Congenital Heart Defects Flashcards

1
Q

what is a patent ductus arteriosis (PDA)?

A

failure of the ductus arteriosus to close

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2
Q

when does ductus arteriosus usually close?

A

stops functioning within 1-3 days of birth and completely closes within 2-3 weeks of birth

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3
Q

risk factors for PDA?

A

rubella

prematurity

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4
Q

presenting features of a small PDA

A

can be asymptomatic causing no functional problems and will close spontaneously
can sometimes be asymptomatic throughout childhood and present in adulthood with sings of heart failure

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5
Q

what is the ductus arteriosus?

A

connection between the aorta and pulmonary artery which exists during foetal development

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6
Q

function of ductus arteriosus?

A

foetal blood gets oxygen from the placenta rather than the lungs during development (the lungs are filled with amniotic fluid)
therefore blood does not need to go through the lungs so the ductus arteriosus acts as a shunt to bypass the lungs so it goes straight from pulmonary artery into aorta

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7
Q

why is PDA an issue?

A

pressure higher in aorta than in pulmonary artery therefore blood will flow from aorta to pulmonary artery
this creates a left to right shunt
this increases pressure in the pulmonary vessels causing pulmonary hypertension and leads to right heart strain as the right side of the heart struggles to contract against the increased resistance in the vessels
pulmonary hypertension and right heart strain lead to right ventricular hypertrophy and increased volume of blood flowing through pulmonary circulation back to left side of heart causes LVH

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8
Q

how does PDA present?

A
often picked up on newborn examination via machine like murmur
can have symptoms of
- short of breath
- difficulty feeding
- poor weight gain
- lower resp tract infection
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9
Q

what is the murmur like in PDA?

A

small PDA might not have any abnormal sounds
larger PDAs cause a normal first heart sounds with a continuous crescendo/decrescendo “machine like” murmur that may continue through second heart sound

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10
Q

how is PDA diagnosed?

A

echo
doppler during echo can help assess size and characteristics of the left to right shunt
echo can also show any complications like hypertrophy

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11
Q

how is PDA managed?

A

monitored until 1 year old using ECHO
if still open after 1 year old trans-catheter or surgical closure can be done
(treated earlier if symptomatic or evidence of heart failure due to PDA)

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12
Q

why are babies with PDA only monitored up until 1 year old?

A

PDA unlikely to close on its own after 1 year

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13
Q

how do atria form?

A

both atria are connected during development
2 walls grow downwards from top of heart then fuse together with the endocardial cushion in the middle of the heart to separate the atria
(2 walls called septum primum and septum secondum)

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14
Q

what hole exists in the septum secondum during development?

A

foramen ovale

usually closes at birth

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15
Q

what does an atrial septal defect (ASD) lead to?

A

shunt of blood between atria (mainly from left to right atrium since pressure is higher in left)
this means blood continues to flow to pulmonary vessels and lungs
this can lead to right heart strain, right heart failure and pulmonary hypertension

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16
Q

what is eisenmenger syndrome?

A

complication of pulmonary hypertension in ASD where the pulmonary pressure is greater than systemic pressure
this reverses the shunt and forms a right to left shunt across the ASD
this means blood will move from rigth atria to left atria and bypass the lungs causing the patient to become cyanotic

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17
Q

what are the 3 types of ASD?

A

ostium secondum = septum scondum fails to close
ostium primum = when septum primum fails to close
patent foramen ovale

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18
Q

4 possible complications of ASD?

A

stroke (in context of venous embolism as DVT can travel to the brain instead of the lungs via the septal defect)
AF or atrial flutter
pulmonary hypertension and right sided heart failure
eisenmenger syndrome

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19
Q

ASD murmur?

A

mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border
fixed split 2nd heart sound

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20
Q

what is a split 2nd heart sound?

A

where you hear the aortic and pulmonary valves close at slightly different times
can be normal on inspiration but if fixed (there all the time) this can indicate ASD

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21
Q

why do you get a split 2d heart sound in ASD?

A

bc blood is flowing from left atrium into right atrium across ASD
this increases the volume of blood that the right ventricle has to empty before the pulmonary valve can close

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22
Q

how does ASD usually present?

A
murmur
can be asymptomatic in childhood then present in adulthood with dyspnoea, heart failure or stroke
typical childhood symptoms
- short of breath
- difficulty feeding
- poor weight gain
- lower resp infections
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23
Q

how is ASD managed?

A

refer to paeds cardio for ongoing management
if small and asymptomatic watch and wait is enough
can be corrected surgically using transvenous catheter closure via femoral vein or open heart surgery
should give anticoagulants to reduce clot/stroke risk in adults

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24
Q

ventricular septal defect (VSD) is seen more often in which conditions?

A

down syndrome

turners syndrome

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25
Q

why is VSD a problem?

A

increased pressure in left ventricle compared to right therefore blood flows from left to right through the hole
leads to right sided overload, right heart failure and pulmonary hypertension which can eventually lead to eisenmenger syndrome

26
Q

how does VSD present?

