Acquired Heart

Disease in Pediatric
{ Dr. Bima Suryaatmaja
Dr. Fahmi Ahmad Muslim
 PEDIATRIC
CARDIOMYOPATI

BIMA SURYAATMAJA

Resource Person : dr. Poppy S. Roebiono, SpJP (K)

 Definition

 Cardiomyopathy is a myocardial disorder in

which the heart muscle is structurally and
functionally abnormal, in the absence of CAD,
hypertension, valvular disease and congenital
heart disease sufficient to cause the observed
myocardial abnormality

Pediatric Cardiology, 3rd Ed. Churchill livingstone.2010

 Classification

1. Hypertrophy cardiomyopathy (HCM).

2. Dilated ( congestive) cardiomyopathy.
3. Restrictive cardiomyopathy
4. Arrhythmogenic right ventricular cardiomyopathy
5. Unclassified cardiomyopathies

Pediatric Cardiology, 3rd Ed. Churchill livingstone.2010

 Hypertrophic
cardiomyopathy
 Prevalence
 The frequency of left ventricular hypertrophy in
children is unknown
 population-based studies from Australia and the

United States  incidence between 0.3 and 0.5

cases per 100,000
 frequency is greater in males, and highest in the first

year of life

Pediatric Cardiology, 3rd Ed. Churchill livingstone.2010

 Definition

 HCM is characterized by a thickened but

nondilated LV in the absence of another
cardiac or systemic disease capable of
producing the magnitude of hypertrophy.

Moss and Adams’Heart Disease in infant, children, and adolescents 7th Ed

 Etiology
 Genetic is the most common cause
 In less than one-tenth of infants and children,

hypertrophic cardiomyopathy can be

associated with inborn errors of metabolism,
neuromuscular disorders, and malformation
syndromes
 In children with idiopathic hypertrophy,

approximately one-third are diagnosed in

infancy, one-third during adolescence, and
the other third between the ages of 1 year and
11 years

Pediatric Cardiology, 3rd Ed. Churchill livingstone.2010

 Pathophysiology of HCM
The pathophysiology of HCM involves 4 interrelated
processes:
 Left ventricular outflow obstruction

 Diastolic dysfunction

 Myocardial ischemia

 Mitral regurgitation

 Arrhytmia

Moss and Adams’Heart Disease in infant, children, and adolescents 7th Ed

LV Outflow Obstruction in HCM
 Approximately one-quarter of children and adults with
hypertrophic cardiomyopathy have obstructed left
ventricular outflow tracts at rest

 Long-standing LV outflow obstruction is a major

determinant for heart failure symptoms and death in HCM
patients

 Obstruction in HCM  True mechanical impedance to

LV outflow  Producing markedly increased
intraventricular pressures  may be detrimental to LV
function by increasing myocardial wall stress and oxygen
demand
 LV Outflow Obstruction in HCM
Subaortic outflow obstruction is caused by systolic
anterior motion (SAM) of the mitral valve – leaflets move
toward the septum

The magnitude of the outflow gradient is directly related to

the duration of mitral-septal contact

In infants and young children with HCM, obstruction to

right ventricular outflow is not uncommon, usually
occurring in association with subaortic obstruction
Pediatric Cardiology, 3rd Ed. Churchill livingstone.2010
 Diastolic dysfunction
 Contractile function of the ventricle is
enhanced, but ventricular filling is impaired
by relaxation abnormalities.
 Myocardial Ischemia

- Often occurs without atherosclerotic coronary artery disease.

Postulated mechanisms :
 Abnormally small and partially obliterated intramural

coronary arteries as a result of hypertrophy.

 Inadequate number of capillaries for the degree of LV

mass.
 Mitral Regurgitation

 Results from the systolic anterior motion

of the mitral valve.
 Severity of MR directly proportional to LV
outflow obstruction.
 Results in symptoms of dyspnea,
orthopnea in HCM patients.
 Arrhythmia

 Rare in children and young adults

 Potentials trigger of arrhytmias :

 The characteristic features of myocytic

disarray and interstitial fibrosis

 myocardial ischaemia
 Clinical Manifestations
 Easy fatigue. Presentation in infancy :
 Palpitation, dizziness. • Breathlessness
 DOE, orthopnea,
• poor feeding
PND. • excessive sweating
• failure to thrive
 Angina.

