PAEDIATRICS THEORY -2020

Cardiovascular
System
Foetal Circulation
Congenital heart diseases
A. Acyanotic heart diseases

B. Cyanotic heart diseases

Acquired heart diseases

1. Rheumatic fever
2. Infective endocarditis
3. Kawasaki’s disease
4. Myocarditis
5. Cardiac arrhythmias

Dr. K.N.H. Thalagahage

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 Foetal Circulations
In Intrauterine life , It is the placenta which involves in gas exchange , not the lungs . Therefore
fetus does not have to sent lot of blood to the lungs . Idea of having a foetal circulation is to
minimize blood sending

In the fetus: the left atrial pressure is low, as relatively little blood returns from the lungs
because its lungs are not yet functioning. Also the resistance in the pulmonary vessels is high.

But the pressure in the right atrium is higher than in the left, as it receives all the venous blood
coming from systemic circulation + the blood coming from the placenta.

So due to this pressure difference some of this blood in the right atrium flows into the left
atrium via the foramen ovale; then from the left atrium into the left ventricle; which then
pumps this blood to the upper body via the aorta.

The blood that is left in the right atrium flows into the right ventricle and is then pumped into
the pulmonary artery.

A connection called the ductus arteriosus exists between the pulmonary artery and the
descending aorta, and most of the blood (90 % ) in the pulmonary arteries flow into the aorta
thus bypassing the lungs.

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 Neonatal Circulation

At birth: with the first breaths, lungs expand and the resistance to pulmonary blood flow falls
and the volume of blood flowing through the lungs increases.

This results in increased blood flow to the left atrium and therefore a rise in left atrial pressure.
Meanwhile, the volume of blood returning to the right atrium falls as the placenta is now not a
part of the baby’s circulation.
This change in pressure difference causes the foramen ovale and the ductus arteriosus to close
up.

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 Congenital Heart Disease
 Congenital heart disease is the most common group of structural malformations in
children.
 8 in 1000 live-born infants have significant cardiac malformations.
 May be associated with non cardiac abnormalities.

Ex; VACTERL

 Are increasingly identified on antenatal ultrasound screening

Aetiology:

Cardiac abnormalities
I. Maternal disorders
 Rubella infection Pulmonary stenosis,
patent ductus arteriosus (PDA)
 Systemic lupus erythematosus (SLE) Complete heart block
 Diabetes mellitus Overall incidence of cardiac abnormalities
increases
II. Maternal drugs
 Warfarin therapy Pulmonary stenosis,
PDA
 Fetal alchohol syndrome ASD,VSD
Tetrology of fallot
III. Chromosomal abnormalities
 Down syndrome (trisomy 21) Atrioventricular septal defect,
VSD
 Edwards syndrome (trisomy 18) Complex heart disease
 Patau syndrome (trisomy 13) Complex heart disease
 Turner syndrome Coartation of Aorta,
Aortic stenosis
 Noonan syndrome Pulmonary stenosis,
Hypertrophic cardiomyopathy.
 Marfan syndrome Aortic regurgitation,
Mitral regurgitation
 Holt-Oram syndrome ASD

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 Classification of congenital heart disease :
Acyanotic heart diseases Cyanotic heart disease

 Ventricular septal defect  Tetrology of fallot (TOF) –

(VSD) – 30% 5%
 Patent Ductus Arteriosus  Transposition of great
– 12% vessels – 5%
 Atrial septal defect – 7%  Tricuspid atresia
 Pulmonary stenosis  Atrioventricular septal
 Coarctation of Aorta defect
 Aortic stenosis  TAPVD
 Truncus arteriosus etc.

Presentation of congenital heart disease

The commonest S/S seen in a child with a heart disease are:

 Heart murmurs ,Cyanosis ,Features of Heart failure ,Failure to thrive ,Shock

1.Heart murmurs:

The most common presentation of a congenital heart disease is a murmur, but it is important to
remember that not all murmurs in children are pathological. Some children may have an
“innocent murmur” or a “venous hum”.

