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MBChB IV Paediatrics > Congenital heart diseases > Flashcards

Congenital heart diseases Flashcards

1
Q

Increased PBF clinical presentation

A 
Failure to thrive
Chest deformity
Cyanotic/acyanotic
Shortness of breath
Sweating
Poor feeding
CCF
Cardiomegaly
Congest
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2
Q

Cyanotic

Increased PBF

A 
TGA
Persistent truncus arteriosus
TAPVD
HLHS
Eisenmenger syndrome
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3
Q

Cyanotic

Decreased PBF

A 
TOF
Tricuspid atresia
Pulmonary atresia
Critical pulmonary stenosis
Ebstein's anomaly
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4
Q

Acyanotic

Increased PBF

A 
LR shunt
ASD
VSD
AVSD
PDA
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5
Q

Acyanotic

Normal PBF

A 
Aortic stenosis
Coarctation
Pulmonary stenosis
Tricuspid regurgitation
Mitral regurgitation
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6
Q

Aetiology

A 
Diabetes
Rubella
SLE
Warfarin
Alcohol
Chromosomal
Genetic syndromes
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7
Q

What is the clinical presentation of an acyanotic normal pulmonary blood flow congenital heart lesion?

A

Asx
Ventricle hypertrophy (displaced apex, LPH)
Low CO

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8
Q

What is the clinical presentation of an acyanotic increased pulmonary blood flow congenital heart lesion?

A 
Pulmonary oedema
Chest deformities
Cardiomegaly
CCF
PHT
FTT
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9
Q

What is the clinical presentation of a cyanotic increased pulmonary blood flow congenital heart lesion?

A 
Pulmonary oedema
Chest deformities
Cardiomegaly
CCF
PHT
FTT
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10
Q

What is the clinical presentation of a cyanotic decreased pulmonary blood flow congenital heart lesion?

A

Severe cyanosis

Stunting

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11
Q

When will cyanosis become apparent?

A

Sats <85%

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12
Q

Name the causes of cyanosis in an infant

A
  1. Airway
    - choanal atresia
    - laryngomalacia
    - vocal cord paralysis
    - tracheal stenosis
    - vascular ring
    - external mass
  2. Breathing
    - pneumonia
    - bronchiolitis
    - congenital lung abnormality
    - congenital diaphragmatic hernia
  3. Circulation
    - O2 carrying
    - congenital cardiac lesions
    - PPHN
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13
Q

Which syndrome is associated with Ebstein anomaly?

A

Wolff Parkinson White (supraventricular tachycardia)

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14
Q

What is the optimal gestational age for screening cardiac defects?

A

18-22w

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15
Q

When should urgent echo be performed in a child 1-2 days old?

A

R hand or legs <90% sats
OR
R hand or legs 90-95%/3% difference on 3 different occasions

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16
Q

Ebstein’s anomaly is linked with which drug?

A

Lithium

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17
Q

What are the features of Ebstein’s anomaly?

A

Tricuspid regurg
RV atrialization (hypertrophy)
Assoc w/ PFO, ASD
PSM at LLSB

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18
Q

Which congenital heart defect improves with age?

A

Ebstein’s anomaly (pulmonary pressure decreases)

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19
Q

What are the features of tricuspid atresia?

A

Absent tricuspid valve
RV hypoplasia
Assoc w ASD, VSD
RA dilation

PSM at LLSB
Left axis deviation (LVH)
Jugular venous distention with a prominent A wave

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20
Q

What are the features of persistent truncus arteriosus?

A

VSD
Single S2
PSM at LLSB w/wo MDM

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21
Q

Which syndrome is associated with persistent truncus arteriosus?

A

DiGeorge Syndrome

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22
Q

What are the features of ToF?

A

Pulmonary infindibular stenosis
Overriding aorta
VSD
RVH

ESM LLSB

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23
Q

Which syndromes are associated with ToF?

A

DiGeorge syndrome

Down’s syndrome

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24
Q

How do you manage a hypercyanotic spell?