 Syncope,

Presyncope.
 Sudden cardiac

death.
Moss and Adams’Heart Disease in infant, children, and adolescents 7th Ed
 Physical Examination
 A sharp upstroke of the arterial pulse.
 bisferiens pulse
 jugular venous pulsation may reveal a prominent a wave
 double apex beat
 A systolic thrill at the apex or along the LLSB.
 Murmur
 Medium-pitch,1 to 3/6 ejection systolic murmur along LLSB or at the apex.
radiates to the right upper sternal edge and apex, but usually not to the
carotid arteries or axilla
 A soft holosystolic murmur of mitral regurgitation (MR) is often
present. radiating to the axilla.
 Dynamic maneuvers
 Murmur intensity increases with decreased preload (i.e.
Valsalva)
 Murmur intensity decreases with increased preload (i.e.
squatting, hand grip).
ELECTROCARDIOGRAPHY
Echocardiography in HCM
Management

Pediatric Cardiology, 3rd Ed. Churchill livingstone.2010

 Infant of Diabetic Mother

 Have a high prevalence of congenital heart

defects, cardiomyopathy, and persistent
pulmonary hypertension of the newborn
(PPHN).
 HCM with or without obstruction is seen in
10% to 20% in these infants.
 In most cases, the hypertrophy spontaneously
resolves within the first 6 to 12 months of life.

Myung K. Park Pediatric Cardiology For Practitioners 5th Ed

 Transient Hypertrophic
Cardiomyopathy in Neonates
 Acute fetal distress with myocardial ischemia
 Initially, echo studies showed abnormal LV

systolic and diastolic function but the LV wall

thickness was normal.
 LVH : days 2 and 7 affected initially the IVS and

later the LV posterior wall.

 Disappeared in all cases between 1 and 5

months of life.

Myung K. Park Pediatric Cardiology For Practitioners 5th Ed

Dilated (congestive)
Cardiomyopathy
Dilated (congestive) Cardiomyopathy
 The most common form of cardiomyopathy.
 males and females are approximately equally

affected
 The majority of children with dilated

cardiomyopathy present before 1 year of age

 Decreased systolic function is associated
with dilatation of all four cardiac chambers.
 Prevalence

36.5 per 100.000 Population

Etiology of DCM
 >60% idiophatic.
 Familial : Autosomal dominan
inheritance.
 Infection (coxsackievirus,
adenovirus, etc)
 Metabolic.
 Nutritional disorders (def carnitin,
thiamin, selenium)
 Cardiotoxic agents such as
doxorubicin, alcohol.
Pathology

 Macroscopically :
 dilated cardiomyopathy is characterised by the presence
of a globular heart, with dilation of the ventricles and
diffuse endocardial thickening.

 Microscopic:
 myocyte degeneration with irregular hypertrophy and
atrophy of myofibers.
PATHOGENESIS
Pathophysiology Myocyte injury

Contractility

Stroke volume

Ventricular filling pressures LV dilatation CO

Pulmonary congestion - Fatigue
Dyspneu MR -Weakness
Ortopneu - sincope
Rales - GIT symptoms
Systemic congestion
Edema
Ascites
JVD
Pathophysiology of Heart Disease, Leonard S. Lilly
Symptoms

 Infants with dilated cardiomyopathy typically

present with :
 poor feeding

 tachypnoea

 respiratory distress

 Diaphoresis during feeding

 failure to thrive
Complications

Heart failure
Atrial or ventricular arrythmias

Sudden death : arrhythmias, and

massive embolization
Electrocardiography
Chest Radiography
ECHOCARDIOGRAPHY
Other Modalities

 Cardiac Biomarkers
 Exercise Testing

 Cardiac Catheterisation

 Cardiac Magnetic Resonance Imaging

Treatment
 HF : Diuretic, ACE inhibitor, digoxin, Beta
blocker, nesiritide
 Anticoagulation

 Antiplatelet and antithrombotic agents

 Prevention & treatment arrythmia

(amiodaron or ICD (?)).

 Cardiac transplantation.
 Restrictive
cardiomyopathy
 Restrictive cardiomyopathy

 Characterized by restrictive filling and reduced

diastolic volume with normal or near normal
ventricular systolic function and wall
thicknesses.

 Extremely rare form of cardiomyopathy, accounting

for 2.5%-5% of cardiomyopathy cases in children.

 Increased interstitial fibrosis may be present.

