SYMPOSIUM: CARDIOVASCULAR
Dilated cardiomyopathy in
children
Thomas G Day
Matthew Fenton
Abstract
Dilated cardiomyopathy (DCM) is the most common paediatric heart
muscle disease, and the most common indication for cardiac transplanta-
tion in this age group. In terms of aetiology, DCM is a heterogeneous
condition, with infectious, inflammatory, metabolic and genetic causes,
although the precise pathogenesis remains unknown in most patients.
Because some of the causes of DCM are potentially reversible, an exten-
sive panel of investigations should be performed at first presentation,
which we describe here. Treatment usually begins with pharmacological
therapy, including beta-blockers, ACE inhibitors, digoxin, and diuretics.
Cardiac transplantation and the recently introduced mechanical ventricular
assist devices are alternatives when medical management is not effective.
Keywords cardiomyopathy; heart failure; myocarditis; paediatrics;
transplantation
Introduction
Dilated cardiomyopathy (DCM) is the most common form of
heart muscle disease in children. Rather than a single disease
entity, DCM can be viewed as a heterogeneous mix of conditions,
all of which share a common phenotype of left ventricular dila-
tation and systolic dysfunction, with or without right ventricular
involvement. DCM results in a substantial degree of morbidity
and mortality, and it is the commonest reason for paediatric
cardiac transplantation outside of infancy.
DCM is an uncommon condition, with an estimated annual
incidence rate of between 0.34 and 0.73 per 100,000 children. It
appears to be more common in boys than girls, probably due to
a subset of DCM caused by X-linked conditions such as the
muscular dystrophies. DCM is also more common in non-white
populations, and in younger age-groups (especially the under-
1s) although it can occur at any age including adolescence.
The most common presentation of DCM is with signs and
symptoms of overt cardiac failure. In infants, this will usually
manifest as feeding difficulties, whereas older children will
usually report a reduction in exercise tolerance, dyspnoea, or
oedema. As discussed below, the outcome can be poor but is
Thomas G Day MBChB MRes MRCPCH is Academic Clinical Fellow in the
Department of Cardiology, Great Ormond Street Hospital, London, UK.
Conflicts of interest: none declared.
Matthew Fenton MB BS BSc MRCPCH is Consultant Cardiologist in the
Department of Cardiology, Great Ormond Street Hospital, London, UK.
Conflicts of interest: none declared.
PAEDIATRICS AND CHILD HEALTH 23:2
improving with increasing expertise in cardiac transplantation,
and exciting new developments such as ventricular assist devices.
Aetiology and investigations
Although the aetiology of the majority of DCM cases remains
unknown even after extensive investigation, it is important to
thoroughly exclude potentially reversible causes of the DCM
phenotype. Studies estimate that a definitive cause can be found
in around 30e40% of DCM cases. Table 1 outlines most of the
possible underlying aetiologies, with the remainder being clas-
sified as idiopathic DCM. This is a diagnosis of exclusion, and at
first presentation an extensive of panel of investigations should
be performed, with the aim of identifying the conditions outlined
below. The panel of investigations performed at our institution is
shown in Table 2.
Myocarditis
In patients with DCM of known cause, the most common
underlying pathogenesis is infectious myocarditis. In the devel-
oped world, viruses are the most common causative agents of
myocarditis, particularly adenoviruses and enteroviruses such as
coxsackieviruses, parvovirus, and echovirus. Worldwide, the
most common pathogen is Chagas disease (Trypanosoma cruzi),
although bacterial and fungal forms have also been reported. The
pathogenic mechanism of viral myocarditis remains open to
debate, but possible theories include cardiac myocyte destruction
by circulating autoantibodies triggered by viral infection, or the
destruction of infected cardiac myocytes by circulating cytotoxic
lymphocytes, as well as direct viral-induced cell damage.
Myocarditis often presents a diagnostic dilemma to clinicians
as it is difficult to confirm with confidence. Some centres perform
endomyocardial biopsy looking for lymphocytic infiltration of the
myocardium and myocytolysis (the Dallas criteria for myocar-
ditis). This approach has several limitations however: it exposes
the patient to the risks of a general anaesthesia often in the
context of cardiac failure, and is an invasive procedure involving
tissue collection from a ventricular wall that is likely to be thin.