Murmur Common causes

Ejection systolic murmur

Pansystolic murmur

Continuous murmur

Diastolic Murmurs

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Innocent murmurs (are ejection systolic murmurs)

Hallmarks of an innocent ejection murmur are (all have an ‘S’, ‘innoSent’):

• aSymptomatic patient
• Soft blowing murmur
• Systolic murmur only, not diastolic
• left Sternal edge.
Also
• Normal heart sounds with no added sounds (vs fixed split which occurs with the murmur of
ASD)
• No parasternal thrill
• No radiation.

During a febrile illness or anaemia, innocent or flow murmurs are often heard because of
increased cardiac output.

Many newborn infants with potential shunts have neither symptoms nor a murmur at birth, as
the pulmonary vascular resistance is still high. Therefore, conditions such as a ventricular septal
defect or ductus arteriosus may only become apparent at several weeks of age when the
pulmonary vascular resistance falls.

Venous hum

 is a continuous, low-pitched rumble.

 Heard beneath either clavicle.
 Occurs due to turbulent blood flow in the head and neck veins.
 Disappears on lying flat or with compression of jugular veins on the same side (vs the
continuous murmur of PDA which will not disappear)

Notes:

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 Cyanosis
 Peripheral cyanosis (blueness of the hands and feet) may occur when a child is cold
or unwell from any cause ( Ex; Circulatory collapse ) or with polycythaemia
 Central cyanosis, seen on the tongue as a slate blue colour, is associated with a fall in
arterial blood oxygen tension.
 It can only be recognized clinically if the concentration of reduced haemoglobin in the
blood exceeds 5 g/dl, so it is less pronounced if the child is anaemic but common in
polycythemia .
 Persistent cyanosis in an otherwise well infant is nearly always a sign of structural
heart disease.

Cyanosis in a newborn infant with respiratory distress (respiratory rate >60 breaths/min) may
be due to:
 Cardiac disorders – cyanotic congenital heart disease
 Respiratory disorders, e.g. surfactant deficiency, meconium aspiration, pulmonary
hypoplasia, etc.
 Persistent pulmonary hypertension of the newborn (PPHN) – failure of the pulmonary
vascular resistance to fall after birth.
 Infection – septicaemia from group B streptococcus and other organisms.
 Metabolic disease – metabolic acidosis and shock.

 Nitrogen washout (hyperoxia) test helps us to determine if cyanosis is due to a cyanotic

heart disease or not.

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 Heart failure
Symptoms
• Breathlessness (particularly on feeding or exertion)
• Sweating
• Poor feeding
• Recurrent chest infections.
Signs
 Poor weight gain or ‘faltering growth’ Tachypnoea
 Tachycardia Heart murmur, gallop rhythm
 Enlarged heart Hepatomegaly
 Cool peripheries.
 Signs of right heart failure (ankle oedema, sacral oedema and ascites)

Management of heart failure:

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 Acyanotic heart diseases
Ventricular Septal Defect (VSD)

 Ventricular septal defects (VSDs) are common, accounting for 30% of all cases of
congenital heart disease.

 There is a defect anywhere in the ventricular septum, perimembranous (adjacent to the

tricuspid valve) or muscular (completely surrounded by muscle).
 Perimembranous VSD is commoner and generally need surgery.

Pathophysiology

left to right shunt present ; when pulmonary vascular resistance falls after about 4-6 weeks of
birth  blood flows from LV to RV  more blood in pulmonary arteries more blood in lungs
(therefore pulmonary oedema, breathlessness and recurrent infections)  more blood drains
into LV from pulmonary veins  LV overload and with time, heart failure.

