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1
Q

What are patients with VSD most at risk of

A

Endocarditis

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2
Q

What is transposition of the great arteries

A

Transposition of the great arteries (TGA) is a form of cyanotic congenital heart disease. It is caused by the failure of the aorticopulmonary septum to spiral during septation. Pulmonary artery is supplied by the LV and vice versa. Incompatible with life except for with PDA

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3
Q

How does transposition of the great arteries present

A

Cyanosis
Tachypnoea

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4
Q

What are prominent right ventricular impulse and loud single S2 seen in

A

transposition of the great arteries

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5
Q

Management of transposition of the great arteries

A

Maintain patent ductus arteriosus with prostaglandins
Balloon atrial septoplasty
Arterial switch surgical correction is definitive

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6
Q

What are the 4 features of tetralogy of fallot

A

Pulmonary stenosis
Leads to RV hypertrophy and right sided outflow obstructions
VSD which leads to eisenmenger syndrome as RV hypertrophy increases pressure to greater than LV
Overriding aorta

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7
Q

How dos tetralogy of fallot present

A

Typically around 2 months unlike other cyanotic heart conditions which present at birth
Tet spells where child is feeding/crying causes spasms of infundibular septum causing cyanosis and tachypnoea may even LOC
Squatting improves symptoms

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8
Q

What are the 3 cyanotic heart disease

A

Tetralogy of fallot
Tricuspid atresia
Transposition of great arteries

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9
Q

Pathophysiology of tricuspid atresia

A

Valve malformed or does not form at all
Incompatible with life unless ASD and VSD

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10
Q

What has egg on side X ray appearance

A

transposition of the great arteries

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11
Q

What determines the severity of tetralogy of fallot

A

Extent of pulmonary stenosis

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12
Q

Murmur in tetralogy of fallot

A

ESM louder on inspiration heard at left sternal edge- VSD tends to not present with murmur

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13
Q

CXR finding of tetralogy of fallot
ECG finding

A

CXR- boot shape
ECG- RVH

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14
Q

Management of tetralogy of fallot

A

Acutely prostaglandins to maintain PDA
If severe cyanotic episodes (over 15mins) where lose consciousness
- sutgical intervention where blood goes to lungs- BT shunt or RV balloon dilatation
- can use propanolol
Corrective surgery from 4 months onwards

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15
Q

Management of tricuspid atresia

A

Balock taussig shunt
Corrective surgery but is very difficult as only one functioning ventricle
Glenn operation then fontan
GF

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16
Q

Murmur in tricuspid atresia

A

ESM heard loudest at lower left sternal edge

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17
Q

What cardiac anomaly is downs associated with

A

ASVD

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18
Q

How is ASVD normally picked up

A

Routine echo for downs
Week 2-3 get cyanosis if not

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19
Q

Managment of ASVD

A

Treat heart failure and then corrective surgery at 3 months

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20
Q

What is point of balock taussig shunt

A

In cyanotic heart conditions like TOF and tricuspid atresia is lack of blood flow to lungs so this provides them with supply to oxygenate the blood

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21
Q

Murmur in ASD

A

Ejection systolic left sternal edge
Fixed splitting of S2

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22
Q

What are 2 types of ASD

A

Most common -Secundum where foramen ovale does not close
Primum- defect in AV septum

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23
Q

Management of the 2 types of ASD

A

Observation as will close spontaneously often
Manage if
- ratio of pulmonary to systemic blood flow ratio over 1.5
- right ventricular dilation
- symptomatic
Secundum- catheterisation and insertion of occlusive devise
Primum- corrective surgery

