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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
What closes the atrioventricular canals?
closed by formation of the tricuspid valve on the right and bicuspid valve on the left
26
Atrioventricular canal defect?
aka endocardial cushion defect | partial or complete
27
Parial Atrioventricular canal defect?
essentially a severe low ASD or high VSD with the AV valves affected but present
28
Complete atrioventricular canal defect?
the av valves are not developed and all four chambers of the heart are contiguous
29
Form the heart valves?
ventricular mesenchyme below the atrioventricular canals "hollows out" leaving behind valves, chordae tendinae and papillary muscles
30
Fault AV valve formation may result in ?
stenosis, atresia, blood reguritation and/or murmurs
31
Left ventricular outflow tract obstruction?
spectrum of pathology involving subvalvular, valvular, or supravalvular portion of the aortic valve complex
32
Milder Left ventricular outflow tract obstruction?
Aortic stenosis | -murmur, increased left sided pressures can lead to heart failure if left untreated
33
Severe Left ventricular outflow tract obstruction?
Hypoplastic left heart syndrome | -cyanotic newborn
34
Right Ventricular Outflow Tract Obstruction?
Spectrum of pathology invlolving subvalvular, valvular or supravalvular portion of the pulmonary valve complex
35
Milder Right Ventricular Outflow Tract Obstruction?
Pulmonary stenosis | -murmur, increased right sided pressures can lead to heart failure if left untreated
36
Severe Right Ventricular Outflow Tract Obstruction?
Tetrology of fallot | -cyanotic newborn
37
Very severe Right Ventricular Outflow Tract Obstruction?
hypoplastic right heart syndrome
38
Tetrology of fallot?
pulmonary stenosis right ventricular hypertrophy overriding aorta VSD
39
tricuspid atresia?
PFO or ASD Atresia VSD
40
Conus cordis, truncus ateriosus, and aortic sac subdivide to become?
the proximal aorta and pulmonary vessels
41
Truncus arteriosus initially?
a single tube leading to the aortic sac, subdivides into two tubes by conotruncal ridges that come largely from neural crest cells
42
The conotruncal ridges spiral?
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
the membranous portion of the interventricular septum meets what from below?
the conotruncal ridge and completely seperates the aorta from the pulmonary trunk
44
Truncus ateriosus?
mixing lesions, cyanotic heart lesion | only one large vessel exists the heart
45
Transposition of the great vessels?
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
Embryonic blood flow from heart?
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
Vitelline arteries?
take blood into the yolk sac
48
Umbilical arteries?
carry deoxygenated blood to the placenta
49
Aortic sac makes?
the ascending aorta and the right brachiocephalic trunk
50
1st and 2nd arches?
dont worry about
51
3rd arch?
left and right common and internal carotid arteries
52
4th arch?
right- part of right subclavian artery | left- part of the aortic arch
53
5th arch?
nonexistant in humans
54
6th arch?
right- right pulmonary artery | left- left pulmonary artery and ductus arteriosus/ligamentum arteriosum
55
Dorsal aorta?
right- part of the right subclavian artery | left- arch of the aorta and descending aorta
56
Double aortic arch?
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
Aberrant origin of right subclavian artery?
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
Vascular rings/ slings?
occurs when abberant vascular structure crosses behind and compresses the trachea/ ex) double aortic arch
59
Examples of vascular rings/ slings?
``` double arch double arch atretic segment left arch: normal right arch: mirror left arch aberrant right SCA right arch aberrant left SCA ```
60
Vascular rings/ slings diagnosis?
definitive diagnosis by CTA | an easy, readily available test is the barium esopha
61
Vascular rings/ slings treatment?
severe, symptomatic cases require vascular surgery to correct constriction
62
Coarctation of the aorta?
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
3 pairs of veins empty into sinus venosus?
vitelline, umbilical, cardinal
64
Vitelline vein empties?
blood from the yolk sac
65
umbilical vein empties?
oxygenated blood from the placenta
66
Cardinal vein empties?
venous blood from the embryo itself
67
Malformations of the cardinal veins?
result in abnormal venous drainage
68
TAPVR/ total anomalous pulmonary venous return?
veins connect to vena cava deoxy blood not direct into right side of heart
69
Fetal blood flow?
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
Blood not pumped to fetal body?
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
Transitional circulation?
at birth, expansion of the lungs and increase in arterial P02 results in rapid decrease in pulmonary vascular resitance
72
Removing the low resistance placental circulation leads to an increase in systemic resistance casuing?
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
Persistant pulmonary hypertension of the newborn (PPHN)?
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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