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Y4 Paeds > Cardio - structural > Flashcards

Cardio - structural Flashcards

1
Q

what are the four features of tetralogy of fallot?

A

Ventricular septal defect (VSD)
Pulmonary stenosis (PS)
Right ventricular hypertrophy (RVH)
Overriding aorta

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2
Q

what % of congenital heart disease is tetralogy of fallot?:

A

10%

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3
Q

what are some risk factors for tetralogy of fallot?

A

male
1st degree FHx
teratogens - alcohol, warfarin, trimethadione
genetics - 22q11 deletion

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4
Q

what two components make up the septum of the ventricles?

A

smaller membraneous septum superiorly

larger muscular septum inferiorly

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5
Q

what is the most common type VSD?

A

perimembraneous VSD

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6
Q

state three types of VSD?

A

perimembraneous VSD
muscular VSD
doubly committed VSDs (near pulmonary and aortic valves)

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7
Q

why are most people with VSD acyanotic ?

A

the left ventricle has a higher pressure than the right ventricle so there is a left to right shunt which can have a complication of pulmonary hypertension and ultimately right to left shunt

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8
Q

what is the most common type of pulmonary stenosis?

A

infundibular septum

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9
Q

what % of PS involves valvular stenosis?

A

10%

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10
Q

what are tet spells ?

A 
intermittent RVOT (right ventricle outflow tract) obstruction 
- hypoxic episodes
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11
Q

what sign on CXR does RVH have?

A

boot sign

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12
Q

in severe tetralogy of fallot what may form around the aorta?

A

multiple aorto-pulmonary collateral arteries (“MAPCAs”) may also form to help increase pulmonary blood flow

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13
Q

what are the clinical signs of TOF?

A 
asymptomatic 
murmur 
cyanosis 
respiratory distress 
faltering growth
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14
Q

what ages are usually affected by tet spells ?

A

2-4 months of life

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15
Q

what three things characterise tet spells?

A

paroxysm of hyperpnoea
irritability
increasing cyanosis

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16
Q

what can precipitate hypoxic spells?

A

dehydration, anaemia, prolonged crying

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17
Q

what type of murmur is present with VSD?

A

pan systolic murmur

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18
Q

what murmur is present with dilated aorta?

A

ejection click

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19
Q

do murmurs increase or decrease in intensity during hypoxic spells?

A

decrease in intensity

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20
Q

what are some signs of congestive heart failure?

A

sweating, pallor, tachycardia, HSM, generalised oedema, bilateral basal crackles

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21
Q

what murmur is present with pulmonary stenosis?

A

loud single S2 murmur

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22
Q

what are DD for TOF?

A 
isolated VSD 
sepsis 
transposition of the great arteries 
hypo plastic left heart syndrome 
totally anomalous pulmonary venous drainage
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23
Q

what investigations should be done for TOF?

A 
ECG 
Microarray 
CXR
ECHO
cardiac CT angiogram 
cardiac MRI 
cardiac catheter
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24
Q

what medical treatment is available for TOF?

A

squatting - increases venous return and increases systemic resistance

prostaglandin infusion (PGE1 - alprostadil or PGE2 - dinoprostone)

beta blockers

morphine

saline 0.9% bolus (increases pulmonary flow through the RVOTO)

25
Q

what surgical treatment is available for TOF?

A

transcatheter RVOT sent insertion (done until child is bigger for valve replacement)
modified blalock taussig shunt (increases pulmonary flow)

stenosis resection, VSD repair

26
Q

what are the complications of TOF?

A 
Polycythaemia
Cerebral abscess
Stroke
Infective endocarditis
Congestive cardiac failure
Death (up to 25% in the 1st year of life4)
Arrhythmias
27
Q

when should TOF be followed up ?

A

life long

- with regular ECG, ECHO, cardiopulmonary exercise testing (CPET)

28
Q

what is the gold standard for diagnosing TOF?

A

echo

29
Q

what genetic defect has the highest percentage incidence of TOF associated with it?

A

CHARGE syndrome

30
Q

what gives the boot shaped heart on CXR?

A

RVH

31
Q

what is the mortality of TOF in the first year of life?

A

25%

32
Q

what is dextro-transposition of the great arteries?

A

the aorta is anterior and to the right of the pulmonary artery

33
Q

what is levo-transposition of the great arteries?

A

the aorta may be anterior and to the left of the pulmonary artery

34
Q

is dextro-transposition or levo-transposition more common?

A

dextro-transposition

35
Q

what is the most common cause of cyanosis in a new born?

A

transposition of great arteries

36
Q

what gender is most affected by transposition of great arteries ?

A

males

37
Q

what three locations can mixing of blood occur to sustain life during transposition of great arteries ?

A

patent foramen ovale
ventricular septal defect
patent ductus arteriosus

38
Q

what valvular problem can result from levo-TGA ?

A

tricuspid regurgitation and heart failure due to high pressure on left side of heart

39
Q

what are maternal risk factors for TGA?

A 
>40yrs 
DM
rubella 
poor nutrition 
alcohol
40
Q

what would be found on examination with TGA?

A

Prominent right ventricular heave
Single second heart sound, loud A2
Systolic murmur potentially if VSD present
No signs of respiratory distress

41
Q

what would be since on ECG for tricuspid atresia?

A

left axis deviation

42
Q

what would be seen on CXR for TGA?

A

egg on a string

43
Q

what investigation is used to diagnosis TGA?

A

echo

44
Q

what is the management of TGA?

A

Emergency prostaglandin E1 infusion to keep the ductus arteriosus patent as a temporary solution that allows mixing of blood

Correct metabolic acidosis

Emergency atrial balloon septostomy to allow for mixing

45
Q

what are the complications of TGA?

A

Neopulmonary stenosis
Neoaortic regurgitation
Neoaortic root dilatation
Coronary artery disease

46
Q

what acid base imbalance does TGA have?

A

metabolic acidosis

- distal organs have low oxygen supply and this respire anaerobically producing lactate

47
Q

what two things are poor development predictors for TGA?

A

low gestational age

high pre operative lactate

48
Q

what is the most common congenital heart defect?

A

VSD

49
Q

what is right to left shunt called?

A

Eisenmengers syndrome

50
Q

what are risk factors for VSD?

A 
maternal DM 
rubella 
alcohol 
uncontrolled maternal phenylketonuria 
FHx of VSD 
Downs syndrome 
Trisomy 18/13
51
Q

what clinical features will patients with small VSD have?

A

systolic murmur

asymptomatic

52
Q

what clinical features will patients with moderate VSD have?

A

excessive sweating
tachypnoea
most obvious at 2-3months

53
Q

what clinical features will patients with large VSD have?

A

symptoms of congestive heart failure
development problems
frequent chest infections

54
Q

what are some DD for VSD?

A 
mitral regurg 
tricuspid regurg 
ASD
patent ductus arteriosus 
pulmonary stenosis 
tetralogy of fallot
55
Q

what is the medical management of VSD?

A

increased caloric density of feedings - ensures weight gain

diuretics

ACEi

digoxin - increases heart muscle strength during heart failure

56
Q

what advice should be given long term management of VSD due to increased risk of endocarditis ?

A

good dental hygiene

avoid piercing and tattoos

57
Q

what are complications of VSD?

A 
Congestive heart failure
Growth failure
Aortic valve regurgitation 
Eisenmenger’s Syndrome
Frequent chest infections
Infective Endocarditis
Arrhythmias
Sudden death
58
Q

what % of small VSDs close spontaneously by 10years?

A

75%

59
Q

what % of downs syndrome have cardiac abnormalities ?

A

40-50%

Y4 Paeds (15 decks)

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