In addition, at a histological level the inflammatory process is
often patchy, and so a seemingly normal area of tissue may be
unwittingly sampled. The current practice in UK cardiac centres
is to avoid biopsy for this indication, as it is felt the risk/benefit
ratio does not support it.
Our investigation panel includes non-specific inflammatory
markers, virology PCR and serology (Table 2). These, coupled
with the presence or absence of recent infectious symptoms will
aid the clinician in deciding whether an infectious aetiology is
likely for an individual patient. This may have important impli-
cations for both treatment and prognosis, as outlined below.
Left ventricular non-compaction cardiomyopathy (LVNC)
LVNC has been defined by the American Heart Association as
a congenital cardiomyopathy characterized by a spongy appear-
ance to the left ventricle. The appearance is most notable
towards the lateral wall and apex of the left ventricle and is
believed to be related to in utero arrest of normal myocardial
compaction. LVNC commonly occurs in conjunction with other
cardiac structural disorders, mainly atrial and ventricular septal
defects. Multiple genetic mutations have been reported and
59 Ó 2012 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: CARDIOVASCULAR

Secondary causes of dilated cardiomyopathy Investigation panel at first presentation of DCM

Infections Ischaemia Echocardiogram Bloods
Viral Hypoxia Electrocardiogram, Full blood count
Bacterial Birth asphyxia including 24-hour tape Erythrocyte sedimentation rate
Fungal Drowning Chest radiograph Vacuolated lymphocytes
Protozoan (Chagas, Kawasaki disease Urine Urea and electrolytes
toxoplasmosis) Coronary artery Organic acids Liver function tests
Rickettsial (Rocky malformation (ALCAPA) Glycosaminoglycans C-reactive protein
Mountain spotted fever) Premature coronary Brain natriuretic peptide
Spirochetal (Lyme disease) artery disease Blood group and save
Lactate
Arrhythmias Toxins Ammonia
Supraventricular tachycardia Anthracyclines Cholesterol and triglycerides
(atrial flutter, ectopic atrial Radiation Thyroid function tests
tachycardia) Other chemotherapeutic Thiamine
Ventricular tachycardia agents Selenium
Bradycardia Sulfonamide sensitivity Red blood cell transketolase
Penicillin sensitivity Carnitine
Endocrine Iron (haemochromatosis) Acyl carnitine profile
Hyper/hypothyroidism Copper (Wilson’s disease) Red cell folate
Infant of a diabetic mother Transferrin
Catecholamine excess Systemic disorders Mitochondrial DNA
(phaeochromocytoma or Systemic lupus Plasma amino acids
neuroblastoma) erythematosus Antinuclear and anti-DNA antibodies
Congenital adrenal hyperplasia Juvenile idiopathic Viral PCR, IgM and IgI for enterovirus,
arthritis EpsteineBarr virus, adenovirus,
Storage disease Polyarteritis nodosa cytomegalovirus, parvovirus,
Glycogen storage diseases Osteogenesis imperfect coxsackievirus, echovirus
Mucopolysaccaridoses Noonan syndrome Ionized calcium
Sphingolipidoses Peripartum Parathyroid hormone
cardiomyopathy Vitamin D
Nutritional deficiencies Haemolytic uraemia
Protein (kwashiorkor) syndrome Table 2
Thiamine (beriberi) Leukaemia
absence of symptoms may make cardiac transplantation more
Vitamin E Amyloidosis
risky or even impossible.
