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MOD 1 Test > Cardiac development and pediatric heart disease > Flashcards

Cardiac development and pediatric heart disease Flashcards

1
Q

Two broad types of congenital heart disease?

A

Cyanotic lesions

Acyanotic lesions

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2
Q

Form the heart tube?

A

the paired endocardial tubes are brought into close proximity and fuse to create the heart tube, just as the visceral layer of the lateral plate mesoderm folds to form the gut tube

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3
Q

How does blood flow into the heart tube?

A

from inferior to superior

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4
Q

Form the myocardium?

A

The visceral mesoderm surrounding the endocardial tubes enlarges
actively beats after 22 days

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5
Q

Early heart regions and blood flow?

A

The blood flows inferior to superior
the inferior part becomes the atria, while the superior part becomes the ventricles, aorta and pulmonary arteries

from inferior to superior (regions)

  • sinus venosos
  • primitive ventricle
  • ventricle
  • bulbus cordis
  • aortic sac
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6
Q

Aortic sac becomes?

A

aorta, pulmonary artery, aortic arches

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7
Q

Bulbus cordis becomes? (conus cordis, truncus arteriosus)

A

Right ventricle, proximal aorta, pulmonary trunk

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8
Q

Ventricle becomes?

A

Left ventricle

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9
Q

Primitive atrium becomes?

A

Right and left auricles and atrium

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10
Q

Sinus venosus becomes?

A

right atrium, vena cava, coronary sinus

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11
Q

4 steps to the development of the 4 chambered heart?

A
  1. the primitive atrium is divided into right and left atria
  2. the primitive ventricle is separated from the bulbus cords to form the left and right ventricles respectively
  3. the primitive atrium is separated from the primitive ventricle
  4. the conus cordis and the truncus arteriosus develop internal partitions to become the proximal aorta and pulmonary trunk
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12
Q

Primitive atrium divides into left and right?

A
  1. septum primum grows, leaves ostium primum open, endocardial cushions eventually close it
  2. cell death creates the ostium secundum
  3. septum secundum forms foramen ovale
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13
Q

PFO/ patent forament ovale?

A

normal at birth, not normal to stay open
25% normal pop may have it
opens during increased right heart pressure
can allow clots to enter the arterial circulation (stroke)

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14
Q

ASD/ atrial septal defect?

A 
sporadic, more in females>men (septum secudum)
3 types/ areas:
 ostium secundum (midportion)
 ostium primun (lower portion)
 sinus venosus (junction of the right atrium and vena cava)

*prium and sinus venosus lesions more often associated with other defects

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15
Q

ASD presentation?

A

small lesions, often asymptomatic
characterisitc murmur may be heard on exam
large lesions can cause left to right shunt leading to right atrial enlargement, increased right heart pressures, and heart failure if left untreated

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16
Q

Treat ASD?

A

small lesions close spontaneously
small to med lesions cna be closed with transcatheter device
complicated leisions and/or large lesions usually close by surgical patch

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17
Q

Form the muscular portion of the interventricular septum?

A

region between the primitive ventricle and the bulbus cordis, grows upward
helps seperated the atrioventricular canal into right and left

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18
Q

Form the membranous portion of the interventricular septum?

A

the endocardial cushions

grows down to meet the muscular

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19
Q

VSD/ Ventricular septal defect?

A

most common CHD lesion
no direct genetic cause–
increased right in family members, increased incidence in chromosomal abnormalities

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20
Q

Typical classification of VSD?

A

Perimembranous (most common)
Muscular (less)
Supracristal (rare)

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21
Q

VSD presentation?

A

small lesion asymptomatic
characteristic murmur may be heard on exam
large lesions can cause left to right shunt leading to increased right pressures and heart failure if left untreated

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22
Q

VSD treatment?

A

small lesions may close spontaneously, but location matters
Spontaneous closure largely occurs before age 4 yo
in symptomatic patients, transcatheter device or surgical patch can be curative

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23
Q

Primitive atrium is separated from the primitive ventricle?