A
often asymptomatic
can present late in adulthood 
can be picked up on antenatal scan or via a murmur during newborn baby check
typical symptoms
- poor feeding
- dyspnoea
- tachypnoea
- failure to thrive
27
Q

VSD murmur?

A

pan-systolic murmur heard best at left lower sternal border in 3rd/4th intercostal spaces
can have a systolic thrill on palpation

28
Q

how is VSD managed?

A

small VSD with no symptoms or pulmonary hypertension etc can just be monitored as they can close spontaneously
can be corrected surgically using transvenous catheter via femoral vein or open heart surgery
should be given antibiotic prophylaxis due to increased endocarditis risk

29
Q

what 4 pathologies make up tetralogy of fallot?

A

VSD
overriding aorta
pulmonary valve stenosis
RVH

30
Q

what is an overriding aorta?

A

refers to the fact that the entrance to the aorta (aortic valve) is placed further to the right than normal, sits above the VSD
this means that when the right ventricle contracts and sends blood upwards, the aorta is in the direction of travel of that blood so blood flows from right ventricle into aorta meaning a large amount of deoxygenated blood enters aorta

31
Q

influence of pulmonary valve stenosis in tetralogy of fallot?

A

stenosis of valve causes increases resistance against flow of blood from right ventricle into pulmonary artery
this encourages blood to flow from right ventricle into aorta via VSD
therefore overriding aorta and pulmonary valve stenosis encourage blood to be shunted from right to left causing cyanosis (blood not going to lungs to be oxygenated)

32
Q

what direction is blood shunted in tetralogy of fallot?

A

right to left

means blood bypasses the lungs causing cyanosis

33
Q

risk factors for tetralogy of fallot?

A

rubella
increased maternal age
alcohol during pregnancy
diabetic mother

34
Q

investigations in VSD?

A

echo
doppler during echo assesses severity of abnormality and shunt
CXR may show classic “boot shaped” heart due to right ventricular hypertrophy

35
Q

how does tetralogy of fallot present?

A

usually picked up during antenatal scans
ejection systolic murmur (pulmonary stenosis)
severe cases may present with heart failure before 1 year old
cyanosis
clubbing
poor feeding
poor weight gain
ejection systolic murmur heart loudest in pulmonary area (left sternal border, 2nd space)
“tet spells”

36
Q

what are tet spells?

A

intermittent periods where right to left shunt is temporarily worse precipitating a cyanotic episode

37
Q

when do tet spells occur?

A

when pulmonary vacular reistance increases or systemic resistance decreases (eg systemic vasodilation in exercise)

38
Q

how are tet spells managed?

A
positioning to increase systemic vascular resistance (squatting in older children, knees to chest in younger children)
oxygen
beta blockers
IV fluids
morphine
sodium bicarbonate
phenylephrine infusion
39
Q

how is tetralogy of fallot managed?

A

can give prostaglandin e2 in neonates to maintain the ductus arteriosus which will allow blood to flow from aorta back to pulmonary arteries
total surgical repair is definitive treatment but 5% mortality rate in surgery

40
Q

which anomalies can be associated with congenital heart defects?

A
downs
edwards (trisomy 18)
pataus (trisomy 13)
turners syndrome
di george
41
Q

what heart defect is downs associated with?

A

atrioventricular septal defect

42
Q

what heart defect is edwards syndrome associated with?

A

VSD

43
Q

which heart defect is pataus associated with?

A

VSD

44
Q

what heart defect is turners associated with?

A

coarctation of aorta

45
Q

what heart defect is di george associated with?

A

VSD

46
Q

how do most symptomatic heart defects present?

A

heart failure

blue tinge due to low oxygen sats

47
Q

why must hypoxia be corrected in babies?

A

hypoxia stunts growth

48
Q

normal resp rate in neonates?

A

<60

49
Q

is cyanosis always abnormal in babies?

A

no

can go blue quickly if cold

50
Q

normal place for apex in adults?

A

6th intercostal space mid-clavicular line

51
Q

normal place for apex in children up to 7?

A

4th intercostal space and more towards left

52
Q

grade 1 murmur?

A

slightest possible murmur

often missed

53
Q

grade 2 murmur?

A

slight murmur

should not be missed

54
Q

grade 3 murmur?

A

moderate murmur

no palpable thrill

55
Q

grade 4 murmur?

A

loud murmur with a palpable thrill

56
Q

grade 5 murmur?

A

very loud murmur with an easily palpable thrill

57
Q

grade 6 murmur?

A

extremely loud murmur

can be heard with the stethoscope not even touching the chest

58
Q

how common are murmurs in kids?

A

very

often go away and not a sign of congenital heart disease

59
Q

what is an innocent murmur?

A

normal, unproblematic murmur not related to congenital heart disease
patient is well
often not there all the time

60
Q

features of most innocent murmurs?

A

soft
short systolic
asymptomatic

61
Q

why do some kids with PDA need to keep it open?

A

mdkl