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24
Q

Presentation of ASD

A

Typically asymptomatic but can get recurrent infection, SOB

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25
How are VSDs classified
Small (<3mm) Large (>3mm)
26
How do small versus large VSD present
Small- SOB with normal saturations, abnormal feeding, tired Large- HF, recurrent infections, hepatomegaly
27
Management of small versus large VSD
Small - will correct naturally but monitor with echos Large- need to prevent eisenmenger syndrome - CDC - increase calories, diuretics(furosemide) and catopril - corrective surgery at 3-6 mths
28
Which type of VSD is endocarditis more common
Small
29
If have cyanosed baby what test determines if cyanotic HD
Hyperoxia test- give 10L for 10mins Measure oxygen in blood gas of right arm, if stays below 15pa then cyanotic HD if have excluded lung disease and persistent pulomanry hypertension of the newborn
30
Complications of VSD
aortic regurgitation infective endocarditis Eisenmenger's complex right heart failure
31
Syndromes associated with VSD
Downs Edward's syndrome Patau syndrome cri-du-chat syndrome
32
What causes closure of ductus arteriosus
High oxygen
33
Risk factors for patent ductus arteriosus
Pre-term Low oxygen ( born at high altitude, lung problems etc) Maternal rubella infection during 1st trimester
34
Management of patent ductus arteriosus
Should close by 1 month - indomethacin post natally - surgical ligation at 1 year if indomethacin fails to close it
35
Signs and symptoms of PDA
Symptoms - Often asymptomatic as should shut by 1 month post partum - SOB, bradycardia and needs O2 Signs - machine like murmur over ULSE - heaving apex beat - wide pulse pressure - bounding pulse
36
What is the name of PDA murmur
Gibsons
37
What is eisenmenger syndrome and what causes it
Eisenmenger occurs in initial L->R shunts where the increased blood flow to pulmonary circulation results in vascular remodelling in the lungs. As such this causes pulmonary hypertensions which causes hypertrophy of RV- this then increases afterload of pulmonary circulation and can become greater than that of left side which reverses shunt. Then get cyanotic heart disease
38
Management of eisenmenger syndrome
Heart and lung transplant
39
How to distinguish innocent murmurs from pathological
InnoSent- 5 s's Asmypomatic Silent Left sternal edge Soft blowing Systolic only Often occur in illness like infections when younger so check for fever etc Also varies with posture
40
Symptoms of HF in infants
Breathless Sweating Feeding Recurrent chest infections
41
Signs of HF
Poor weight gain Tachypnoea Tachycardia Heart murmur Enlarged heart Hepatomegaly Cool peripheries
42
Causes of HF in neonates
Typically from obstructed systemic circulation - hypoplastic left heart syndrome - critical aortic valve stenosis - severe aortic coarctation
43
Causes of HF in infants
VSD AVSD Large PDA
44
Causes of HF in older children
Eisenmenger syndrome Rheumatic fever Cardiomyopathy
45
What causes cyanosis in an infant
Cyanotic heart disease Respiratory disorders- surfactant deficiency, meconium aspiration, pulmonary hypoplasia Peristent pulmonary hypertension of the newborn Infection
46
Causes of outflow obstruction
Pulmonary stenosis Aortic stenosis Aortic coarctation- most common
47
How does aortic stenosis present
Moderate- SAD (syncope, angina, dyspnoea) Severe- severe heart failure with shock in neonates
48
Signs of aortic stenosis
Carotid thrill ESM radiating to the neck Slow rising pulse
49
Management of aortic stenosis
Balloon valvulotomy If needed transcatheter aortic valve replacement
50
What are the 2 types of aortic coarctation
Infantile- proximal to DA Adult- distal to DA
51
Presentation of aortic coarcation
Asymptomatic could be Infants- collapse on second day of life onwards when DA closes
52
Examination findings of aortic coarctation
High blood pressure in arms but low in legs Systolic murmur heard loudest in the back
53
Management of aortic coarctation in infants
ABC and prostaglandin to open DA Balloon repair or surgery
54
What is hypoplastic left syndrome
Underdevelopment of the whole left side of heart- present the sickedt of all congenital heart conditions
55
Difference between hypoplastic left heart syndrome and coarctation of aorta
Absent pulses in all peripheries- HLHD Absent pulses in legs- CA
56
Management of PA
Prostaglandin ABC Norword procedure OR BK shunt ASAP
57
What are 2 innocent murmurs
Venous hum- Due to the turbulent blood flow in the great veins returning to the heart. Heard as a continuous blowing noise heard just below the clavicles Stills murmur- Low-pitched sound heard at the lower left sternal edge