A

separated by the endocardial cushions

often seen on prenatal screening as a cross formed by the atriventricular valves, atrial septum, and ventricular septum

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24
Q

The fusing endocardial cushions leaves gaps that become what?

A

the left and right atrioventricular canals

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25
Q

What closes the atrioventricular canals?

A

closed by formation of the tricuspid valve on the right and bicuspid valve on the left

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26
Q

Atrioventricular canal defect?

A

aka endocardial cushion defect

partial or complete

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27
Q

Parial Atrioventricular canal defect?

A

essentially a severe low ASD or high VSD with the AV valves affected but present

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28
Q

Complete atrioventricular canal defect?

A

the av valves are not developed and all four chambers of the heart are contiguous

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29
Q

Form the heart valves?

A

ventricular mesenchyme below the atrioventricular canals “hollows out” leaving behind valves, chordae tendinae and papillary muscles

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30
Q

Fault AV valve formation may result in ?

A

stenosis, atresia, blood reguritation and/or murmurs

31
Q

Left ventricular outflow tract obstruction?

A

spectrum of pathology involving subvalvular, valvular, or supravalvular portion of the aortic valve complex

32
Q

Milder Left ventricular outflow tract obstruction?

A

Aortic stenosis

-murmur, increased left sided pressures can lead to heart failure if left untreated

33
Q

Severe Left ventricular outflow tract obstruction?

A

Hypoplastic left heart syndrome

-cyanotic newborn

34
Q

Right Ventricular Outflow Tract Obstruction?

A

Spectrum of pathology invlolving subvalvular, valvular or supravalvular portion of the pulmonary valve complex

35
Q

Milder Right Ventricular Outflow Tract Obstruction?

A

Pulmonary stenosis

-murmur, increased right sided pressures can lead to heart failure if left untreated

36
Q

Severe Right Ventricular Outflow Tract Obstruction?

A

Tetrology of fallot

-cyanotic newborn

37
Q

Very severe Right Ventricular Outflow Tract Obstruction?

A

hypoplastic right heart syndrome

38
Q

Tetrology of fallot?

A

pulmonary stenosis
right ventricular hypertrophy
overriding aorta
VSD

39
Q

tricuspid atresia?

A

PFO or ASD
Atresia
VSD

40
Q

Conus cordis, truncus ateriosus, and aortic sac subdivide to become?

A

the proximal aorta and pulmonary vessels

41
Q

Truncus arteriosus initially?

A

a single tube leading to the aortic sac, subdivides into two tubes by conotruncal ridges that come largely from neural crest cells

42
Q

The conotruncal ridges spiral?

A

as they pass through the conus cordis and truncus arteriosus, the spiral allows the aorta and pulmonary trunk to meet the left and right ventricles respectively

43
Q

the membranous portion of the interventricular septum meets what from below?

A

the conotruncal ridge and completely seperates the aorta from the pulmonary trunk

44
Q

Truncus ateriosus?

A

mixing lesions, cyanotic heart lesion

only one large vessel exists the heart

45
Q

Transposition of the great vessels?

A

Don’t twist right
not much 02
aorta now leaves form the right ventricle, visa versa
ductus arteriosus will still circulate blood from left to the right, but sill not perfect

46
Q

Embryonic blood flow from heart?

A

Blood is pumped by the early ventricle, aortic sac, aortic aortic arches, dorsal aorta
left and right dorsal aortae fuse into single aorta that supplies the rest of the embryo

47
Q

Vitelline arteries?

A

take blood into the yolk sac

48
Q

Umbilical arteries?

A

carry deoxygenated blood to the placenta

49
Q

Aortic sac makes?

A

the ascending aorta and the right brachiocephalic trunk

50
Q

1st and 2nd arches?

A

dont worry about

51
Q

3rd arch?

