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Cardiovascular physiology > Lecture 5 > Flashcards

Lecture 5 Flashcards

Congenital heart defects

1
Q

What is a congenital heart defect?

A

structural abnormalities of the heart present from birth. They affect blood flow through the heart and to the rest of the body.

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2
Q

How many babies are born with some kind of congenital heart defect?

A

1 in 100

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3
Q

How many CHD are chromosomal?

A

16%

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4
Q

How many CHD are from maternal diabetes?

A

2%

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5
Q

How many CHD are from syndromes?

A

2%

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6
Q

How many CHD are from unknown environmental factors?

A

80%

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7
Q

What is the first organ in the body to develop?

A

The heart

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8
Q

What is the thick layer of cardiac muscle in the heart called?

A

Myocardium

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9
Q

What are the individual cells that make up the myocardium called?

A

Cardiomyocytes

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10
Q

What is the endothelial layer that lines the inside of the heart called?

A

The endocardium

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11
Q

What does the endocardium give rise to?

A

The endocardial cushions which are precursors of the valves and membranous septa.

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12
Q

What cells are essential components of the outflow tract cushions?

A

Neural crest cells

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13
Q

What are the great arteries coated in?

A

Smooth muscle cells

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14
Q

What is the single layer of epithelial cells lining the heart called?

A

Epicardium

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15
Q

What is the whole heart enclosed in?

A

The pericardium.

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16
Q

How long is the embryonic stage of development and what occurs?

A

8 weeks. Many differentiation and formation of the different organs. Differentiation of cells.

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17
Q

How long is the foetal stage of development and what occurs?

A

Last 32 weeks of pregnancy. Mainly growth of the foetus.

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18
Q

What are the three distinct layers that form during gastrulation?

A

Ectoderm, mesoderm and endoderm

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19
Q

What does the ectoderm fold into?

A

The neural tube (brain and spinal cord)

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20
Q

What two distinct regions does the mesoderm become?

A

Splenic mesoderm and the pre-myocardium

21
Q

What do the two types of mesoderm form?

A

The cardiac crescent

22
Q

What are the two fields of cell types that form the hear?

A

Primary and secondary heart fields

23
Q

What do cells from the primary heart field become?

A

The left ventricle and some atria

24
Q

What do cells from the secondary heart field become?

A

The right ventricle, outflow tract and most atria.

25
Q

What are the two types of foetal shunts?

A

Arterial duct and foreman ovale

26
Q

What type of shunt is the arterial duct?

A

Right to left

27
Q

Where is the foreman ovale?

A

Between the atria. It is also a right-to-left shunt.

28
Q

In what CHD is keeping the arterial duct open beneficial?

A

Transposition of the great arteries.

29
Q

What is produced during pregnancy to keep the arterial duct open?

A

Prostaglandins PGE1 and PGE2

30
Q

What receptors do the prostaglandins act on?

A

EP2 and EP4

31
Q

Which prostaglandin and receptor pair is major in induced AD dilation?

A

PGE2 and EP4. Levels of their expression go down after birth to close the duct.

32
Q

What are the three types of intracardiac malformations?

A

ASD, VSD and tetralogy of Fallot

33
Q

What type of shunt is VSD and ASD?

A

left-to-right

34
Q

What three things is occurring in tetralogy of Fallot?

A

VSD, overriding aorta and pulmonary stenosis. Causes a right to left shunt.

35
Q

What is a symptom of ToF?

A

Blue colour/cyanosis from right-to-left shunt

36
Q

What are the valves derived from?

A

endocardial cushions

37
Q

Where do cardiac neural crest cells stem from?

A

The cranial portion of the neural tube

38
Q

Where do cardiac neural crest cells migrate through?

A

Pharyngeal arches 3, 4 and 6 to form the outflow tract, valves and arteries.

39
Q

What are three types of outflow tract malformations?

A

Transposition of great arteries, double-outlet right ventricle and common arterial trunk

40
Q

Which is most severe?

A

CAT

41
Q

What other defect does DORV have to come with or it would be fatal?

A

VSD

42
Q

Which process does not occur properly in DORV?

A

twisting

43
Q

What is CAT?

A

Common arterial trunk. septation does not occur so there is just one outflow tract.

44
Q

How many cusps does each valve have?

A

mitral valve is bicuspid, all rest are tricuspid (aortic, pulmonary and right AV valve)

45
Q

What is hypoplastic left ventricle syndrome?

A

Narrowed aorta and very small left ventricle

46
Q

What mutation is present in 65% of people with charge syndrome?

A

CHD7 on chromosome 8

47
Q

In down syndrome, what are the more common heart defects?

A

AV septal defects or VSD. 5% have tetralogy of Fallot.

48
Q

What gene defect is associated with Holt Oram syndrome?

A

TBX5

49
Q
A

Cardiovascular physiology (15 decks)

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