CVS Embryology and Congenital Disease Flashcards

1
Q

Where does the cardiogenic field lie within the emryo?

A

Cranial end

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2
Q

When do the endocardial tubes develop?

A

week 3

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3
Q

How is the heart tube created?

A

Lateral folding

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4
Q

How does cephalocaudal folding effect development of the heart?

A

Brings the tube into the thoracic region

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5
Q

At what end does blood enter the primitive heart?

A

Caudal through the sinus venous and pumping it out through the aortic roots.

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6
Q

What are the 4 segments of the primitive heart tube?

A

Primitive atrium
Primitive ventricle
Bulbous cordis
Truncus arteriosus

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7
Q

How and when does bending occur?

A

Continued elongation causes it to bend approx. 23rd - 28th day

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8
Q

How does the cephalic, cranial portion bend?

A

ventrally (forwards), caudally (downwards) and to the right

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9
Q

What way does the caudal portion bend?

A

Dorsally (backwards), cranially (upwards) and to the left

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10
Q

How does looping affect the orientation of the primitive heart?

A

Places both the inflow and outflow cranially with the inflow behind the outflow

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11
Q

How does the atrium and ventricle communicate after looping?

A

Via the atrioventricular canal - constriction between the 2 making the first division between them.

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12
Q

What is the Transverse Pericardial Sinus?

A

gap between the arteries (inflow in front) and veins (outflow behind) as a result of looping

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13
Q

What happens to the sinus horns?

A

Initially equal in size, but as venus return shifts to the right hand side the left horn recedes. The enlarging RA absorbs the right sinus horn.

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14
Q

How is the right atrium formed?

A

Develops from most of the primitive atrium and absorbs the right horn of the sinus venous. It receives venous drainage from the body and heart.

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15
Q

How is the left atrium formed?

A

Forms from a small portion of the primitive atrium and absorbs proximal parts of the pulmonary veins (first 4 branches). Receives oxygenated blood from the lungs.

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16
Q

How is the oblique pericardial sinus formed?

A

THe left atrium expands and absorbs the pulmonary veins

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17
Q

How do the great vessels develop?

A

Begin as a bilaterally symmetrical system of arched vessels that undergo extensive remodelling.

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18
Q

Which aortic arch has no derivatives in humans?

A

Arch 5

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19
Q

What arteries develop from arch 4?

A

from the right, the proximal part of the right subclavian artery
From the left, the arch of the aorta

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20
Q

What is derived from the 6th arch?

A

Recurrent laryngeal nerve
from the right, the right pulmonary artery
from the left, the left pulmonary artery and the ductus arteriosus

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21
Q

Where do endocardial cushions develop?

A

The dorsal and ventral sides of the atrioventricular canal (first step in septation)

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22
Q

What do the endocardial cushions do?

A

Grow towards each other forming the left and right channels of the heart

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23
Q

How many septa and holes form in the division of the atria?

A

2 septa, 3 holes

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24
Q

What is the first septa called?

A

Septa primum

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25
Q

What is the hole present before the fusion of the septum primum with the endocardial cushions called?

A

Ostium primum

26
Q

When does the ostium secundum form and what is it?

A

second hole in the septa primum formed before the closure of the ostium primum by apotosis.

27
Q

What is the hole in the septum secundum called?

A

Foramen ovale

28
Q

What are the 2 components of the ventricular septum?

A

Muscle (largest component)

Membrane

29
Q

In what direction does the muscular portion of the ventricular septum grow?

A

Upwards

30
Q

What is the hole at the top of the muscular septum called?

A

Primary interventriular foramen

31
Q

What fills the primary interventricular foramen?

A

The membranous portion of the septum

32
Q

Where is the membranous portion of the septum derived from?

A

The spiral septum that grows to separate the truncus artiousus into the outflow vessels.

33
Q

How does the foetus receive oxygenated blood?

A

Via the placenta and umbilical vein. This bypasses the lungs and returns to the placenta via the umbilical arteries

34
Q

What enables blood to bypass the liver?

A

ductus venosus (umbilical vein to inferior vena cava)

35
Q

What enables blood to bypass the lungs?

A

ductus arteriosus (pulmonary trunk to aorta)

36
Q

Describe the pathway of blood through the foetus from the placenta

A

Passes through the Ductus Venosus into the vena cava via the umbilical vein.
Blood reaches the right atrium and shunted to the left through the foramen ovale
It is the pumped around the body via the aorta
Any blood in the pulmonary trunk passes through the ductus arteriosus into the aorta

37
Q

What happens when the baby takes it’s first breath?