Vitamin D Sarcoidosis
Selenium Reye syndrome Genetic and familial causes
Carnitine There are over 40 genes that have been identified as having
Phosphate a causative role in DCM. Around 30e40% of DCM cases are
From Dadlani GH, Harmon WG, Lipshultz SE. Dilated cardiomyopathy. In:
considered familial, defined as at least one first-degree relative
Chang AC, Towbin JA, eds. Heart failure in children and young adults. Phila- having DCM or sudden cardiac death at a young age. The most
delphia: Saunders Elsevier; 2006: 248e263. common inheritance mode is autosomal dominant, often with
incomplete penetrance, but X-linked, autosomal recessive, and
Table 1 mitochondrial forms of DCM have all been described. The
identification of familial cases of DCM is important, as it allows
crossover between genes causing dilated, hypertrophic and close follow-up of potentially affected siblings and other family
restrictive cardiomyopathy is common. As a relatively newly members. It has been shown that seemingly unaffected relatives
defined condition outcomes are being evaluated. In a recent can show echocardiographic changes before becoming symp-
review from Zuckerman and colleagues of 50 patients from their tomatic, and also that circulating cardiac autoantibodies can
institution, 26 patients had either died or been transplanted predict the development of disease in this population. Hence
within a year from presentation. Patients who survive beyond therapeutic intervention before the development of clinically
a year have significantly better outcome. Within our institution apparent heart failure may often be possible.
a number of patients with LVNC have diastolic dysfunction and Clearly if the causal mutation can be identified, the risk
the subsequent risk of developing raised pulmonary vascular assessment of family members will become far easier. Despite
resistance, a pathophysiological process similar to restrictive the number of identified genetic defects increasing over the
cardiomyopathy. This process requires careful monitoring as years, routine genetic testing is not currently performed in the
a significant increase in pulmonary vascular resistance in the paediatric clinic in index cases. Family screening with

PAEDIATRICS AND CHILD HEALTH 23:2 60 Ó 2012 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: CARDIOVASCULAR
echocardiography and ECG are recommended when a new case
is identified within a family group. Several research projects are
ongoing attempting to characterize and identify defects but
clinically the information is useful from a screening perspective
and not important with respect to the management of patients.
The identification of an abnormal genotype in a patient with
a normal cardiac phenotype may have wide ranging implications
without necessarily being of any benefit to the individual.
Genetic testing in families with an abnormal gene identified and
strongly linked to a cardiomyopathic disease may be useful in
identifying which patients need to continue with regular
screening. Guidelines suggest that first-degree relatives of
patients with idiopathic DCM are screened on a 2 yearly basis,
however if a gene is identified and not present it is reasonable not
to continue to follow that patient in the clinic.
Dilated cardiomyopathy with skeletal myopathy
X-linked disorders involving dystrophin gene mutations warrant
specific mention in the context of dilated cardiomyopathy,
Duchenne and Becker muscular dystrophies being the most
familiar. These disorders of both cardiac and skeletal muscle
present with progressive muscle weakness from early childhood
and have devastating effects on both quality and duration of life,
particularly in Duchenne where boys become non-ambulatory in
early adolescence and by the start of their third decade nearly all
the boys affected will have changes suggestive of dilated
cardiomyopathy. The histopathology suggests fibro-fatty changes
in the base and lateral aspects of the left ventricle which prog-
resses to involve the remainder of the myocardium. This leads to
significant ventricular dysfunction and eventually cardiac failure.
Cardiac deterioration is becoming an increasing problem for
patients and the clinicians caring for them. Patients are regularly
receiving pulsed steroids to ameliorate the abnormalities of
skeletal and respiratory muscle progression with good effect,
meaning that as individuals survive longer morbidity form
cardiac deterioration later in life becomes more significant. From
a management perspective drugs for cardiac dysfunction, mainly
angiotensin-converting enzyme (ACE) inhibitors, have been used
to improve cardiac function once echocardiographic appearances
develop. The benefit of using prophylactic ACE inhibitors has
been controversial but using an ACE inhibitor to reduce left
ventricular wall stress when the safety profile of the drug is good
and the effects of the disease process so devastating does not
seem unreasonable. A multi-centre international trial, including
our own, is currently in the process of randomizing patients prior
to the onset of echocardiographic features of cardiomyopathy to
assess its benefit.
Becker muscular dystrophy is a less severe form both from
a skeletal and cardiac perspective and in some cases skeletal
muscle function can be preserved enough to consider cardiac
transplantation if cardiac function should deteriorate. Cardiac
transplantation for boys with Duchenne muscular dystrophy is
not usually considered because of the co-morbidities involved.