 Etiology

It may be idiopathic or associated

with another disease.
 Pathophysiology of RCM

Diastolic JVD, hepatomegaly,

Venous
ventricular Ascites, peripheral edema
congestion
pressure

Rigid
myocardium

Weakness
Ventricular CO Fatigue
filling
 Clinical Manifestation

 JVD
 S3, S4, or both

 Elevation in CVP

 Peripheral edema, liver enlargement, ascites

 The Kussmaul Sign

 AV block

 Symptomatic bradycardia

 Atrial fibrillation
Electrocardiography
 Chest Radiography

 it is abnormal in approximately 90% of cases

 Cardiomegaly caused by atrial enlargement

 pulmonary venous congestion

ECHO RCM
Other Modalities

 Ambulatory Electrocardiographic
Monitoring
 Exercise Testing

 Cardiac Catheterisation

 Cardiac Magnetic Resonance Imaging

 Treatment
 Diuretics are beneficial to relieve congestive symptoms,
but should be carefull.
 Beta blocker, amiodaron

 CCB may be used to increase diastolic compliance.

 Anticoagulant & antiplatelet.

 Corticosteroids for sarcoidosis

 Chelation therapy for hematochromatosis

 A permanent pacemaker for TAVB.

 Cardiac transplantation is the only definitive treatment

 PROGNOSIS: Generally poor

 ARRHYTHMOGENIC
RIGHT VENTRICULAR
CARDIOMYOPATHI
 definitions

 Arrhythmogenic right ventricular

cardiomyopathy is a myocardial disease
characterised by progressive replacement of
right ventricular myocardium by fibrous
tissue and fat, initially with regional, and
later with global, right and left ventricular
involvement.
Epidemiology

 In children, arrhythmogenic cardiomyopathies

account for less than 3% of all cardiomyopathies

 There is a male predominance across all ages

 ETIOLOGY

 Inherited in up to half the cases :

 The mode of transmission is

usually autosomal dominant

with variable penetrance
 Natural History
 Early concealed phase : asymptomatic, and clinical
features are absent
 Electrical disorder phase : symptomatic ventricular

arrhythmia and morphological abnormalities

 Right ventricular phase : extension of fibro-fatty

infiltration throughout the right ventricular myocardium

cardiac failure
 Biventricular phase :left ventricular involvement and
biventricular failure (indistinguishable from dilated
cardiomyopathy)
 Pathology
 Early stages :
 abnormal pathological findings are localised to the

apical, inflow, and infundibular areas of the right

ventricle, initially referred to as the triangle of
dysplasia
 Progression :

 the left ventricle may also become involved, with

particular involvement of the posterolateral wall and
relative sparing of the septum
 Clinical Features

 The first presentation of the disease is often

sudden cardiac death in previously
asymptomatic individuals, including young
children and teenagers
 experienced syncope

 arrhythmic symptoms predominate

 Cardiac failure
ELECTROCARDIOGRAPHY
ECHOCARDIOGRAPHY
 Other Modalities

 Ambulatory Electrocardiographic Monitoring

 Exercise Testing

 Cardiac Magnetic Resonance Imaging

 Endomyocardial Biopsy
 TREATMENT

 Antiarrhythmic agents : β-adrenoreceptor

blockers and amiodarone
 HF : diuretics,ACEI, and anticoagulation.

 ICD

 Cardiac Transplantation
 UNCLASSIFIED
CARDIOMYOPATHIES

 Left Ventricular Non-compaction

 Endocardial Fibro-elastosis

 Tako-tsubo Cardiomyopathy
Left Ventricular Non-compaction

 a disorder of myocardial morphogenesis that results in multiple prominent

trabeculations and deep intertrabecular recesses in the left ventricular
myocardium
 The clinical features in adults and children include left ventricular systolic
and diastolic dysfunction, thromboembolism, and ventricular arrhythmia.
 The medical treatment aims to improve symptoms and prevent
complications.
 Aspirin has been recommended in all patients by some groups, with

warfarin for patients with documented thromboembolic events

 vasodilators and β-blockers,

 insertion of an implantable cardioverter defibrillator. In some children

refractory to medical therapy, cardiac transplantation may beconsidered.
 Poor Prognosis
 Endocardial Fibro-elastosis

 a rare and poorly understood disease of the endomyocardium.