A

THe pressure in the lungs massively decreases causing more blood to enter them and more blood to return to the left ventricle. The pressure in the left ventricle exceeds that of the right and the foramen ovale closes. The ductus arteriosus contracts. The ductus venosus closes as the placental support has been removed.

38
Q

What is the fate of the foramen ovale?

A

fossa ovalis

39
Q

what is the fate of the ductus arteriosus?

A

ligamentum arteriosus

40
Q

What is the fate of the ductus venous?

A

Ligamentum venosum

41
Q

What is the fate of the umbilical vein?

A

Ligamentum teres (hepatis)

42
Q

What is the most common heart defect?

A

Ventricular Septal Defects followed by Atrial Septal Defects

43
Q

What is an Atrial Septal Defect?

A

An opening in the septum between the two atria - Generally the foramen ovale fails to close however can occur anywhere in the septum

44
Q

What is the incidence rate of ASD?

A

67 in 100, 000 live births

45
Q

What is the result of ASD?

A

Shunt left -> right as left pressure > right.

This means there is no mixing of deoxygenated and oxygenated blood.

46
Q

What is a Patent Foramen Ovale?

A

A remnant of the foramen ovale

47
Q

What is the result of a PFO?

A

It is generally clinically silent since the higher left atrial pressure causes functional closure of the flap valves.

48
Q

How might a paradoxical embolism be caused by a PFO?

A

If pressure on the right side increases even slightly, a venous embolism may be shunted across and enter the systemic circulation causing arterial thrombosis

49
Q

What is a Ventricular Septal Defect?

A

An opening in the Interventricular Septum. Most commonly in the membranous portion of the septum, but can occur anywhere. Blood flows left to right due to the higher left ventricular pressure

50
Q

What is a patent ductus arteriosus?

A

Failure of the ductus arteriosus to close. Blood still flows from the aorta to the pulmonary artery.

51
Q

What is a mechanical murmur?

A

A sound heard throughout systole and diastole due to pressure in the aorta beign greater than in the pulmonary artery

52
Q

What can result from chronic left to right shunting?

A

Vascular remodelling of the pulmonary circulation and an increase in pulmonary resistance. If resistance increases beyond that of the systemic circulation the shunt will reverse direction

53
Q

What is coarctation of the aorta?

A

Narrowing of the aortic lumen in the region of the ligamentum arteriosum. This increases the afterload on the left ventricle and can lead to left ventricular hypertrophy.

54
Q

How does aortic coarctation affect the rest of the body?

A

THe head and upper limb vessels usually emerge proximally to the coarctation so are unaffected but blood supply to the rest of the body is reduced. The extent of the symptoms is dependent on the severity. In very severe cases, may present with symptoms of heart failure. Mild cases, may be detected in adult life. Femoral pulses will be weak and delayed and, with upper body hypertension.

55
Q

What is tetralogy of fallot?

A
A group of 4 lesions occurring together as a result of a single development defect placing the outflow portion of the interventricular septum too far in the anterior and cephallad directions
VSD
Overriding Aorta
Pulmonary Stenosis
Right Ventricular Hypertrophy
56
Q

What does problems does pulmonary stenosis cause?

A

right ventricular hypertrophy as teh right ventricle must operate at a higher pressure

57
Q

What causes right to left shunting (and therefore mixing of deoxygenated and oxygenated blood causing cyanosis) in tetralogy of fallot?

A

VSD, overriding aorta and the increased pressure on the right side of the heart due to pulmonary stenosis (pulmonary stenosis determines the severity)

58
Q

What is tricuspid atresia?

A

Lack of development of the tricuspid valve. There is no inlet to the right ventricle.

59
Q

How does the heart compromise for tricuspid atresia?

A

Complete shunt right to left by ASD or PFO and a VSD or PDA to allow blood to flow to the lungs.

60
Q

What is transposition of the great arteries?

A

Right ventricle is connected to the aorta and the left ventricle is connected to the pulmonary trunk.
2 unconnected parallel circulations

61
Q

How does the heart compromise for transposition of the great arteries?

A

It is not compatible with life after birth unless a shunt exists to allow the 2 circulations to communicate after birth until surgical correction can be made. The ductus arteriosus can b maintained or patent and/or an atrial septal defect formed.

62
Q

What is hypoplastic left heart?

A

Left ventricle and aorta fail to develop properly. A PFO or ASD are also present and blood supply to the systemic circulation is via a PDA. Without surgical correction, this is lethal.