Embryology and Congenital Heart Disease Flashcards

1
Q

what layer is the heart derived from?

A

visceral mesoderm

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2
Q

What type of folding brings the heart to its final position?

A

Cranio-Caudal folding

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3
Q

Name the 4 heart tube regions from top to the bottom

A

Truncus Arteriosus
Bulbus Cordis
Ventricle
Atrium

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4
Q

what heart tube region is responsible for aorta and pulmonary trunk?

A

truncus arteriosus

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5
Q

what heart tube region is responsible for RV and outflow of both ventricles?

A

bulbus cordis

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6
Q

what heart tube region is responsible for LV?

A

ventricle

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7
Q

what heart tube region is responsible for both atria?

A

Atrium

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8
Q

How many dilatations does the heart tube develop?

A

5

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9
Q

What is the direction of BF through the primitive heart?

A

caudal to cranial

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10
Q

What are the right and left horns called in atrium?

A

sinus venosus

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11
Q

d: sinus venosus

A

smooth part R atrium

Coronary sinus

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12
Q

What happens to atrium heart tube in looping and folding?

A

becomes dorsal and cranial

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13
Q

What happens to ventricle heart tube in looping and folding?

A

displaced left

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14
Q

What happens to bulbus Cordis heart tube in looping and folding?

A

inferiorly, ventrally to right

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15
Q

f: the placenta in utero (8)

A
Fetal homeostasis
Gas exchange
Acid base balance
Nutrient transport to fetus
Waste product transport from fetus
Hormone production
Transport of IgG
PGE2
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16
Q

How does the circulation differ in the fetus?

A
The placenta is included in the circulation:
Gas exchange
Nutrition
Waste excretion
Homeostasis
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17
Q

How do the lungs differ in the fetus?

A

they are unexpanded and fluid filled

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18
Q

How does the liver differ in the fetus?

A

has little role in nutrition and waste management

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19
Q

Is the gut in use in the fetus?

A

no

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20
Q

Describe fetal circulation route?

A

placenta to the R side of the heart to brain and body back to placenta etc

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21
Q

What arteries does the foetal heart use to pump blood to the placenta?

A

umbilical

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22
Q

How does blood from the placenta return to the foetus?

A

via umbilicial vein

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23
Q

How does oxygenated, nutrient rich blood return from the placenta to the right side of the heart and get distributed to the growing foetus?

A

3 “shunts” specific to foetal life:
Ductus venosus
Foramen Ovale
Ductus Arteriosus

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24
Q

what does the ductus venosus do?

A

connects the umbilical vein to the inferior vena cava

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25
Q

what does the foramen Ovale do?

A

opening in atrial septum connecting RA to LA

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26
Q

What does the ductus arteriosis do?

A

connects pulmonary bifurcation to descending aorta

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27
Q

WHere does the ductus venosus carry the majority of placental blood to and how?

A

straight into IVC bypassing portal circulation

28
Q

where does the foetus get its nutrients?

A

placenta

29
Q

Name 2 things the foramen ovale allows?

A

Allows blood to flow from right to left atrium

Allows the best oxygenated blood to enter left atrium then on to LV, ascending aorta, carotids
Membrane flap on left atrium side

30
Q

what percentage of blood goes to the lungs in foetus? where does the rest go and how?

A

7%

The rest goes via ductus arteriosus to join descending aorta

31
Q

How is patency maintained? how is this produced?

A

maintained by circulating prostaglandin E2 produced by placenta

32
Q

what happens immediately after baby born?

A

Massive changes in the few minutes following birth
Baby inflates lungs and cries
Goes from blue to pink
Cord clamped and cut

33
Q

describe the circulatory changes after birth?

A
Pulmonary Vascular Resistance decreases:
-breath in - lungs -physically expand
increased SV
-cord clamped and cut
more CO to the lungs
foramen ovale close
34
Q

why does the foramen ovale close?

A

As PVR falls and SVR rises the LA pressure exceeds the RA pressure
The flap is pushed closed

35
Q

Describe the adaption of the ductus arteriosus after birth?

A

Functional closure within hours to days
Anatomical closure within 7-10 days
Ends up as fibrous ligament – ligamentum arteriosum

36
Q

How does the ductus arteriosus constrict after birth?

