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2nd year > Congenital heart abnormalities > Flashcards

Congenital heart abnormalities Flashcards

1
Q

what are congenital cardiac abnormalities associated with?

A

chromosomal abnormalities

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2
Q

what causes congenital cardiac defects?

A

chromosomal abnormalities
teratogens
mostly multi-factorial complex interplay between genetic and environmental factors
maternal disease - such as diabetes

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3
Q

embryological origin of heart

A

heart tubes from lateral plate mesoderm

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4
Q

when do heart tubes begin to form?

A

4th week of development

single primitive heart tube forms and then week 5-8 it septates

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5
Q

when does the heart begin to beat?

A

day 22 of development

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6
Q

when does blood begin to circulate?

A

day 24 of development

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7
Q

what causes vascular abnormalities?

A

failure of remodelling

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8
Q

right sided aortic arch

A

due to involution of left aortic arch and failure of involution of right aortic arch

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9
Q

persistence of both primitive aortic arches

A

results in vascular ring encircling the oesophagus and trachea, potentially constricting these and causes breathing difficulties or dysphagia

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10
Q

foramen ovale

A

between atria in fetal heart

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11
Q

ductus arteriosus

A

between pulmonary circulation and aorta in fetal heart

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12
Q

what forms the outflow tract of ventricles?

A

conus cordis

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13
Q

abnormalities of lateralisation

A

reversal of direction of heart tube folding

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14
Q

what are abnormalities of lateralisation?

A

dextrocardia
situs inversus
levocardia

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15
Q

dextrocardia

A

heart on right but abdominal contents are normally positioned (situs solitus) discordance between viscera and so there are often cardiac abnormalities, such as atrioventricular discordance, septal defects, single ventricle, transposition of great arteries, anomalous pulmonary venous return

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16
Q

situs inversus

A

reversal of laterality of thoracic and abdominal organs

  • concordance between reversal of laterality can mean patients are asymptomatic
  • patients with situs inversus not particularly at increased risk of cardiac defects but increased chance of pulmonary disease
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17
Q

Kartagener’s syndrome

A 
disorder of ciliary motility:
chronic sinusitis 
nasal polyposis 
bronchiectasis 
risk in situs inversus
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18
Q

levocardia

A

normal thoracic viscera positioning
reversed abdominal contents positioning
rarer
discordance means there are congenital defects in 95% of patients

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19
Q

what forms the vena cava?

A

sinus venosus

becomes incorporated into right atrium

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20
Q

what forms the pulmonary veins?

A

left atrium

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21
Q

crista terminalis

A

border between trabeculated and smooth walled part of right atrium

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22
Q

left atrial appendage

A

primitive left atrium

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23
Q

when does atrial septation occur?

A

week 4 and onwards

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24
Q

what divides the common primitive atria?

A

septum primum and septum secondum

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25
Q

how is the foramen ovale formed?

A

opening left by septum secondum

26
Q

what is the foramen ovale?

A

shunt from right to left atrium

27
Q

fossa ovalis

A

closure of foramen ovale

can be seen on medial wall of right ventricle

28
Q

what causes an ASD?

A

failure of formation of atrial septa

29
Q

what causes a patent foramen ovale?

A

failure of fusion of septum primum and secondum = PFO

30
Q

ASD prevalence

A

10% of congenital cardiac defects

31
Q

ASD

A

continuous left to right shunting of blood
impacts right side of heart
risk of paradoxical embolus

32
Q

Paradoxical embolus in PFO

A

thrombus passing between venous and arterial circulation

indication for closure of PFO

33
Q

PFO

A

common
present in 20-30% of population
often asymptomatic

34
Q

risks of PFO

A

paradoxical embolus if right atrial pressure exceeds left side in pulmonary disease

35
Q

atrioventricular canal defect

A

failure of formation of endocardial cushions
affects all chambers of heart
can be associated with chromosomal abnormalities, specifically trisomy 21

36
Q

atrioventricular canal defect

A

frequent communication between sides of the heart
increased right sided heart pressure
pulmonary hypertension
heart failure
can cause recurrent respiratory tract infections

37
Q

when is the ventricular septum formed?

A

end of week 4

38
Q

formation of interventricular septum

A

involves neural crest cells

39
Q

trabeculated part of right ventricle

A

bulbus cordis

40
Q

trabeculated part of left ventricle

A

common primitive ventricle

41
Q

what causes VSD?

A

failure of formation of ventricular septum

most common congenital cardiac abnormality in children and 2nd most common in adults

42
Q

which part is most commonly affected in VSD?

A

membranous part of ventricular septum

43
Q

VSD symptoms

A

vary depending on severity and size

can be asymptomatic if there is minimal shunting or can show symptoms of heart failure

44
Q

what are the symptoms of heart failure?

A 
hepatomegaly
failure to thrive 
exertional dyspnoea 
raised JVP
peripheral oedema
45
Q

mitral valve

A

anterior and posterior cusps

46
Q

tricuspid valve

A

anterior, posterior and septal cusps

47
Q

what forms the aortic and pulmonary trunks?

A

truncus arteriosus

48
Q

neural crest cells

A

influence development of cardiac septa and outflow tracts

49
Q

failure of formation of conotruncal septa

A

results in persistent truncus arteriosus

50
Q

persistent truncus arteriosus

A

single blood vessel carrying blood from heart to systemic, pulmonary and coronary circulations
always associated with VSD

51
Q

how does persistent truncus arteriosus present?

A

congestive heart failure

cyanosis

52
Q

what causes transposition of great vessels?

A

failure of correct formation of conotruncal septa

53
Q

transposition of great vessels

A

aorta arises from right ventricle and pulmonary outflow tract arises from left ventricle

54
Q

transposition of great vessels presentation

A

within 24 hours

cyanosis

55
Q

tetralogy of fallot

A

pulmonary infundibular stenosis, VSD, overriding aorta and right ventricular hypertrophy (develops after birth)

56
Q

tetralogy of fallot presentation

A

cyanosis

right to left shunting of blood

57
Q

transition to post-natal circulation

A

at birth pulmonary vascular resistance falls and blood circulates through lungs
patent ductus arteriosus and foramen ovale close forming ligamentum arteriosum and fossa ovalis

58
Q

failure of closure of foramen ovale

A

PFO

59
Q

failure of closure of ductus arteriosus

A

= patent ductus arteriosus, in some circumstances can be necessary to preserve life where there is structural heart disease, e.g. tetralogy of fallot
- patent ductus arteriosus symptoms vary depending on size

60
Q

presentaation of patent ductus arteriosus

A

if large can present within 1 year of birth
failure to thrive
breathing difficulties
small one may not present until adulthood

61
Q

uncorrected patent ductus arteriosus

A

can lead to pulmonary hypertension and right sided heart failure

2nd year (48 decks)

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