A

ASD/PFO and VSD

65
Q

what does tricuspid atresia cause?

A

RVH

66
Q

what is TAPVR (total anomalous pulmonary venous return). why does it occur?

A

pulmonary veins do not directly drain into LA; left atrial hypoplasia. d/t common pulmonary vein failure of development

67
Q

how does ASD/PFO help?

A

allows oxygenated blood to enter systemic circulation

68
Q

what are 2 types of aortic coarctation?

A

preductal/infantile (tubular hypoplasia with PDA); postductal/adult (ridgelike infolding at ligament without PDA)

69
Q

what is the definition of pulmonary stenosis?

A

PV obstruction d/t hypoplasia, dysplasia, or abnormal number of cusps

70
Q

results of isolated PV stenosis?

A

RV dilation and hypertrophy. post-stenotic injury to PA.

71
Q

what occurs with PV atresia with intact VS?

A

hypoplastic RV and TV. PDA needed to get blood to lungs.

72
Q

long term changes d/t PS?

A

pulm artery dilation, RVH & RA dilation

73
Q

isolated aortic valve stenosis results in?

A

LVH and LA dilation

74
Q

AS murmur type?

A

systolic murmur

75
Q

what is hypoplastic left heart syndrome due to?

A

aortic valve atresia with intact VS. need PDA for survival.

76
Q

what is an ebstein anomaly?

A

inferiorly displaced and adherent septal and posterior leaflets of tricuspid valve. redundant anterior leaflet. dilated annulus with tricuspid regurgitation.

77
Q

secondary effects of ebstein anomaly?

A

RV and RA dilation

78
Q

other features of ebstein anomaly?

A

arrhythmias (including WPW). may be asymptomatic until adulthood.

Cardiovascular Unit (16 decks)

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