A

left and right common and internal carotid arteries

52
Q

4th arch?

A

right- part of right subclavian artery

left- part of the aortic arch

53
Q

5th arch?

A

nonexistant in humans

54
Q

6th arch?

A

right- right pulmonary artery

left- left pulmonary artery and ductus arteriosus/ligamentum arteriosum

55
Q

Dorsal aorta?

A

right- part of the right subclavian artery

left- arch of the aorta and descending aorta

56
Q

Double aortic arch?

A

if the right dorsal aorta remains intact below the 7th intersegmental artery, it will migrate toward the left side during subsequent growth and constrict the trachea and esophagus

57
Q

Aberrant origin of right subclavian artery?

A

similarily, if the right dorsal aorta remains intact below the 7th intersegmental artery but disappears more superiorly, instead of a double aortic arch, the right subclavian artery passes posteriorly behind the trachea and esophagus

Fortunately this does not usually compress them severely

58
Q

Vascular rings/ slings?

A

occurs when abberant vascular structure crosses behind and compresses the trachea/
ex) double aortic arch

59
Q

Examples of vascular rings/ slings?

A 
double arch
double arch atretic segment
left arch: normal 
right arch: mirror
left arch aberrant right SCA
right arch aberrant left SCA
60
Q

Vascular rings/ slings diagnosis?

A

definitive diagnosis by CTA

an easy, readily available test is the barium esopha

61
Q

Vascular rings/ slings treatment?

A

severe, symptomatic cases require vascular surgery to correct constriction

62
Q

Coarctation of the aorta?

A

can occur anywhere from the transverse arch to the iliac bifurcation
usually below the ductus arteriosus
mild forms are asymptomatic
severe obstruction can lead to lower body hypoperfusion and/or heart failure

narrowing leads to increased turbulance, led to increased atherosclerosis

63
Q

3 pairs of veins empty into sinus venosus?

A

vitelline, umbilical, cardinal

64
Q

Vitelline vein empties?

A

blood from the yolk sac

65
Q

umbilical vein empties?

A

oxygenated blood from the placenta

66
Q

Cardinal vein empties?

A

venous blood from the embryo itself

67
Q

Malformations of the cardinal veins?

A

result in abnormal venous drainage

68
Q

TAPVR/ total anomalous pulmonary venous return?

A

veins connect to vena cava deoxy blood not direct into right side of heart

69
Q

Fetal blood flow?

A

Oxy blood from umbilical v passes through the liver via the ductus venosus to reach the IVC, mingles with the deoxy blood, IVC enters the right atrium and much of the blood is preferentially shnuted through the foramen ovale to the left atrium
blood flows into the left ventricle and is then pumped out the aorta to the rest of the body

70
Q

Blood not pumped to fetal body?

A

some blood remains in right atrium and is pumped into the right ventricle and through pulmonary artery to the lungs
pressure from fluid in the lungs cause most of this blood to shunt through the ductus arteriosus to reach the aorta, blood in aorta travels throughout the body, a significant amount of aortic blood travels through the 2 umbilical arteries to reach the placenta and begin the process anew

71
Q

Transitional circulation?

A

at birth, expansion of the lungs and increase in arterial P02 results in rapid decrease in pulmonary vascular resitance

72
Q

Removing the low resistance placental circulation leads to an increase in systemic resistance casuing?

A

foramen ovale to close
ductus arteriosus to reverse to a L to R shunt
high arterial P02 then constricts ductus arteriosus until it closes and becomes the ligamentum arteriosum

73
Q

Persistant pulmonary hypertension of the newborn (PPHN)?

A

failure of normal circulatory transition
pulmonary vascular resistance remains elevated
continued right to left shunting results in severe hypoxemia
previously mortality was as high as 60% but ECMO has reduced substantially
current mortality still 10% with higher numbers suffering permanent neurodevelopmental impairment

MOD 1 Test (5 decks)

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