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Paediatric Cardiology
Paediatric cardiology
• Examination of the cardiovascular system
• Acyanotic congenital heart disease: ventricular septal defect, atrial septal defect and patent
ductus arteriosus
• Cyanotic congenital heart disease: Fallot’s tetralogy
• Down’s syndrome and the heart
Extras: post course notes on
• Kawasaki’s disease
• Heart failure
• Rheumatic heart disease
• Co-arctation
• Transposition of the great arteries
• Subacute bacterial endocarditis
Examination of the cardiovascular system
M Radford: with permission
Approach to examination
• Wash hands, introduce yourself and ask
permission
• Look: scars, clubbing, oxygen,
breathlessness, cyanosis, respiratory rate,
activity, JVP
• Feel: pulses, praecordium, liver
• Listen: heart, lung bases
• Finally thank child and parents and wash
hands
Please note
• It is reasonable to perform auscultation first while the child is quiet
• “Babies don’t have a neck” so check for enlargement of liver instead of assessing the JVP
Auscultation: question stop
What causes the first and second heart sounds?
Normal heart sounds
lub
dub
ventricular systole
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diastole
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Normal splitting of the second heart sound
• During inspiration, there is an increase in the negative intra-thoracic pressure
• This increases venous return from the body into the right atrium
• And therefore increases the volume of blood to be ejected by the right ventricle
• Causing a slight delay in the closure of the pulmonary valve (P2)
Lub
dub
Lub
Inspiration
de
Expiration
Wide fixed splitting of the second heart sound with an atrial septal defect (ASD)
Lub
splat
Lub
Inspiration
splat
Expiration
Please note
• You are not expected to diagnose wide fixed splitting of the second sound!
• It is a subtle sign and many paediatricians have never heard it
• This is why it is much harder to diagnose ASDs compared with VSDs
• But it is still useful to be aware of this sign as it is often asked about in exams
Pansystolic murmur: “burrr”
no gap between murmur and HS2
lub
dub
Pansystolic murmur
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diastole
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Ejection systolic murmur: “burr de”
audible gap between
mumur and HS2
lub
dub
diastole
ventricular systole
Murmurs: above or below the nipple line?
Radiates to neck
Radiates to back
Aortic stenosis
Pulmonary stenosis
PDA
machinery
murmur
Ejection systolic above
VSD
(MR)
Pansystolic below
The Nipple Line
Continuous murmur: BurrrDurrr
lub
dub
diastole
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Four useful sounds
• ASD (Lub splat)
• Pansystolic murmur (Burrr)
• Ejection systolic murmur (Burr de)
• Machinery murmur (BurrrDurrr)
Innocent murmurs
• Heard at some time in about 25% of children
• Soft ejection systolic murmur at left sternal edge
• May occasionally sound harsh
• No radiation to carotids and no thrills
• No symptoms
• Often heard with fever (tachycardia with increased cardiac output)
The 7 S’s
• Short
• Soft
• Systolic
• S1 & S2 normal
• Standing and sitting variation
• Symptomless and
• Special tests normal (ECG, CXR, Echo)
Fetal Circulation
Placenta
Umbilicus
Umbilical vein
Joins inferior vena cava
Ductus venosus
Two umbilical arteries
From internal iliac arteries
SVC
SVC
Pulmonary veins
IVC
Pulmonary veins
IVC
Foramen ovale
Right
atrium
Right
ventricle
Left
ventricle
Ductus
arteriosus
Pulmonary artery
Crosses
Foramen ovale
Left
atrium
Oxygenated blood
from umbilical vein
(via ductus venosus
and IVC)
Aorta
Pulmonary artery
Ductus
arteriosus
Aorta
Fetal circulation
• In utero, oxygenated blood is provided by placenta
• Fetal lung is bypassed by most circulating blood
• High pulmonary blood pressure means blood follows alternate path via:
• Foramen ovale (from RA to LA)
• Ductus Arteriosus (from PA to aorta)
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Normal transition
At birth first breath triggered by
• Hypoxia secondary to cord clamping
• Stimulation
SVC
Pulmonary veins
IVC
RA
Air drawn into lungs
• Oxygen is powerful pulmonary vasodilator
• Large drop in pulmonary blood pressure
RV
LA
LV
Blood redirected through lungs
Ductus closes within a day or two
Pulmonary artery
Aorta
Congenital Heart Disease (CHD)
• Commonest congenital malformation
• Approx 1% of live births
• 10% of stillbirths have cardiac lesions
• VSD and PDA are the commonest abnormalities
Classification of CHD
• Acyanotic: ASD, VSD, PDA, pulmonary stenosis, aortic stenosis, co-arctation
• Cyanotic: Tetralogy of Fallot, transposition of the great arteries
J Peterson: with permission
Central cyanosis
Blueness occurs when there is more than 5g/dl
de-oxygenated haemoglobin
If not anaemic, this will usually occur with an