If not treated,With time Pulmonary arterial resistance will start increasing to cope up with the
increased flow  pulmonary hypertension (therefore RV failure)  reversal of shunt leading to
Eisenmenger’s syndrome

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 Small VSD Large VSD
Definition
These are smaller than the aortic valve in These defects are the same size or bigger
diameter, perhaps up to 3 mm. than the aortic valve
Clinical features
Symptoms Symptoms
• Asymptomatic. • Heart failure with breathlessness and
Physical signs failure to thrive (faltering growth)
• Loud pansystolic murmur at lower left • Recurrent chest infections.
sternal edge (loud murmur implies smaller
defect) Physical signs
• Quiet pulmonary second sound (P2).( As • Tachypnoea, tachycardia and enlarged
there is no Pul. Hypertension tender liver from heart failure
• Active precordium
• Soft pansystolic murmur (implying large
defect)
• Apical mid-diastolic murmur (from
increased flow across the mitral valve after
the blood has circulated through the lungs)
• Loud pulmonary second sound (P2) – from
raised pulmonary arterial pressure.
Investigations
Chest radiograph- Normal. Chest radiograph
ECG- Normal. • Cardiomegaly
Echocardiography- Demonstrates the precise • Enlarged pulmonary arteries
anatomy of the defect. • Increased pulmonary vascular markings
• Pulmonary oedema.
ECG
• Biventricular hypertrophy by 2 months of
age.
Echocardiography
• Demonstrates the anatomy of the defect,
haemodynamic effects and pulmonary
hypertension (due to high flow).
Management
These lesions will close spontaneously. Drug therapy for heart failure.
While the VSD is present, prevention of Additional calorie input.
bacterial endocarditis is by maintaining good Surgery is usually needed
dental hygiene. • Manage heart failure and failure to thrive
Follow up 6 monthly to detect if it closes or • Prevent permanent lung damage from
weather pulmonary hypertension develops pulmonary hypertension and high blood flow.

Complications:

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 Patent Ductus Arteriosus (PDA)
The ductus arteriosus connects the pulmonary artery to the descending aorta.
In term infants, it normally closes shortly after birth.

In persistent ductus arteriosus it has failed to close by 1 month after the expected date of
delivery due to a defect in the constrictor mechanism of the duct.
The flow of blood across a persistent ductus arteriosus (PDA) is then from the aorta to the
pulmonary artery (i.e. left to right), following the fall in pulmonary vascular resistance after
birth.

Clinical features:
 Symptoms are not common, but when the duct is large there will be increased
pulmonary blood flow with heart failure and pulmonary hypertension. (mechanism
similar to that of a VSD)
 Signs:
o continuous murmur beneath the left clavicle. (The murmur continuesinto
diastole because the pressure in the pulmonary artery is lower than that in the
aorta throughout the cardiac cycle).
o collapsing or bounding pulse (due to increased pulse pressure)

Investigations:

 echocardiography – diagnostic
 CXR and ECG – usually normal, but if the PDA is large, features similar to VSD.

Management:

Indomethezine ( A prostaglandin inhibitor )

Closure with a coil or occlusion device introduced via a cardiac catheter at about 1 year of
age.(to prevent pul HTN, bact. endocarditis etc.)
Occasionally, surgical ligation is required.

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 Atrial Septal Defect (ASD)

Occurs commonly due to failure of closure of foramen ovale after birth.

There are two main types of ASD:

 Secundum ASD (80% of ASDs) – more common
 Primum ASD or Partial atrioventricular septal defect (pAVSD)

Primum ASD Secondum ASD

is a defect of the atrioventricular Is a defect in the centre of the atrial septum
septum and is characterised by: involving the foramen ovale.
• An inter-atrial communication between the
bottom end of the atrial septum and the AV
valves.
• Abnormal AV valves (with a left AV valve
which has three leaflets and tends to leak i.e.
regurgitant valve).
Clinical features – similar in both
Symptoms
 None (commonly)

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  Recurrent chest infections ( In large defects )
 Arrhythmias

Physical signs
 An ejection systolic murmur best heard at the upper left sternal edge – due to
increased flow across the pulmonary valve .
 A fixed and widely split second heart sound – due to the right ventricular stroke
volume being equal in both inspiration and expiration.
 With a primum AVSD, an apical pansystolic murmur from AV valve regurgitation.
Investigations
1. Echocardiography – diagnostic
2. CXR - Cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular
markings.
3. ECG- Secondum ASD may be associated with
In Primum ASD - a ‘superior’ QRS axis. partial right bundle branch block.
right axis deviation due to right ventricular
enlargement.
Management
Surgical correction (open surgery) done at cardiac catheterisation
about 3-5 yrs of age. with insertion of an occlusion device if the
defect is large.

