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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
What are the features of pulmonary atresia?
PDA ASD VSD
26
How do you manage all PDA-dependent lesions?
PGE1 IVI PGE2 orally Treat metabolic acidosis Refer
27
At what age are cyanotic incr PBF lesions common?
<3-6mo
28
What are the features of transposition of the great vessels?
PFO, ASD PDA VSD
29
What is an intrauterine risk factor for transposition of the great vessels?
Infant to diabetic mother
30
What are the features of TAPVD?
ASD PDA RVH
31
What syndromes are associated with HLHS?
Trisomy 13 Trisomy 18 Jacobsen syndrome Turner syndrome
32
What are the features of HLHS?
ASD | PDA
33
Name complications of cyanosis
Polycythaemia CVS events Clotting risk Bleeding risk
34
What is the most common congenital heart defect?
VSD
35
What murmur is caused by a VSD?
Pansystolic at LLSB -> pressure LV > RV in systole, no difference in diastole
36
What changes occur with a VSD?
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)
37
How do you manage a VSD?
Manage CCF IE prophylaxis Closure - surgery/cath lab
38
What murmur is caused by a PDA?
Continuous murmur at ULSB -> pressure during both systole and diastole AO > PA
39
What is the importance of Eisenmenger syndrome in PDA?
Cyanotic only in feet! | Aortic arch perfuses upper body
40
How do you manage a PDA?
Indomethacin/aspirin Manage CCF IE prophylaxis Closure - surgery/cath lab
41
What murmur is heard in an AVSD?
None (big defects balance quickly) OR Pansystolic (TR LLSB/MR Apex)
42
What is the importance of Eisenmenger syndrome in AVSD?
Double shunt -> happens much faster
43
How do you manage an AVSD?
Manage CCF IE prophylaxis Closure - surgery/cath lab
44
What murmur is heard in an ASD?
Ejection systolic ULSB
45
What changes occur with an ASD?
Right heart dilation -> RV dilates
46
How do you manage an ASD?
Manage CCF Watch for arrythmias Closure - surgery if large/cath lab
47
What are features of an aortic stenosis?
Reduced CO -> syncope PDA dependent if tight LVH
48
What are features of coarctation?
Reduced/absent lower limb pulse, BP, sats PDA dependent if tight LVH Hypertension
49
What murmur is heard in mitral regurgitation?
Pansystolic at apex
50
What are features of a pulmonary stenosis?
RVH PDA-dependent if tight Soft S2
51
What murmur is heard in an aortic stenosis?
Ejection systolic at RUSB
52
What murmur is heard in a pulmonary stenosis?
Ejection systolic at LUSB
53
What murmur is heard in tricuspid regurgitation?
Pansystolic at LLSB
54
What is a sign of tricuspid regurgitation on examination?
Pulsatile liver
55
Which syndrome is associated with AVSD?
Down's syndrome
56
What arrhythmia is associated with ASD?
Atrial fibrillation
57
Which axis does conduction in neonates favour?
Right axis
58
What are the features of a tricuspid atresia?
ASD VSD - PDA if no VSD Single 1st heart sound Left axis deviation on ECG
59
What is the differential diagnosis for a left axis deviation on ecg in a congenital defect?
``` Cyanotic = tricuspid atresia Acyanotic = AVSD ```
60
Which cardiac defect causes a "wall to wall heart" on CXR?
Ebstein's anomaly | PA without VSD
61
What murmur is heard in a ToF?
EJM at LUSB | You don't hear the VSD murmur because PS -> high RV pressure -> pressures are balanced -> no flow over the VSD
62
What are the clinical features of hypercyanotic spells?
``` Acidotic breathing Hyperinflated Cyanosis Limpness Convulsions ```
63
How can pulmonary atresia appear on CXR?
No VSD = wall to wall heart | VSD = boot shaped heart
64
What kind of congenital heart defect is a ToF?
Cyanotic, decr PBF
65
What kind of congenital heart defect is a persistent truncus?
Cyanotic incr PBF
66
What kind of congenital heart defect is a pulmonary atresia?
Cyanotic, decr PBF
67
What kind of congenital heart defect is an aortic stenosis?
Acyanotic, normal PBF
68
What kind of congenital heart defect is a PDA?
Acyanotic, incr PBF
69
What kind of congenital heart defect is a TGA?
Cyanotic, incr PBF
70
What kind of congenital heart defect is a TAPVD?
Cyanotic, incr PBF
71
What kind of congenital heart defect is a pulmonary stenosis?
Acyanotic, normal PBF
72
What kind of congenital heart defect is a mitral regurg?
Acyanotic, normal PBF
73
What kind of congenital heart defect is a VSD?
Acyanotic, incr PBF
74
What kind of congenital heart defect is a tricuspid atresia?
Cyanotic, decr PBF
75
What kind of congenital heart defect is a critical pulmonary stenosis?
Cyanotic, decr PBF
76
What kind of congenital heart defect is a tricuspid regurg?
Acyanotic, normal PBF
77
What kind of congenital heart defect is a PDA?
Acyanotic, incr PBF
78
What kind of congenital heart defect is a HLHS?
Cyanotic, incr PBF
79
What kind of congenital heart defect is an Ebstein's anomaly?
Cyanotic, decr PBF
80
What kind of congenital heart defect is a coarctation?
Acyanotic, normal PBF
81
What kind of congenital heart defect is an AVSD?
Acyanotic, incr PBF
82
What complication do children with persistent truncus arteriosus present with very quickly?
Pulmonary hypertension
83
What are the 3 locations in TAPVD?
Supracardiac - SVC Infracardiac - ICV Cardiac - RA
84
Which murmurs are heard in a TAPVD?
``` ESM LUSB (PS) MDM LLSB (TS) ```
85
What is the dose of prostin?
0.05ug/kg/min - 0.1ug/kg/min
86
What are common side effects of prostin?
``` Fever Apnoea Wheezing Diarrhoea Elevated neutrophils Transient feeding difficulties Abdominal distension Elevated K+ level Bartter syndrome ```
87
What types of polycythaemia are there?
Absolute (incr RBC mass) - primary (rubra vera) - secondary Relative (decr plasma volume)
88
Name causes of secondary polycythaemia
CCHD High altitudes Tumours Smoking
89
What are clinical features of polycythaemia?
``` Purple appearance (mucosa, lips, limbs) Dyspnoea Headache Hypertension Thromboembolism Epistaxis ```
90
At what Hct is there an exponential increase in blood viscosity?
Hct >65
91
Why do children with polycythaemia get thromboembolism and epistaxis?
Thromboembolism - hypercoagulable | Epistaxis - platelet clumping
92
What are complications of polycythaemia?
Stroke (hypercoaguable, microcytic RBC)
93
How do you manage unoperated CCHD to decrease polycythaemia risk?
Iron + folic acid supplementation | Partial exchange transfusion

MBChB IV Paediatrics (81 decks)

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