oxygen saturation of 85% or less
Acyanotic congenital heart disease
Three left-to-right shunts
• VSD
• ASD
• PDA
Ventricular Septal Defect (VSD)
• Common: 30% of CHD
• Sometimes diagnosed antenatally if a good
four chamber view is obtained on ultrasound
• Variable size
• Loudness of murmur inversely related to size
of shunt
SVC
Pulmonary veins
IVC
VSD
Buzz words
If large, a VSD may cause
“a left to right shunt at ventricular level”
Pulmonary artery
Aorta
Student report
“My case was examination of a 3 year old with a VSD”
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Small VSD
• High velocity jet with loud “blowing” or “rasping” pansystolic murmur
• May have a thrill, but no significant left to right shunt
• Increased risk of endocarditis
• Often close spontaneously before age 5
• Differential diagnosis: other pansystolic murmurs eg mitral and tricuspid regurgitation
Large VSD
• Typically presents with heart failure at 4-6 weeks
• Breathless and sweaty on feeding or crying
• May also be a cause of faltering growth or
• Recurrent chest infections
Large VSD: on examination
• Left parasternal heave (due to right ventricular hypertrophy)
• Quiet or absent pansystolic murmur
• Pulmonary ejection murmur
• In other words, signs of pulmonary hypertension
Typical case: small VSD
• Blowing pansystolic murmur at lower left sternal edge
• With an associated thrill
• But no right ventricular heave or signs of heart failure
VSD investigations
• ECG- right ventricular hypertrophy (dominant R wave in V1)
• CXR- cardiomegaly, prominent pulmonary artery and plethoric lung fields
• Echo- shows size of lesion and doppler flow may indicate size of shunt
VSD treatment
• None if small
• Antibiotic prophylaxis controversial
• NICE (2008) do not recommend it, but many paediatric cardiologists do!
• Diuretics and ACEI for heart failure
• Repair if large defect with risk of pulmonary hypertension
• No need for antibiotic prophylaxis once repaired and “endothelialised”
Atrial septal defect
• May be asymptomatic
• Recurrent chest infections or heart failure
• Arrhythmias common in 30s and 40s (SVT and AF)
• Flow across the defect itself does not create a murmur (as low pressures in atria)
• Isolated ASD: low risk of endocarditis and antibiotic prophylaxis not needed (NICE 2008)
Buzz words
If large, an ASD may cause
“a left to right shunt at atrial level”
SVC
Pulmonary veins
IVC
ASD
Pulmonary artery
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Aorta
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Wide fixed splitting of the second heart sound
Delayed P2
Lub
splat
Lub
Inspiration
splat
Expiration
ASD treatment
• Trans-catheter closure (via femoral vein and IVC to right atrium) or
• Open heart surgery with patch repair
• Before 5th birthday
Patent ductus arteriosus (PDA)
• 12% of CHD cases
• Second commonest after VSD
• Commoner in premature babies (kept open by hypoxia)
• Normally closes within a day or two of birth
• If persists, left to right shunt occurs as right sided pressures fall with lung expansion
SVC
Pulmonary veins
IVC
Patent ductus
arteriosus
Pulmonary artery
Aorta
PDA: on examination
• Collapsing pulses: the shunting leads to extra blood flow through the lungs and hence extra
blood returning to the left of the heart (volume overload)
• Extra blood ejected from LV causes high systolic pulse pressure
• Rapid “run-off” through the ductus leads to low diastolic pressure
• Auscultation: continuous “machinery” murmur
• Loudest below left clavicle and radiates to back
BurrrrDurrr
lub
dub
diastole
PDA: treatments
• Prostaglandin synthetase inhibitor (eg indometacin infusion)
• Transcatheter occlusion
• Surgical ligation
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Cyanotic CHD
SVC
www.doctortipster.com
Fallot’s tetralogy
P
pulmonary stenosis causing
R
right ventricular hypertrophy
O
over-riding aorta; R to L shunt across
V
VSD
E
ejection systolic murmur- pulmonary
Pulmonary veins
Aorta displaced
to the right
IVC
LA
Pulmonary
stenosis
VSD
RA
Right to left shunt
through VSD due to
pulmonary stenosis
LV
RV
Pulmonary stenosis
Aorta
RV hypertrophy
“Unfolded”
“Folded”
On examination
• Clubbing and cyanosis
• Right ventricular hypertrophy
• Ejection systolic murmur
Buzz words
“Right ventricular outflow tract obstruction”
“Infundibular pulmonary stenosis”
Ie not just the valve that is narrow, the outflow
tract just below the valve is affected
Patent ductus in Fallot’s
• Cyanotic spells often become apparent
around day 2 or day 3 when the ductus
closes
• In 1944 Dr Helen Taussig noted that babies
with a persistent patent ductus lived longer
than those without
• She thought an operation to create a similar
shunt into the pulmonary artery would help
• She persuaded Alfred Blalock to perform
the first shunt operation
Poem
The aorta’s displaced to the right
Squashing the PA so tight
Where can the blood go?