Notes:

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 Coarctation of aorta (COA)

 is the commonest cause of collapse due to left outflow obstruction.

 is due to duct arteriosus tissue encircling the aorta just at the point of insertion of the
duct.
So when the duct closes after birth, the aorta also constricts, causing severe obstruction
to the left ventricular outflow.
 Is an example of duct dependent circulation.

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Clinical features:

 The neonates usually present with acute circulatory collapse at 2 days of age when the
duct closes.
 Physical signs
o A sick baby, with severe heart failure.
o Absent femoral pulses.
o Severe metabolic acidosis.
o

Management:

1) Management is to resuscitate first (ABC).

2) Prostaglandin should be commenced at the earliest opportunity. (to keep the ductus
arteriosus open)
3) Referral for cardiac surgery as soon as possible.

There is another uncommon variant of COA called adult type COA

Adult type COA:

 Is not duct dependent.

 Gradually becomes more severe over many years.
 It is an example of outflow obstruction in a well looking child
(Other examples of this are aortic stenosis and pulmonary stenosis)

Clinical features:

 Asymptomatic
 Systemic hypertension in the right arm
 Ejection systolic murmur at upper sternal edge radiating to back
 Collaterals heard with continuous murmur at the back
 Radio-femoral delay. This is due to blood bypassing the obstruction via collateral vessels
in the chest wall and hence the pulse in the legs is delayed.
 Femoral pulses are weaker
 Discrepancy in BP in Upper and Lower Limbs . ( Upper limb BP > Lower Limb BP )

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Investigations:

 Chest radiograph
 ‘Rib notching’ due to the development of large collateral intercostal arteries
running under the ribs posteriorly to bypass the obstruction.
 ‘3’ sign, with visible notch in the descending aorta at site of the coarctation.
 ECG
 Left ventricular hypertrophy. ( Tall R Waves in V5,V6 Deep S Waves in V1,V2 )

Management:
When the condition becomes severe, as assessed by echocardiography, a stent may be inserted
at cardiac catheter.
Sometimes surgical repair is required.

Aortic Stenosis

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 Pulmonary stenosis

Cyanotic heart diseases

Tetrology of Fallot

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  This is the most common cause of cyanotic congenital heart disease.
 In tetralogy of Fallot, as implied by the name, there are four cardinal anatomical
features:
 A large VSD
 Overriding of the aorta with respect to the ventricular septum.
 Subpulmonary stenosis causing right ventricular outflow tract obstruction.
 Right ventricular hypertrophy as a result.

Uncommon to manifest soon after birth but few months later

Clinical features:

 Hypercyanotic spells and squatting on exercise, developing in late infancy.

It is important to recognise hypercyanotic spells, as they may lead to myocardial
infarction, cerebrovascular accidents and even death if left untreated.
They are characterised by a rapid increase in cyanosis, usually associated with irritability
or inconsolable crying because of severe hypoxia, and breathlessness because of tissue
acidosis.
 Clubbing of the fingers and toes will develop in older children.
 A loud harsh ejection systolic murmur at the left sternal edge (due to pulmonary
stenosis) from day 1 of life.
 Heart failure is rare .

Investigations:

1. CXR -
 Relatively small heart, possibly with an uptilted apex (boot-shaped) due to right
ventricular hypertrophy.
 A pulmonary artery ‘bay’, a concavity on the left heart border where the convex-
shaped main pulmonary artery and right ventricular outflow tract would
normally be seen.
 Decreased pulmonary vascular markings reflecting reduced pulmonary blood
flow.
2. ECG – will show RV hypertrophy if present.
3. Echocardiography – will show the 4 cardinal features.

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Management:

Acute management of hypercyanotic spells:

are usually self-limiting but if they last for > 15mins:

 Keep child in knee chest position.