X-linked cardiomyopathy is a lesser known dystrophin gene
mutation disorder with poor outcome. The dystrophin gene
mutation is isolated to the cardiac muscle and boys develop
progressive cardiac dysfunction and arrhythmia in adolescence
and young adulthood with death from cardiac failure unless
cardiac transplantation can be performed.
PAEDIATRICS AND CHILD HEALTH 23:2
A number of other gene mutations can lead to a phenotype of
skeletal muscle weakness, cardiomyopathy and commonly
arrhythmia. Lamin A/C gene mutations lead to limb-girdle
muscular dystrophies and some EmeryeDreifuss muscular
dystrophies (as well as an emerin gene mutation) both involving
significant cardiomyopathy.
Metabolic causes
Inborn errors of metabolism (IEM) can lead to DCM, and this is
the underlying cause in around 4e16% of cases. These patients
present at an earlier age on average than idiopathic cases, often
in infancy. Among DCM cases caused by an IEM, oxidative
phosphorylation defects and systemic carnitine deficiency are the
most common underlying problems, accounting for 40% of
metabolic cases. An oxidative phosphorylation disease that often
features DCM is Barth syndrome, an X-linked disorder of lipid
metabolism. The mutation causing Barth syndrome results in an
abnormal form of cardiolipin, a lipid essential for normal mito-
chondrial metabolism. The syndrome can be identified by raised
levels 3-methylglutaconic acid in the urine, hence the urine
organic acid screen included in our investigation panel.
Other metabolic syndromes that can result in DCM include
mucopolysaccharidosis type I (Hurler syndrome) and type VI
(MaroteauxeLamy syndrome), glycogen storage disorder type IV
(Anderson disease), long-chain 3-hydroxyacyl-CoA dehydroge-
nase deficiency, and mitochondrial disorders other than Barth
such as MERFF (myoclonic epilepsy with red-ragged fibres). Our
investigation panel is aimed at identifying as many of these
underlying defects as possible.
It is important to recognize when a DCM case has an under-
lying metabolic cause and whilst only a small number of child-
ren present with this type of cardiomyopathy treatment may
be possible, dramatically improving cardiac function. Barth
syndrome is a good example of the importance of early identifi-
cation of IEM in cases of DCM, as the disease responds well to
medical management, and heart function often improves after
puberty. Many children with a metabolic aetiology to their
cardiomyopathy will present to the intensive care with a life
threatening condition usually as infants. They will have features
suggestive of poor energy metabolism, including failure to thrive
and severe acidosis sometimes out of context with the severity of
cardiac dysfunction. An important rare reversible cause of
cardiomyopathy in these patients may be identified by initial
cardiomyopathy screening investigations. Of particular impor-
tance are disorders leading to a primary carnitine deficiency.
Defects in the OCTN2 carnitine transporter gene leads to failure
of fatty acid transport across cellular membranes, resulting in
a lack of substrate for cellular energy and accumulation of fat,
physically affecting the function of myocytes. This clearly has
important implications for decisions such as cardiac transplant.
Other causes and investigations
Vitamin D deficiency, as well other causes of hypocalcaemia
such as primary hypoparathyroidism, is a rare but important
cause of DCM. With the resurgence of clinically apparent rickets
in recent years, this cause is increasing in incidence, and is
particularly amenable to medical treatment. Indeed, vitamin D
supplementation in some patients has been shown to normalize
ventricular function, hence the early identification of deficiency
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 SYMPOSIUM: CARDIOVASCULAR
is vital. Deficiencies in other micronutrients such as selenium
and thiamine can also result in a reduction in cardiac function,
therefore these are also included on our investigation panel.
Anaemia, whether caused by dietary insufficiency or inherited
haemoglobinopathies, can result in a high cardiac-output state.
In some circumstances this increase in myocardial workload can
result in DCM, and so simple tests such as a full blood count
should not be omitted.