There’s right to left blood flow
Across the septum- that’s right
(Ventricular hypertrophy for you)
SVC
Pulmonary veins
IVC
Right to left shunt
through VSD due to
pulmonary stenosis
Pulmonary stenosis
Patent ductus
bypasses
obstruction
Surgery
• Two stage procedure
• Shunt operation to increase pulmonary flow in order to help develop the pulmonary arteries,
which have been “starved” of blood flow by the stenosis; improves oxygenation
• Followed by definitive correction
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Down’s syndrome and the heart
Down’s syndrome: face
R
Round face
O
Occipital flattening (& nasal flattening)
S
Speckled iris (Brushfield spots)
E
Epicanthic folds
O
Open mouth with protruding tongue
L
Low set ears
A
Almond (oval) upward slanting eyes
Hands: single transverse
palmar crease, short
fingers, curved little finger
(clinodactyly)
Strabismus (squint)
Brushfield
spots
on iris
Epicanthic fold
Feet: sandal gap between
big toe and other digits
Flat bridge of nose
Open mouth
Protruding tongue
Duodenal atresia
“Double bubble”
Down’s syndrome
• Hands: single transverse palmar crease, short fingers, curved little finger (clinodactyly)
• Feet: sandal gap between big toe and other digits
• Increased risk of: duodenal atresia, squint, hypothyroidism, leukaemia, Hirschprung’s
• 40-50% have a cardiac abnormality: all should have an echo at time of diagnosis
Down’s syndrome associated with
• ASD
• VSD
• Atrioventricular canal defect (low ASD and high VSD)
• Mitral and tricuspid valve regurgitation
Buzz words
• “Endocardial cushion defect”
• Leads to “failure of septation” of the heart
• “AVSD”
LA
v
RA
RV
LV
Endocardial Cushions divide the heart into
left and right; and into atria and ventricles
Student report
“Examine this child’s cardiovascular system. He was aged 2, with a systolic murmur. I was
asked about Eisenmenger’s syndrome.”
Question stop
Eisenmenger’s syndrome refers to the process of shunt reversal- why does this happen?
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Shunt reversal
• Large shunt leads to pulmonary artery hypertension
• As this develops, the degree of left to right shunting gets less which may lead to initial
improvement in heart failure symptoms
• Finally, the right sided pressures are so high that right to left shunting occurs causing
cyanosis
• This is called the “Eisenmenger syndrome”, causing acquired cyanotic heart disease
• Only treatment option at this stage is heart-lung transplantation
SVC
Pulmonary veins
IVC
Reversed shunt
Right ventricular
hypertrophy
Aorta
Pulmonary artery
hypertension
Post Course Notes
Post course notes on
• Kawasaki’s disease
• Heart failure
• Rheumatic heart disease
• Co-arctation
• Transposition of the great arteries
• Subacute bacterial endocarditis
• Understanding splitting of the second sound in ASD
• Making sense of Fallot’s
• Prostaglandins and the ductus
Case history
This 3 year old has had a prolonged fever. She has developed a rash on her hands and feet
which is now starting to peel. What is the likely diagnosis? What cardiac complication may
occur?
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