A

Decreased flow due to decreased pulmonary vascular resistance
Increased pO2 – oxygen sensitive muscular layer
Decreased circulating PGE2 due to increased lung metabolism
Shunt becomes bidirectional then left to right

37
Q

What babies more commonly have failure to close from ductus arteriosus?

A

preterms

38
Q

treatments for failure of duct to close?

A

include wait and see, NSAIDs and surgery

39
Q

How can duct be kept open for duct dependent circulation until surgery carried out?

A

IV prostaglandin E2 can be used to keep the duct open until an alternative shunt established

40
Q

what happens to pulmonary resistance after birth?

A

continues to drop until reaches normal adult levels by 2-3months

41
Q

Name some of the consequences failures of adaptation

A

Persistent pulmonary hypertension of the newborn
More likely in sick babies
Sepsis
Hypoxic ischaemic insult
Meconium aspiration syndrome
Cold stress
Can be related to underlying anatomical abnormality such as congenital diaphragmatic hernia

42
Q

pathophysiology for Persistent pulmonary hypertension in newborn’s(PPHN)?

A

Lung vascular resistance fails to fall
Shunts remain
Right to left flow at PFO
Right to left flow at PDA

43
Q

What colour are babys with PPHN?

A

BLUE

44
Q

large/small difference between pre and post ductal O2 sats?

A

large

45
Q

treatment of PPHN?

A

Ventilation, oxygenation, high systemic blood pressure, inhaled nitric oxide, ECLS

46
Q

d: congenital heart disease

A

Abnormality of the structure of the heart or intrathoracic great vessels
Present at birth

47
Q

How do you find congenital heart disease?

A
Screening
-Antenatal
-Newborn baby check
Well baby with clinical signs
Unwell baby
-Cyanosis
-Shock
-Cardiac failure
48
Q

describe the 3 grades of congenital heart disease?

A

Mild = asymptomatic, Moderate = specialist intervention, Severe = present severely ill, Major = Surgery in 1st year

49
Q

Symptoms of congenital HD?

A

Unwell baby, cyanosis, shock, cardiac failure

50
Q

What is done to predict congenital defects?

A

antenatal screening

51
Q

d: blue baby syndrome

A

Cyanosis (blue baby) – anything where heart allows deoxygenated blood to enter circulation

52
Q

name some of the differntial diagnoses for blue baby syndrome?

A

Differential: cardiac disease, resp. disease, Persistent Pulmonary Hypertension of the Newborn (PPHN)

53
Q

Name the most common newborn cyanosis presenting example

A

transposition of great vessels

pulmonary and aortic vessels swapped

54
Q

clinical signs of failure in babies?

A
  • Failure to thrive
  • Slow/ reduced feeding
  • Breathlessness
  • Sweatiness
  • Hepatomegaly
  • Crepitations
55
Q

what is hypoplastic left heart?

A

left side of the heart underdeveloped

56
Q

what is pulmonary atresia and what is it coupled with?

A

underdeveelopment of right ventriclular outflow

often with VSD

57
Q

What is VSD?

A

ventricular septal defect

58
Q

treatment of duct closure issues?

A

ABC – support airway and breathing as necessary
Prostaglandin E2 to open duct
Multisystem supportive treatment
Transfer to cardiac surgical centre for definitive management

59
Q

name the duct dependent systemic circulation?

A

Hypoplastic left heart, critical aortic stenosis, interrupted aortic arch, critical coarctation of aorta

60
Q

name the duct dependent pulmonary circulation

A

Tricuspid atresia

Pulmonary atresia

61
Q

is there an initial murmur for large VSD?

A

Often no murmur at baby check

Murmur develops as pulmonary pressures drop over first weeks

62
Q

if theres a big VSD what is the gradient more/less?

A

less

63
Q

how is patent ductus arteriosus repaired?

A

cathether procedure

64
Q

What does VSD repair require?

A

surgery

65
Q

HLHS requires what for treatment?

A

3 Stage complex surgery
Significant mortality at each stage and between
Ends with RV supplying systemic circulation
Will fail over time
Transplant