 diagnosis is made histologically, and the pathological hallmarks consist
of deposition of collagen and elastin, ventricular hypertrophy, and diffuse
endocardial thickening
 aetiology is not fully understood. Possible aetiopathogenic factors
include viral infections (coxsackievirus, adenovirus, parvovirus, and
mumps virus), metabolic and storage disorder
 Endocardial fibroelastosis usually presents in infancy, and the clinical
features are typically indistinguishable from those seen in dilated
cardiomyopathy
 The management is symptomatic, and follows that of dilated
cardiomyopathy, including diuretics, vasodilators and digoxin.
Tako-tsubo Cardiomyopathy
 Cardiomyopathy characterised by transient and rapidly
reversible left ventricular apical ballooning and systolic
dysfunction in the absence of coronary arterial disease,
triggered usually by profound psychological stress
 It is associated with characteristic elevation of the ST
segments on the electrocardiogram that mimics acute
myocardial infarction
 Most affected patients are over 60 years old. Recently, was
reported in a 2-year-old girl, following withdrawal of
buprenorphine treatment
KAWASAKI DISEASE
Fahmi Ahmad Muslim

NARASUMBER
Dr. Poppy S Roebiono, SpJP (K)
 Kawasaki disease was first described in 1967,
by a Japanese paediatrician, Tomisako
Kawasaki.
 Characterised the illness, then termed
mucocutaneous lymph node syndrome, at the
time, the disease was thought to be self-
limited, without longterm consequences.
 Later, Kawasaki disease was shown to cause
coronary arterial aneurysms, which may
cause complications of angina, myocardial
infarction, and sudden death.
 Kawasaki disease has surpassed rheumatic
fever as a cause of acquired cardiac disease
in children.

Introduction Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Kawasaki disease has an incidence of
approximately 138 cases for each
100,000 children under the age of 5 years.
 In the united States of america, Kawasaki
disease has been reported to be most
common among asians and Paciic Islanders,
with an incidence of 32.5 cases for each
100,000 children under the age of 5
years, intermediate in non-Hispanic african
americans, where the number is 16.9, and
Hispanics, at 11.1 cases, and lowest in
Caucasians, with 9.1 cases in each 100,000
children under the age of 5 years.

Epidemiology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Kawasaki disease is most common in children
younger than age 5 years, but one-
quarter of cases in the united States of
america occur in older children.
 Young infants have the highest rate of
formation of coronary arterial aneurysms,
and often present with incomplete
features.
 Children older than age 8 years also
have a higher rate of coronary arterial
involvement.

Epidemiology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Coronary arterial disease is responsible
for almost all deaths in patients with
Kawasaki disease. The case fatality rate is
0.08% in Japan.
 In the united States, reported in hospital
mortality for Kawasaki disease has varied
from 0% to 0.17%.
 Although the highest risk of myocardial
infarction and death occurs in the first
months after illness onset, sudden death
from ischaemic heart disease may occur
many years later in patients with
coronary arterial aneurysms and stenoses.
Epidemiology Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 The cause of Kawasaki disease remains
unknown, despite decades of investigation
and spirited controversy.
 Clinical signs and symptoms overlap with
those in known toxin mediated or viral
infections.
 Predilection for young children, with rare
occurrence in neonates and adults, suggest
that immunity is acquired.
 Reports of selective expansion of Vβ 2
and Vβ 8 T-cell receptor families in the
acute stage of the disease, have suggested
that the illness may be caused by
bacterial superantigens.

Aetiology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Other investigations have suggested that
the immune response in the disease is
evoked by a conventional antigen.
 Cytoplasmic antigen, bound by a synthetic
IgA antibody, has been observed in the
proximal bronchial epithelium and coronary
arteries of the majority of postmortem
specimens from children with the disease,
but not in postmortem control patients.

Aetiology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 The data suggests that the disease may be
caused by a respiratory infectious agent with
tropism for vascular tissue. It is also possible
that it may be triggered by more than one
microbial agent in a susceptible host.
 The importance of genetic factors in
susceptibility is supported by the inluence of
race and family history on its incidence .
 In addition, an increasing literature has
explored the association of genetic
polymorphisms to susceptibility to the disease,
or to development of aneurysms.

Aetiology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 marked immune activation, with release of
proinlammatory cytokines and growth
factors, activation of endothelial cells, and
iniltration of coronary arteries and other
medium-sized extraparenchymal arteries by
CD68+ monocyte/macrophages, CD8+
cytotoxic lymphocytes, and oligoclonal Iga
plasma cells.
 The integrity of the arterial wall may be
disrupted by infiltration of macrophages and
release of matrix metalloproteinases.

Pathogenesis Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Mortality peaks between 15 and 45 days after
onset of fever, when patients are in a
hypercoagulable state and have
thrombocytosis and disrupted vascular
endothelium.
 Mortality declines signiicantly beyond the irst
year after the onset of the illness. because of
progressive coronary arterial stenosis, however,
myocardial infarction may occur many years
later.
 an increasing literature has shown that when
diagnosis is not made in childhood, the disease
can cause myocardial infarction in young adults.