A 
Knee chest position
100% Oxygen (usually has minimal effect)
Morphine 0.1 mg/kg
- calm child
- relax infindibulum
Fluid bolus 10 - 20 ml/kg crystaloid /colloid
- incr preload, incr SVR and LV pressure
Sodium Bicarb 
- severe acidosis
B blocker (esmolol 0.5 mg/kg stat, then 0.1 mg/kg/min)
- incr CO
Phenylephrine
- incr SVR 
Emergency surgery
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25
Q

What are the features of pulmonary atresia?

A

PDA
ASD
VSD

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26
Q

How do you manage all PDA-dependent lesions?

A

PGE1 IVI
PGE2 orally
Treat metabolic acidosis
Refer

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27
Q

At what age are cyanotic incr PBF lesions common?

A

<3-6mo

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28
Q

What are the features of transposition of the great vessels?

A

PFO, ASD
PDA
VSD

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29
Q

What is an intrauterine risk factor for transposition of the great vessels?

A

Infant to diabetic mother

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30
Q

What are the features of TAPVD?

A

ASD
PDA
RVH

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31
Q

What syndromes are associated with HLHS?

A

Trisomy 13
Trisomy 18
Jacobsen syndrome
Turner syndrome

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32
Q

What are the features of HLHS?

A

ASD

PDA

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33
Q

Name complications of cyanosis

A

Polycythaemia
CVS events
Clotting risk
Bleeding risk

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34
Q

What is the most common congenital heart defect?

A

VSD

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35
Q

What murmur is caused by a VSD?

A

Pansystolic at LLSB

-> pressure LV > RV in systole, no difference in diastole

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36
Q

What changes occur with a VSD?

A

Left heart dilation -> PA pressure rises -> RH hypertrophy -> PA pressure rises higher -> RH hypertrophy increases -> PA pressure rises -> pressures balance and PSM disappears -> shunt reverses (Eisenmenger syndrome)

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37
Q

How do you manage a VSD?

A

Manage CCF
IE prophylaxis
Closure - surgery/cath lab

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38
Q

What murmur is caused by a PDA?

A

Continuous murmur at ULSB

-> pressure during both systole and diastole AO > PA

39
Q

What is the importance of Eisenmenger syndrome in PDA?

A

Cyanotic only in feet!

Aortic arch perfuses upper body

40
Q

How do you manage a PDA?

A

Indomethacin/aspirin
Manage CCF
IE prophylaxis
Closure - surgery/cath lab

41
Q

What murmur is heard in an AVSD?

A

None (big defects balance quickly)
OR
Pansystolic (TR LLSB/MR Apex)

42
Q

What is the importance of Eisenmenger syndrome in AVSD?

A

Double shunt -> happens much faster

43
Q

How do you manage an AVSD?

A

Manage CCF
IE prophylaxis
Closure - surgery/cath lab

44
Q

What murmur is heard in an ASD?

A

Ejection systolic ULSB

45
Q

What changes occur with an ASD?

A

Right heart dilation -> RV dilates

46
Q

How do you manage an ASD?

A

Manage CCF
Watch for arrythmias
Closure - surgery if large/cath lab

47
Q

What are features of an aortic stenosis?

A

Reduced CO -> syncope
PDA dependent if tight
LVH

48
Q

What are features of coarctation?

A

Reduced/absent lower limb pulse, BP, sats
PDA dependent if tight
LVH
Hypertension

49
Q

What murmur is heard in mitral regurgitation?

A

Pansystolic at apex

50
Q

What are features of a pulmonary stenosis?

A

RVH
PDA-dependent if tight
Soft S2

51
Q

What murmur is heard in an aortic stenosis?

A

Ejection systolic at RUSB

52
Q

What murmur is heard in a pulmonary stenosis?

A

Ejection systolic at LUSB

53
Q

What murmur is heard in tricuspid regurgitation?

A

Pansystolic at LLSB

54
Q

What is a sign of tricuspid regurgitation on examination?

A

Pulsatile liver

55
Q

Which syndrome is associated with AVSD?

A

Down’s syndrome

56
Q

What arrhythmia is associated with ASD?

A

Atrial fibrillation

57
Q

Which axis does conduction in neonates favour?

A

Right axis

58
Q

What are the features of a tricuspid atresia?

A

ASD
VSD - PDA if no VSD
Single 1st heart sound
Left axis deviation on ECG

59
Q

What is the differential diagnosis for a left axis deviation on ecg in a congenital defect?