An electrocardiogram (ECG) should be performed on all patients
presenting with DCM. Resting ECG usually shows a sinus tachy-
cardia with possible signs of atrial enlargement and ventricular
hypertrophy. A 24-hour ECG recording should also be performed,
as arrhythmias may be a cause, as well as a consequence, of DCM.
Sustained disturbances of rhythm may result in alterations of flow
dynamics through the heart, resulting in ventricular dysfunction
and ultimately DCM. The alteration in cardiac morphology caused
by DCM can also result in secondary arrhythmias that may further
lower cardiac output. In either case, prompt identification may lead
to anti-arrhythmic therapy that may improve ventricular function.
A further use of ECG in DCM is in the identification of anomalous
origin of the left coronary artery from the pulmonary artery
(ALCAPA), a rare condition that can often go unrecognized, even
until adulthood. In this condition, the myocardium perfused by the
left coronary artery becomes increasingly ischaemic with resulting
wall motion abnormalities and eventually global hypokinesia. An
ECG may show deep Q waves in lead I and wide Q waves in lead
AVL, so helping the diagnosis.
ALCAPA can also be diagnosed on echocardiography, an
investigation that is perhaps the most important in DCM patients.
Echocardiography forms the basis of the DCM diagnosis by
defining and quantifying the extent of left ventricular dilatation
and dysfunction. In addition, echocardiography allows the
exclusion of structural abnormalities that can lead to DCM and
are potentially correctable, such as ALCAPA, valve disease, and
other congenital anomalies.
An array of other medical conditions can also lead to DCM.
Thyroid function tests should be performed as both hyper- and
hypothyroidism can lead to severe myocardial dysfunction.
Systemic autoimmune disease including rheumatoid arthritis and
systemic lupus erythematosus (SLE) can be associated with
cardiomyopathy, and we screen for these with serum autoanti-
bodies. Although unlikely in the paediatric setting, excess alcohol
and use of cocaine can both result in cardiomyopathy. There are
also iatrogenic causes of DCM, especially anthracycline-based
chemotherapy, and serial echocardiograms should be per-
formed on all children at risk.
Management
Medical therapy
Compared to the adult literature, studies on children with heart
failure of any cause are limited in both number and size, and
there are relatively little data on the effect of most medical
therapies on long-term outcomes such as mortality. Because of
this weak evidence base, the medical management strategy for
treating children with DCM has largely been drawn from expe-
rience in treating adults with heart failure.
Angiotensin-converting enzyme inhibitors (ACEi) are now
widely used in the treatment of paediatric heart failure. Heart
PAEDIATRICS AND CHILD HEALTH 23:2
failure is associated with activation of the renineangiotensine
aldosterone system, an attempt by the body to maintain adequate
systemic perfusion pressures in the face of falling cardiac output.
Although this may have immediate beneficial effects, evidence in
adults shows that the longer term effects may exacerbate
myocardial damage and worsen heart failure. By reducing
systemic vascular resistance, ACEi improve both stroke volume
and cardiac output. Small-scale studies have shown that this can
translate into clinical improvement, and that ACEi are safe in the
majority of children. Despite recommendations that all children
with ventricular dysfunction should be started on ACEi therapy,
studies have shown that only 66e70% of suitable children
receive these drugs.
Diuretics are in widespread use in paediatric heart failure
patients, despite little trial evidence of either clinical improvement
or long-term benefit. Guidelines for adult patients recommend the
use of diuretics to control fluid overload, and there are specific
long-term benefits of the use of the aldosterone-antagonist spi-
ronolactone. The mechanism of this survival benefit is thought to
be distinct from its diuretic effect, and for this reason we prefer-
entially use spironolactone over other potassium-sparing diuretics
in heart failure patients in our institution.
Beta-blocker use has become ubiquitous amongst adult heart
failure patients, but rates of use in children remain low. Similarly
to ACEi, the mechanism of beta-blockers involves counteracting
the body’s natural adrenergic response to a poor cardiac-output
state, resulting in a reduction in cardiomyocyte damage. Large
scale multi-centre trials have shown significant improvements in
mortality in adults. Unsurprisingly, the evidence base in children
is much more limited, but several smaller trials have shown
beneficial effects of beta-blockade on both echocardiographic
parameters and heart failure symptoms. Despite this, one US
study found that beta-blockers were only used in 6% of paedi-
atric cases of DCM.