Pathology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 In the first 9 days of illness, indings include
acute perivasculitis and vasculitis of the
microvessels and small arteries, as well as
acute perivasculitis and endarteritis of the
three major coronary arteries, with inlux of
neutrophils.
 Inlammation in the pericardium,
myocardium, atrioventricular conduction
system, and endocardium are also present.
 Deaths in this early period are caused by
myocarditis and abnormalities of conduction.

Pathology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 In the second through fourth weeks of the disease,
the coronary arteries are affected by panvasculitis,
and aneurysms form, with coronary arterial
thrombosis.
 The cells iniltrating the arterial wall range from
neutrophils to large mononuclear cells, and include
lymphocytes and plasma cells. at this stage, the
internal elastic lamina shows segmental destruction.
Pericarditis, myocarditis, inlammation of the
atrioventricular conduction system, and endocarditis
with valvitis, continue to be present.
 Mortality in these weeks is most likely to be
secondary to ischaemic heart disease, rupture of
aneurysms, and myocarditis, including lesions of the
conduction system.

Pathology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 After 1 month, active inlammation begins to be replaced by
progressive neointimal proliferation, neoangiogenesis, and fibrosis
with formation of scars. Late deaths are characterised by severe
coronary arterial stenosis.
 aneurysms produced by the disease occur in locations that are
similar to those of atherosclerotic lesions, that is, in the
proximal segments and branches of the coronary arteries,
suggesting a role for shear stress in both types of coronary
lesions.
 In addition, growth factors are prominently expressed at the
inlets and outlets of the aneurysms, these also being sites of high
shear stress.
 aneurysms can occur in medium-sized extraparenchymal
arteries other than the coronary arteries.
 In particular, the coeliac, mesenteric, femoral, iliac, renal, axillary,
and brachial arteries can be affected.
 Peripheral arterial aneurysms, however, never occur in the
absence of aneurysms involving the coronary arteries.

Pathology Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 The epidemiologic deinition for diagnosis
includes fever for 4 days with at least four
principal clinical criterions, or fever and
fewer than four principal criterions in the
presence of coronary arterial
abnormalities.
 the first day of the disease is considered
to be the day on which the fever initially
occurs.

Clinical Diagnosis Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Clinical Features Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Conjunctival injection, lip edema and erythema on day 6 of illness in a two-year-old
boy with Kawasaki disease.
Figure 1, Rash on day 4 of illness in a seven-month-old boy with Kawasaki disease.

Edema and desquamation

Periungual erythema on day
on 6day
of illness in a one
12 of illness and one-half–year-
in Kawasaki disease.
old girl with Kawasaki disease.

Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 The diagnosis may be based upon
abnormalities of the coronary arteries
by echocardiography when the classic
epidemiologic definition is not fulilled.
 Laboratory features are relective of an
acute inlammatory response.

Clinical Diagnosis Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Differential Diagnosis American Heart Association,
Circulation 2004
Laboratory
American Heart Association,
Circulation 2004
 The acute phase of the disease may be
associated with myocarditis, pericarditis, valvitis,
and inlammation in the coronary arterial wall.
 Cardiac auscultation typically reveals a
hyperdynamic praecordium, tachycardia, and
a gallop rhythm, even in the absence of fever.
 Almost all children have an innocent low
murmur related to anaemia and fever. In
addition, some children with significant mitral
regurgitation have a pansystolic regurgitant
murmur at the apex.
 The disease may occasionally present with low
cardiac output syndrome or shock.
 Electrocardiography may show arrhythmia,
prolonged PR interval, or nonspecific ST and T
wave changes.
Cardiac Finding Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Coronary arterial aneurysms are the most serious
longterm complications of the disease.
 Approximately one in five children who are not
treated with high doses of immune globulin
given intravenously within the first 10 days of
illness develops coronary arterial ectasia or
aneurysms.
 The aneurysms may be detected by
echocardiography beginning 7 days after the first
appearance of fever, with their diameter usually
peaking around 4 weeks after onset of the illness.
 Independent predictors of development have
included age less than 1 year, male gender, delayed
treatment with immunoglobulins, persistent or
recrudescent fever after immunoglobulins, so called
immunoglobulin resistance, and laboratory
measures suggesting worse inlammation.