58
What is most common childhood arrythmia
SVT Sinus arrythmia common in children as detectable cyclical increase by 30bpm with inspiration
59
How does SVT present in utero
HF or hydrops fetalis leads to reduced CO and pulmonary oedema
60
How does SVT show on ECG
Narrow complex tachycardia
61
Wolf parkinson white on ECG
Delta wave
62
Management guidelines for SVT
Circulation and resp support Vagal manoevers IV adenosine DC cardioversion Once sinus rythm restored maintenance with sotalol and flecainide
63
What is given for maintenance after SVT
Flecainide or sotalol
64
What is done if children relapse with SVT
Percutaneous radiofrequency ablation or cryoablation
65
Cause of rheumatic fever
Group A strep infection Scarlet fever Typically occurs 2-6 weeks after the pharyngitis
66
Signs and symptoms of rheumatic fever
J- polyarthritis O- carditis (any) N- subcut nodules E- erythema marginatum S- syndenhams chorea
67
What diagnoses rheumatic fever
Jones criteria- Strep throat and 2 majors or 1 major and 2 minors Major J- joints O- carditis N- subcut nodules E- erythema marginatum S- sydenhams chorea Minor F- fever R- raised CRP or ESR A- arthralgia P- prolonged PR P- previous RF
68
How can history of strep throat be shown
Group A strep on throat culture Antistreptolysin titre
69
Acute management of rheumatic fever Long term too
High dose aspirin as suppresses inflam in joints and heart Amoxicillin if presence of current infection Steroids if fever and inflammation does NOT respond rapidly Valve replacement if needed
70
Prophylaxis for rheumatic fever
Monthly injections of benzathine penicillin for 10 years after or until age of 21 Lifelong if severe valve damage
71
Management of hyperyanotic attacks in tetralogy of fallot
Knee to chest position Administer O2 IV morphine, adrenaline and propanolol Potentially sodium bicarbonate
72
What do if at GP examine child and are absent femoral pulses
Same day consultation with paeds
73
What causes ebsteins anomaly
Lithium use during pregnancy
74
Pathophysiology of ebsteins anomaly
Posterior leaflets of tricuspid valve are displaced anteriorly causing stenosis and regurgutation
75
Murmur in ebsteins anomaly
Stenosis- mid-diastolic Regurg- pansystolic Presents as murmur during diastole and systole
76
CXR finding of ebsteins anomaly
Right atrial enlargement
77
CXR finding of ebsteins anomaly
Right atrial enlargement
78
Resp infection and then cardiac issues with cardiac enlargement
Viral myocarditis-> dilated cardiomyopathy
79
What typically presents with feeling faint and chest pain when exercising
Aortic stenosis
80
What syndrome is aortic stenosis associated with
Williams
81
How are hypercyanotic episodes managed in cyanotic babies with TOF
IV propanolol Pain relief Fluids IV bicarbonate
82
Systolic murmur which radiates over the praecordium
VSD
83
Best way to assess venous congestion from RHF in a child
Hepatosplenmegaly as neck is short to assess neck veins
84
With congenital heart defects how do children appear during feeds
Breathless and sweaty
85
Which heart diseases worsen on closing of ductus arteriosus
Pulmonary stenosis Tricuspid atresia TOF Transposition of the great arteries
86
On top of prostaglandins what else should be given to a child awaiting an echo
Abx to cover for sepsis
87
What is most common presentation of rheumatic fever
Polyarthritis
88
Murmur in VSD
Holosystolic (pansystolic) murmur heard over LLSE
89
Murmurs if ULSE
Pulmonary stenosis PDA ASD
90
Murmur in pulmonary stenosis
Starts at beginning of systole Radiates to back Thrill Loudest at ULSE
91
If patient presents unwell with VSD what is first management
NG tube to promote calory intake
92
Cyanosis with left axis deviation and apical impulse
Tricuspid atresia
93
Which cyanotic condition at birth can present with no murmur
Transposition of great vessels as valves intact
94
What is eisenmenger most associated with
VSD
95
Cardiac anomaly associated with DiGeorge
Interruption of aortic arch
96
What can exacerbate innocent murmurs
Febrile illness
97
Complications of aortic coarctation affecting brain vessels
Cerebral aneurysms due to increased pressure pre coarctation due to activation of RAAS
98
What can cause delayed presentation of transposition of the great arteries
If VSD present
99
Systolic murmur heard loudest in the back
Aortic coarctation
100
Maternal risk factor for transposition of the great arteries
Uncontrolled DM
101
What is target sign
concentric alternating echogenic and hypoechoic bands

Paeds (17 decks)

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