A 
Cyanotic = tricuspid atresia
Acyanotic = AVSD
60
Q

Which cardiac defect causes a “wall to wall heart” on CXR?

A

Ebstein’s anomaly

PA without VSD

61
Q

What murmur is heard in a ToF?

A

EJM at LUSB

You don’t hear the VSD murmur because PS -> high RV pressure -> pressures are balanced -> no flow over the VSD

62
Q

What are the clinical features of hypercyanotic spells?

A 
Acidotic breathing
Hyperinflated
Cyanosis
Limpness
Convulsions
63
Q

How can pulmonary atresia appear on CXR?

A

No VSD = wall to wall heart

VSD = boot shaped heart

64
Q

What kind of congenital heart defect is a ToF?

A

Cyanotic, decr PBF

65
Q

What kind of congenital heart defect is a persistent truncus?

A

Cyanotic incr PBF

66
Q

What kind of congenital heart defect is a pulmonary atresia?

A

Cyanotic, decr PBF

67
Q

What kind of congenital heart defect is an aortic stenosis?

A

Acyanotic, normal PBF

68
Q

What kind of congenital heart defect is a PDA?

A

Acyanotic, incr PBF

69
Q

What kind of congenital heart defect is a TGA?

A

Cyanotic, incr PBF

70
Q

What kind of congenital heart defect is a TAPVD?

A

Cyanotic, incr PBF

71
Q

What kind of congenital heart defect is a pulmonary stenosis?

A

Acyanotic, normal PBF

72
Q

What kind of congenital heart defect is a mitral regurg?

A

Acyanotic, normal PBF

73
Q

What kind of congenital heart defect is a VSD?

A

Acyanotic, incr PBF

74
Q

What kind of congenital heart defect is a tricuspid atresia?

A

Cyanotic, decr PBF

75
Q

What kind of congenital heart defect is a critical pulmonary stenosis?

A

Cyanotic, decr PBF

76
Q

What kind of congenital heart defect is a tricuspid regurg?

A

Acyanotic, normal PBF

77
Q

What kind of congenital heart defect is a PDA?

A

Acyanotic, incr PBF

78
Q

What kind of congenital heart defect is a HLHS?

A

Cyanotic, incr PBF

79
Q

What kind of congenital heart defect is an Ebstein’s anomaly?

A

Cyanotic, decr PBF

80
Q

What kind of congenital heart defect is a coarctation?

A

Acyanotic, normal PBF

81
Q

What kind of congenital heart defect is an AVSD?

A

Acyanotic, incr PBF

82
Q

What complication do children with persistent truncus arteriosus present with very quickly?

A

Pulmonary hypertension

83
Q

What are the 3 locations in TAPVD?

A

Supracardiac - SVC
Infracardiac - ICV
Cardiac - RA

84
Q

Which murmurs are heard in a TAPVD?

A 
ESM LUSB (PS)
MDM LLSB (TS)
85
Q

What is the dose of prostin?

A

0.05ug/kg/min - 0.1ug/kg/min

86
Q

What are common side effects of prostin?

A 
Fever
Apnoea
Wheezing
Diarrhoea
Elevated neutrophils
Transient feeding difficulties
Abdominal distension
Elevated K+ level
Bartter syndrome
87
Q

What types of polycythaemia are there?

A

Absolute (incr RBC mass)
- primary (rubra vera)
- secondary
Relative (decr plasma volume)

88
Q

Name causes of secondary polycythaemia

A

CCHD
High altitudes
Tumours
Smoking

89
Q

What are clinical features of polycythaemia?

A 
Purple appearance (mucosa, lips, limbs)
Dyspnoea
Headache
Hypertension
Thromboembolism
Epistaxis
90
Q

At what Hct is there an exponential increase in blood viscosity?

A

Hct >65

91
Q

Why do children with polycythaemia get thromboembolism and epistaxis?

A

Thromboembolism - hypercoagulable

Epistaxis - platelet clumping

92
Q

What are complications of polycythaemia?

A

Stroke (hypercoaguable, microcytic RBC)

93
Q

How do you manage unoperated CCHD to decrease polycythaemia risk?

A

Iron + folic acid supplementation

Partial exchange transfusion

MBChB IV Paediatrics (81 decks)

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