Clinical Finding Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Moss and Adams' Heart Disease, 7th
Ed, 2008
Evaluation of Suspected
Incomplete Kawasaki Disease

American Heart Association,

Circulation 2004
 Aneurysms are considered to be saccular
when their axial and lateral diameters are
nearly equal.
 Fusiform aneurysms occur when there is
symmetric dilation, with gradual proximal
and distal tapering.
 Coronary arteries are considered to be ectatic
when the dimension is dilated without a seg-
mental aneurysm.
 Aneurysms are classiied as giant when the
internal diameter is at least 8 mm.

Aneurysm Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
FIGURE 61.2 Serial right coronary angiograms in a girl who
developed Kawasaki disease at 2 months of age. A: Right
coronary arteriogram obtained 8 months after the onset
shows two large saccular aneurysms. B: Follow-up right
coronary arteriogram 3.5 years later shows thrombotic
obstruction of both aneurysms. The distal right coronary
artery is opacified via tortuous recanalized arteries.

Moss and Adams’ Heart Disease, 7th

ed, 2008
 Depression of myocardial function is common
in the acute phase of the disease.
 Endomyocardial biopsies have suggested that
myocarditis is a universal feature of Kawasaki
disease, and myocardial inlammation is present
in from half to three quarters of patients based
upon nuclear imaging.
 Fortunately, myocardial function usually
improves rapidly after administration of
intravenous immune globulin, and long-term,
clinically signiicant abnormalities of systolic
function are uncommon in the absence of
ischaemic heart disease secondary to coronary
arterial aneurysms.

Myocarditis Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 In the acute phase, mitral regurgitation may
result from valvitis, or from transient
dysfunction of the papillary muscles. More
than one-quarter of children have mitral
regurgitation at the time of presentation.
 Late mitral regurgitation is usually the
result of ischaemic disease.
 Aortic regurgitation, presumably secondary
to valvitis, is infrequently detected by
echocardiography in the acute phase.
 Only rare instances of late mitral
regurgitation unrelated to ischaemia or late
aortic regurgitation of clinical signiicance
have been reported.

Heart Sound Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
FIGURE 61.4 Representative electrocardiogram leads in a 4-
month-old girl with Kawasaki disease recorded on illness days
17 and 20. The latter tracings show marked decrease in R-
wave voltage (V2–V5), indicative of acute anterior wall
infarction, which was proven at autopsy. Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Echocardiographic imaging of the coronary
arteries is essential in the evaluation of all
patients with definite or suspected disease.
 Its sensitivity and specificity for the
detection of dilation of the proximal coronary
arterial segments are high
 Aneurysms also occur in the absence of the
classic criterions, echocardiography has an
important role in evaluation of children with
protracted fever and some findings consistent
with features of Kawasaki disease.

Echocardiography Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Echocardiography should be performed shortly after
diagnosis to provide a baseline examination of
coronary dimensions, left ventricular function,
valvular regurgitation, and pericardial effusion.
 The most common site for formation of
aneurysms are the proximal segments of the anterior
interventricular and right coronary arteries,
followed in descending order by the main stem of
the left coronary artery, the circumlex artery and
the distal part of the right coronary artery, and the
junction between the right coronary artery and
inferior interventricular artery.
 Measurements are made from inner edge to inner
edge, excluding points of branching.

Echocardiography Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Microstructure of Blood Vessels
Hemodynamic of Vascular,
Physiology
Pathophysiology of Heart Disease,
Leonard S. Lilly.— 5th ed © 2011
 These classify coronary arteries as
abnormal if the internal luminal diameter
is greater than 3 mm in children less than
5 years of age, or 4 mm in children at
least 5 years of age, if the internal
diameter of a segment measures at least
1.5 times that of an adjacent segment, or
if the coronary arterial lumen is clearly
irregular.

Aneurysm of Coronary Arteries

American Heart Association,
Circulation 2004
 Normal dimensions are related to body size,
dimensions may also be expressed as units of
standard deviation, or z scores, adjusted for body
surface area.
 Use of such z scores has suggested that the criterions
adopted by the Japanese Ministry of Health may
result in under diagnosis and underestimation of the
true prevalence of arterial dilation in the first weeks
of the illness
 There is no normative data for febrile patients with
other diseases. Z scores are available only for the
main stem of the left coronary artery and the
proximal segments of the anterior interventricular
and right coronary arteries.
 For this reason, the criterion of size 1.5 times
that of the surrounding segment is still useful for
diagnosis of aneurysms in peripheral sites.