Digoxin has positive inotropic and negative chronotropic
actions, and is used in heart failure with the aim of increasing
cardiac output thus ameliorating symptoms. It has been shown to
improve symptoms in adults, but there is no evidence of
a reduction in mortality. There is even less evidence for its use in
children, but despite this it is commonly prescribed. However
despite the polarized opinions that digoxin produces amongst
cardiologists the tide of opinion seems to be turning. Heart rate
reduction in cardiac failure is becoming more en vogue high-
lighted by the beneficial effects that Ivabradine has had for
patients with ischaemic heart disease. Anecdotal evidence from
within our own institution supports the benefit of using digoxin
in patients with severe ventricular dysfunction when weaning
inotropes and establishing oral medications. Digoxin used in low
dose for heart rate reduction enables oral therapy to be escalated
without the toxicity that may otherwise tarnish its reputation.
Attention needs to be focussed on the potential for cardiac
arrhythmias in relation to high potassium levels when other
agents causing hyperkalaemia are used in conjunction.
Resynchronization
Biventricular pacing has been shown to improve outcomes in
adult heart failure patients. By improving the synchronous
contraction of both the left ventricular free wall and inter-
ventricular septum, so-called ‘resynchronization therapy’ aims
62 Ó 2012 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: CARDIOVASCULAR
to optimize left ventricular function and maximize cardiac
output. Paediatric DCM patients are known to have high rates of
mechanical dyssynchrony, and so may benefit from pacemaker
insertion. Results of clinical trials of resynchronization in DCM
have so far been mixed, although it has found to have resulted in
marked clinical improvement for many patients.
Ventricular assist devices
Ventricular assist devices (VADs), such as the Berlin Heart
EXCOR device, are now part of routine clinical practice in many
centres. At our centre, DCM is the most common indication for
VAD insertion, and many patients have been successfully
bridged to cardiac transplant using the device. Recent data
indicate that worldwide around 69% of idiopathic cardiomyop-
athy patients treated with a Berlin heart device were trans-
planted, 24% died whilst on the device, and 8% were
successfully weaned off the device after significant myocardial
recovery. Use of the Berlin Heart is not without risks however,
and the patients are at greatly increased risk of haemorrhage
(especially intracranial), thrombosis, and nosocomial infection.
Transplantation
The first paediatric heart transplant was performed over 40 years
ago, and currently around 350 are performed each year world-
wide. DCM is the most common indication for paediatric heart
transplant, and the outcome is favourable compared to trans-
plants performed for other indications. Analysis of our institu-
tional data demonstrates that for those patients surviving beyond
a year, median survival is approaching 20 years after transplant.
Clinical approach to management
Cardiomyopathy in children remains a rare clinical problem and
requires expertise to identify reversible causes amenable to
treatment. Within our institution at Great Ormond Street
Hospital, London we are fortunate to have dedicated resources to
manage children not only with dilated cardiomyopathy but also
other types of heart muscle dysfunction. Clinical treatment for
cardiomyopathy requires dedicated outpatient services with the
resources to assess patients and screen relatives on a regular
basis as well as expert intensive care and access to mechanical
support and cardiac transplantation when required. The aim of
this integrated service is to focus expertise in one location and
support families as well as provide a research resource to
improve outcomes for this difficult group of patients. A cardiac transplantation
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Practice points
C DCM is uncommon, but is the most common disease of heart
muscle in children, and the most common indication for
C The causes are varied, but potentially reversible, therefore all
patients with DCM should be extensively investigated at first
presentation
C Treatment consists of medical therapy, cardiac resynchroniza-
tion (in selected cases), mechanical ventricular support, and
cardiac transplantation
C Recent data suggest that the median life expectancy after
a cardiac transplant is around 20 years
63 Ó 2012 Elsevier Ltd. All rights reserved.