 Sedation and pain relief with morphine to prevent hyperventilation.
 IV propranolol , which probably works both as a peripheral vasoconstrictor and by
relieving the subpulmonary muscular obstruction.
 intravenous volume administration.
 bicarbonate to correct acidosis.

Long-term management:

Infants who are very cyanosed in the neonatal period require a shunt to increase pulmonary
blood flow.

This is usually done by surgical placement of an artificial tube between the subclavian artery
and the pulmonary artery (amodified Blalock–Taussig shunt), or sometimes by balloon
dilatation of the right ventricular outflow tract.

Definitive surgery at around 6 months of age; involves closing the VSD and relieving right
ventricular outflow tract obstruction.

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 Transposition of Great Vessels (TGV) or (TGA)

The aorta is connected to the right ventricle, and the pulmonary artery is connected to the left
ventricle.  The blue blood is therefore returned to the body and the pink blood is returned
to the lung.

So there are two parallel circulations; unless there is mixing of blood between them at some
point, this condition is incompatible with life.
Fortunately, TGV is usually associated with ASD, VSD or PDA; thus allowing mixing of blood.

Clinical features:
Cyanosis occurring within 2 days after birth.
May have a loud single (no physiological splitting) second heart sound.

Investigations:
CXR – egg on side appearance
Echo – to visualize the defect.

Management:
 In the sick cyanosed neonate, the key is to improve mixing.
 Maintaining the patency of the ductus arteriosus with a prostaglandin infusion is
mandatory.
 A balloon atrial septostomy may be a life-saving procedure which may need to be
performed in 20% of those with TGA.
 Definitive surgery – arterial switch performed within the first few days of life.

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 Tricuspid atresia

In tricuspid atresia, only the left ventricle is effective, the right being small and non-functional.
There is total absence of the tricuspid valve.
Presents with cyanosis in newborn period.
Management- BT Shunt, pulmonary art banding.
Total correction is not possible.
Palliative surgery with Fontan or Glenn operation.

Total Anomalous pulmonary venous Drainage

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 Truncus Arteriosus

Summary of Congenital Heart Diseases

Left-to-Right Shunts
Lesion Symptoms signs Management
ASD
Secondum None ESM at ULSE Catheter device
Fixed split S2 closure at 3–5 years

Primum None ESM at ULSE Surgery at 3 years

Fixed split S2
Pansystolic murmur at apex
VSD
Small None Pansystolic murmur at LLSE None

Large Heart failure Active precordium, loud P2, Mx heart failure

Recurrent infections soft murmur, tachypnoea, calories
FTT etc hepatomegaly Surgery at 3–6
months old
PDA None Continuous murmur at Coil / device closure at
ULSE ± bounding pulses cardiac catheter at 1
year, or ligation

Right-to-Left Shunts (cyanotic congenital heart diseases)

Lesion C/F Management
TOF Loud murmur at ULSE
Clubbing of fingers and toes Surgery at 6–9 months
(older)
Hypercyanotic spells
Neonatal cyanosis Prostaglandin infusion
TGA No murmur Balloon atrial septostomy
Arterial switch operation in
neonatal period
Eisenmenger syndrome No murmur Medication to delay
Right heart failure (late) transplantation

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Outflow obstruction in the well child

Lesion Signs Management

Aortic stenosis Murmur, upper right sternal Balloon dilatation
edge; carotid thrill
Pulmonary stenosis Murmur, upper left sternal Balloon dilatation
edge; no carotid thrill
Coarctation (adult type) Systemic hypertension Stent insertion or surgery
Radio-femoral delay

Outflow obstruction in the sick infant (i.e. duct dependent lesions)

Lesion C/F Management

Coarctation of the aorta Circulatory collapse Maintain ABC
Absent femoral pulses Prostaglandin infusion
Interruption of the Aortic Circulatory collapse Maintain ABC
arch Absent femoral pulses and Prostaglandin infusion
absent left brachial pulse
Hypoplastic left heart Circulatory collapse Maintain ABC
syndrome All peripheral pulses absent Prostaglandin infusion

Rheumatic fever (RF)

The most important cause of heart disease in children worldwide.
Mainly affects children aged 5–15 years.
Common in developing countries.