Aneurysm of Coronary Arteries Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Z Scores
American Heart Association,
Circulation 2004
 Children with aneurysms should undergo periodic
stress testing, with assessment of myocardial
perfusion or function.
 Most methods of stress testing used in adult
cardiology have been reported in small series of
children with Kawasaki disease.
 Adult guidelines should be followed to choose the
best test based upon specific characteristics of
the patients.
 False positive tests are more likely in patients
with a low prior probability of coronary arterial
disease.
 Not recommend performing stress tests in
patients without a history of coronary arterial
enlargement.

Stress Testing Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Ultrafast computerised tomography, and
magnetic resonance imaging and angiography,
may provide valuable noninvasive imaging data
in patients whose coronary arteries cannot
adequately be imaged by echocardiography
 In addition to imaging, cardiac resonance tests
can be performed together with pharmacologic
stress, and may allow assessment of myocardial
infarction using delayed enhancement
 Ultrafast computerised tomography has been
shown to have excellent sensitivity for detection
of coronary arterial stenoses in patients with
the disease.

Other Modality Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Cardiac catheterisation and coronary angiography provide
the gold standard for imaging, against which other
methods are assessed
 its use should be restricted to selected patients with
coronary arterial aneurysms, clinical signs, noninvasive
studies indicating myocardial ischaemia, or those in whom
noninvasive methods fail to provide adequate images.
 angiography is usually performed 6 to 12 months after the
onset of the illness in patients with significant aneurysms
who have no signs or symptoms of ischaemic heart
disease.
 angiography can be helpful in guiding anti-thrombotic
therapy when proximal aneurysms have regressed, but
the distal parts of the coronary arteries cannot be imaged
by noninvasive means.
 Some cardiologists follow patients who have undergone
surgical revascularisation or catheter intervention by
cardiac catheterisation to evaluate the eficacy of their
treatment.

Coronary Angiography Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 FIGURE 61.1 Serial right coronary arteriograms in a boy who
developed Kawasaki disease at 3 months of age. A: Right
coronary arteriogram performed 3 months after the onset
shows an extensive segmented aneurysm involving the entire
right coronary artery. B: Follow-up study obtained 16 months
later shows near-complete regression of the aneurysm. (From
Takahashi M, Mason W, Lewis AB. Regression of coronary
aneurysms in patients with Kawasaki syndrome. Circulation
1987;75:390, with permission.)

Moss and Adams' Heart Disease in Infants,

Children, and Adolescents: Including the Fetus and Jane Newburger, Chapter 52 in
Young Adults, 7th Edition PAEDIATRIC CARDIOLOGY, 3rd
Copyright ©2008 EDITION © 2010
 Follow-up for the past three decades.
 Aneurysms peak diameter in 4 to 6 weeks after.
 A myointimal proliferation  regression of approximately
half to two-thirds of aneurysmal segments
 Aneurysms that persist may develop significant stenoses
secondary to myointimal proliferation at either end of the
aneurysm, calcification, tortuosity, or thrombotic
occlusion.
 The principal cause of death is myocardial infarction
produced by thrombotic occlusion in a coronary arterial
segment with an aneurysm and/or stenosis.
 Regression of the aneurysms restores the internal luminal
diameter to normal. Fibrous intimal thickening on
histopathologic examination.
 At least 10 years after onset, greater thickening of the
intimal and medial layers was signiicantly associated with
larger initial diameter of the coronary arteries.
 Vasodilation is progressively impaired & endothelial
dysfunction  constriction .
Natural History Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 The First weeks after onset is aimed at lowering
fever for comfort, reducing inlammation and shear
stress in the arterial wall, and preventing thrombosis.
 To reduce shear stress, children in whom coronary
aneurysms are developing should undergo
transfusion of red blood cells if they are
profoundly anaemic, ideally to achieve a
haematocrit of at least 30%, and
 β-blockers should be administered to reduce
myocardial consumption of oxygen.
 Among patients with aneurysms, prevention and, if
needed, treatment of coronary thrombosis are key
components of therapy.
 Patients with coronary arterial stenosis or occlusion
and evidence of reversible ischaemia are candidates
for interventional catheterisation and surgical
procedures.

TREATMENT Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Anti pyretic : 80 mg/kg/day  afebrile for
at least 48 hours.
 Anti platelet : lowered to 3 to 5 mg/kg/day
 Low doses is continued for 6 weeks,
discontinued  without aneurysms
 Coronary arterial abnormalities low dose
aspirin is continued, may be used with
other anti thrombotic therapies, such as
clopidogrel or warfarin

Aspirin Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Effective to control of inflammation 
decreases of aneurysmal formation
 Administered in the irst 10 days, and ideally
within the irst 7 days of illness.
 Beneficial for children beyond the tenth day
of illness (fever persists, or who have
coronary arterial abnormalities together
with persistent clinical and laboratory
evidence of inflammation).
 The standard dosage is 2 g/kg, administered
over 8 to 12 hours.
 Administering a second or third infusion have
persistent or recrudescent fever.

Intravenous Immunoglobulin Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Corticosteroid
 Corticosteroids are the mainstay of therapy for many
childhood vasculitides
 Methylprednisolone did not improve outcomes with
regard to coronary arterial
 Administered to the patient resistant to
intravenous immune globulin
Abciximab,
 Monoclonal antibody to the platelet glycoprotein
IIbIIIa receptor, controls to promote regression in
the maximal diameter of the aneurysms.
 vascular remodeling through the anti-inlammatory
effects
Infliximab,
 a monoclonal antibody to TnF-α, increasingly as
rescue therapy in resistant to immunoglobulins

Other Therapies Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Plasma exchange has been reported to
lower the incidence of aneurysms in
uncontrolled studies.
 Cytotoxic agents have been used to treat
refractory patients with acute disease

Other Therapies Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Small aneurysms are treated with aspirin in
doses sufficient to produce an anti-platelet
effect at the other end of the spectrum,
 Giant aneurysms, with a diameter of greater
than 8 mm, anticoagulation with warfarin or
LMWH
 Clopidogrel or anti coagulation may be added
to treatment with aspirin, depending upon
the individual situation.
 Important to consider the z scores

Prevention of Thrombosis Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Tissue plasminogen activator, at doses of
0.1 to 0.5 mg/kg/hr for 6 hours,
administered together with aspirin and
heparin or LMWH.
 Combination of tissue plasminogen
activator and abciximab, given as a
bolus of 0.25 mg/kg over 30 minutes,
followed by an infusion of 0.125
μg/kg/minute for 12 hours.

Treatment of Trombosis Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
Endogenous Antithrombotic
Pathophysiology of Heart Disease, Leonard S.
Lily, 5th Ed / 2011
Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Have not been assessed
 Should be considered in such patients
 Presentation with ischaemic symptoms, presence of
reversible ischaemia on stress testing, or presence of at
least 75% stenosis of the anterior interventricular
coronary artery.
Median follow-up of 3.6 years.
 For vessels in which angioplasty was used, the early
success rate was 86%. For rotational atherectomy, the
figure was 96%, and for placement of stents, 90%.
At the latest follow-up,
 Stenoses had recurred in 29% of arterial segments in
which angioplasty had been performed: in 28% of
segments following rotational atherectomy, and in 8% of
segments following placement of a stent.
 neoaneurysms were noted in 7% of segments treated with
angioplasty and rotational ablation, likely related to the
high pressure of inflation needed to relieve stenoses in stiff
coronary arteries.
Interventional Catheterisation Jane Newburger, Chapter 52 in
PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 Coronary arterial bypass surgery is performed with the
goal of improving symptoms of angina, enhancing
myocardial perfusion, and lowering the future risk of
myocardial infarction or sudden death.
 Children with reversible ischaemia on stress-imaging tests
are generally considered for intervention, be it
transcatheter or surgical.
 In addition, high-grade obstructions in at least two major
coronary arteries, or in the main stem of the left coronary
artery, are indications for surgery because these
findings predict a high risk of myocardial infarction.
 Cardiac transplantation can be performed in children
with Kawasaki disease who have end-stage ischaemic
cardiomyopathy in whom severe coronary arterial lesions
can not be treated further with interventional
catheterisation or coronary arterial bypass procedures.

Surgery Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
 all children should undergo periodic assessment and counseling
about known risk factors.
 all patients and families should be counseled regarding a
heart-healthy diet, the importance of exercise, and the risks of
smoking. They should be encouraged to limit adverse effects
from passive smoking, so a smoke-free home should be
advised.
 a lipid proile should be obtained approximately 1 year after
the onset of the illness. new recommendations from the
american Heart association have established the thresholds at
which pharmacologic management of hyperlipidaemia and
hypertension should be started, depending upon the severity
of coronary arterial disease.
 Paediatric cardiologists should strive to balance the need for
preventive counseling for exercise and a heart healthy diet
against the production of cardiac non-disease.
 Regular aerobic exercise should be strongly encouraged

LONG-TERM FOLLOW-UP Jane Newburger, Chapter 52 in

PAEDIATRIC CARDIOLOGY, 3rd
EDITION © 2010
American Heart Association,
Circulation 2004
Forever Until
The End