Pathophysiology:
In susceptible individuals, there is an abnormal immune response to a preceding infection with
group A β-haemolytic streptococcus.
The bacterial Ag imitates body Ag so the antibodies prepared against the bacterial antigens
cross-react with body antigens.

Diagnosis:
is based on C/F and investigations; by the modified Duckett-Jones criteria.

Major criteria
 Migratory polyarthritis
 Carditis

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  Sydenham chorea
 Erythema marginatum
 Subcutaneous nodules

Minor criteria
 Fever
 Polyarthralgia
 Raised actue phase reactants (ESR,CRP)
 Prolonged P-R interval on ECG
 History of Rheumatic fever

To make the diagnosis :

 Two major criteria / one major and two minor criteria
 + supportive evidence of preceding group A streptococcal infection
(markedly raised ASO titre/other streptococcal antibodies, or group A streptococcus on
throat culture).

Polyarthritis (80%)
 Ankles, knees and wrists
 Exquisite tenderness,
 moderate redness and swelling
 'Flitting', lasting <1 week in a joint, but migrating to other joints over 1–2 months

Pancarditis (50%)
Endocarditis
• significant mid diastolic murmur (Carey Coomb murmur due to swelling of mitral valve)
• valvular dysfunction
Myocarditis
• may lead to heart failure and death
Pericarditis
• pericardial friction rub
• pericardial effusion
• tamponade

Sydenham chorea (10%)

 Occurs 2–6 months after the streptococcal infection
 Involuntary movements and emotional lability for 3–6 months

Erythema marginatum (<5%)

 Uncommon, early manifestation
 Rash on trunk and limbs
 Pink macules spread outwards, causing pink border with fading centre. Borders may
unite to give a map-like outline.

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Subcutaneous nodules (rare)
 Painless, pea-sized, hard
 Mainly on extensor surfaces

RF occurs about 2 weeks after a pharyngeal infection by Group A streptococci.

But only 80-85% of patients with acute RF have a raised ASO titre.

Chronic rheumatic heart disease

The most common form of long-term damage from scarring and fibrosis of the valve tissue of
the heart is mitral stenosis.
Mitral stenosis causes a mid diastolic murmur. (vs the Carey Coomb murmur in acute setting)
Although the mitral valve is the most frequently affected, aortic, tricuspid and, rarely,
pulmonary valve disease may occur.

Management:

Management of acute episode:

 Admit patient
 Bed rest
 Aspirin - Aspirin is very effective at suppressing the inflammatory response of the joints
and heart. It needs to be given in high dosage and serum levels monitored.
Dose -
 If carditis or heart failure is present, corticosteroids will be required.
 But anti inflammatory agents (aspirin, corticosteroids) should be withheld if arthralgia is
the only presentation as these drugs could interfere with the development of the
characteristic migratory polyarthritis.
 Anti-streptococcal antibiotics (oral penicillin for 10 days or IM bezathine penicillin single
dose) may be given if there is any evidence of persisting infection.

Prevention of recurrence:
Monthly injections of benzathine penicillin is the most effective prophylaxis.
Alternatively, the penicillin can be given orally every day, but compliance may be a problem.
Oral erythromycin can be substituted in those sensitive to penicillin.
Dose – 1.2 million units/dose if weight>30kg or 0.6 million units if <30kg
Length of prophylactic treatment:
 Only RF – for 5 yrs or until 21 yrs of age (whichever is longer)

 RF with carditis – for10 yrs or until 25yrs of age (whichever is longer)

 RF with residual heart disease – for10 yrs or until 40 yrs of age (whichever is loger) or
even lifelong prophylaxis
Complications of RF: chronic valve disease, n therefore the risk of infective endocarditis

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 Infective Endocarditis (IE)
Is the inflammation of the endocardium due to an infection.
The most common causative organism is α-haemolytic streptococcus (Streptococcus viridans).

For infection to occur, there should be a predisposing risk factor, such as:
 Children of any age with congenital heart disease (except secundum ASD), including
neonates.
 Patients with prosthetic heart valves etc.

Diagnosis: is based on modified Duke criteria

Major criteria
 2 positive blood cultures with typical organisms, 2 or more for less typical organisms
 Evidence of endocarditis on echo
o Intracardiac mass on a valve or other site (vegetations)
o Regurgitation near a prosthesis or abscess.
Minor criteria
o Predisposing conditions
o Fever
o Emboli-vascular signs
o Immune complex phenomena (glomerulonephritis, arhtitis, rheumatoid factor, osler
nodes, roth spots etc)
o Single positive blood culture/ serologic evidence of infection/ echo signs not meeting
the major criteria
o Presence of newly diagnosed clubbing/spelnomegaly
o Splincter hmges and petechiae
o High ESR , CRP
o Presence of non feeding lines or peripheral lines
o Microscopic haematuria.

Diagnosis : 2 major criteria / 1 major + 3 minor/ 5 minor

Investigations:
Blood culture – taken before starting antibiotics
Echo
Acute phase reactants

Management :

Treatment:
IV Antibiotic treatment for 4-6 weeks (penicillin+ aminoglycosides)
Prophylaxis :

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The most important factor in prophylaxis against endocarditis is good dental hygiene, and this
should be strongly encouraged in all children with congenital heart disease.
Antibiotic prophylaxis may be required in other countries for:
• Dental treatment, however trivial
• Surgery, which is likely to be associated with bacteraemia.
Dose: oral amoxicillin 50mg/kg 1 hr before procedure.

Child with fever and new onset murmur= IE

Complications:
 heart failure if aortic/mitral valve is involved.
 Myocardial abscess
 Myocarditis
 Life threatening arrhythmias
 Systemic emboli, often with CNS manifestations
 Pulmonary emboli may occur in children with VSD or TOF

Myocarditis / Dilated Cardiomyopathy

Dilated cardiomyopathy (a large, poorly contracting heart) may be :
 inherited,
 secondary to metabolic disease or
 may result from a direct viral infection of the myocardium.

It should be suspected in any child with an enlarged heart and heart failure who has previously
been well.

Diagnosis : with echo

Treatment :
 symptomatic treatment for heart failure.
 Role of steroids & immunoglobulins is controversial

Myocarditis usually improves spontaneously, but some children ultimatelyrequire heart

transplantation.
Other cardiomyopathies (hypertrophic/restrictive) are rare in childhood and are usually related
to a systemic disease (e.g. Hurler, Pompe or Noonan syndromes).

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 Kawasaki Disease
 Kawasaki disease (KD) is a systemic vasculitis.
 Although uncommon, it is an important diagnosis to make because of it’s most serious
complication - aneurysms of the coronary arteries.
 This mainly affects children of 6 months to 5 years, with a peak at the end of first year.
 Is thought to be due to immune hyperreactivity to a variety of triggers in a genetically
susceptible host.

Diagnosis : is made on clinical findings.

 Fever for > 5days

+ 4 of the following features:
 Conjuctivits
 Mucous membrane changes : pharyngeal infection;
red, dry, cracked lips;
strawberry tongue.
 Cervical lymphadenopathy
 Polymorphous rash
 Red and oedematous palms and soles / peeling of fingers and toes.

There may be inflammation a the BCG vaccination site.

Investigations:

 FBC (increased neutrophils during 1st week of illness, and increased platelets in 2 nd-3rd
weeks)
 Increased ESR & CRP
 Echo – may show aneurysms in prolonged untreated illness.

Treatment :

 Prompt treatment with intravenous immunoglobulin (IV Ig) given within the first 10 days
has been shown to lower the risk of coronary artery aneurysms.
 Aspirin is used to reduce the risk of thrombosis.

Complications:
Cardiovascular signs:
 gallop rhythm
 myocarditis, pericarditis
 Coronary and peripheral aneurysms
 Sudden death

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