SYMPOSIUM: CARDIOVASCULAR

Dilated cardiomyopathy in improving with increasing expertise in cardiac transplantation,

and exciting new developments such as ventricular assist devices.

children Aetiology and investigations

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

PAEDIATRICS AND CHILD HEALTH 23:2 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 A number of other gene mutations can lead to a phenotype of
is identified within a family group. Several research projects are skeletal muscle weakness, cardiomyopathy and commonly
ongoing attempting to characterize and identify defects but arrhythmia. Lamin A/C gene mutations lead to limb-girdle
clinically the information is useful from a screening perspective muscular dystrophies and some EmeryeDreifuss muscular
and not important with respect to the management of patients. dystrophies (as well as an emerin gene mutation) both involving
The identification of an abnormal genotype in a patient with significant cardiomyopathy.
a normal cardiac phenotype may have wide ranging implications
without necessarily being of any benefit to the individual. Metabolic causes
Genetic testing in families with an abnormal gene identified and Inborn errors of metabolism (IEM) can lead to DCM, and this is
strongly linked to a cardiomyopathic disease may be useful in the underlying cause in around 4e16% of cases. These patients
identifying which patients need to continue with regular present at an earlier age on average than idiopathic cases, often
screening. Guidelines suggest that first-degree relatives of in infancy. Among DCM cases caused by an IEM, oxidative
patients with idiopathic DCM are screened on a 2 yearly basis, phosphorylation defects and systemic carnitine deficiency are the
however if a gene is identified and not present it is reasonable not most common underlying problems, accounting for 40% of
to continue to follow that patient in the clinic. metabolic cases. An oxidative phosphorylation disease that often
features DCM is Barth syndrome, an X-linked disorder of lipid
Dilated cardiomyopathy with skeletal myopathy metabolism. The mutation causing Barth syndrome results in an
X-linked disorders involving dystrophin gene mutations warrant abnormal form of cardiolipin, a lipid essential for normal mito-
specific mention in the context of dilated cardiomyopathy, chondrial metabolism. The syndrome can be identified by raised
Duchenne and Becker muscular dystrophies being the most levels 3-methylglutaconic acid in the urine, hence the urine
familiar. These disorders of both cardiac and skeletal muscle organic acid screen included in our investigation panel.
present with progressive muscle weakness from early childhood Other metabolic syndromes that can result in DCM include
and have devastating effects on both quality and duration of life, mucopolysaccharidosis type I (Hurler syndrome) and type VI
particularly in Duchenne where boys become non-ambulatory in (MaroteauxeLamy syndrome), glycogen storage disorder type IV
early adolescence and by the start of their third decade nearly all (Anderson disease), long-chain 3-hydroxyacyl-CoA dehydroge-
the boys affected will have changes suggestive of dilated nase deficiency, and mitochondrial disorders other than Barth
cardiomyopathy. The histopathology suggests fibro-fatty changes such as MERFF (myoclonic epilepsy with red-ragged fibres). Our
in the base and lateral aspects of the left ventricle which prog- investigation panel is aimed at identifying as many of these
resses to involve the remainder of the myocardium. This leads to underlying defects as possible.
significant ventricular dysfunction and eventually cardiac failure. It is important to recognize when a DCM case has an under-
Cardiac deterioration is becoming an increasing problem for lying metabolic cause and whilst only a small number of child-
patients and the clinicians caring for them. Patients are regularly ren present with this type of cardiomyopathy treatment may
receiving pulsed steroids to ameliorate the abnormalities of be possible, dramatically improving cardiac function. Barth
skeletal and respiratory muscle progression with good effect, syndrome is a good example of the importance of early identifi-
meaning that as individuals survive longer morbidity form cation of IEM in cases of DCM, as the disease responds well to
cardiac deterioration later in life becomes more significant. From medical management, and heart function often improves after
a management perspective drugs for cardiac dysfunction, mainly puberty. Many children with a metabolic aetiology to their
angiotensin-converting enzyme (ACE) inhibitors, have been used cardiomyopathy will present to the intensive care with a life
to improve cardiac function once echocardiographic appearances threatening condition usually as infants. They will have features
develop. The benefit of using prophylactic ACE inhibitors has suggestive of poor energy metabolism, including failure to thrive
been controversial but using an ACE inhibitor to reduce left and severe acidosis sometimes out of context with the severity of
ventricular wall stress when the safety profile of the drug is good cardiac dysfunction. An important rare reversible cause of
and the effects of the disease process so devastating does not cardiomyopathy in these patients may be identified by initial
seem unreasonable. A multi-centre international trial, including cardiomyopathy screening investigations. Of particular impor-
our own, is currently in the process of randomizing patients prior tance are disorders leading to a primary carnitine deficiency.
to the onset of echocardiographic features of cardiomyopathy to Defects in the OCTN2 carnitine transporter gene leads to failure
assess its benefit. of fatty acid transport across cellular membranes, resulting in
Becker muscular dystrophy is a less severe form both from a lack of substrate for cellular energy and accumulation of fat,
a skeletal and cardiac perspective and in some cases skeletal physically affecting the function of myocytes. This clearly has
muscle function can be preserved enough to consider cardiac important implications for decisions such as cardiac transplant.
transplantation if cardiac function should deteriorate. Cardiac
transplantation for boys with Duchenne muscular dystrophy is Other causes and investigations
not usually considered because of the co-morbidities involved. Vitamin D deficiency, as well other causes of hypocalcaemia
X-linked cardiomyopathy is a lesser known dystrophin gene such as primary hypoparathyroidism, is a rare but important
mutation disorder with poor outcome. The dystrophin gene cause of DCM. With the resurgence of clinically apparent rickets
mutation is isolated to the cardiac muscle and boys develop in recent years, this cause is increasing in incidence, and is
progressive cardiac dysfunction and arrhythmia in adolescence particularly amenable to medical treatment. Indeed, vitamin D
and young adulthood with death from cardiac failure unless supplementation in some patients has been shown to normalize
cardiac transplantation can be performed. ventricular function, hence the early identification of deficiency

PAEDIATRICS AND CHILD HEALTH 23:2 61 Ó 2012 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: CARDIOVASCULAR

is vital. Deficiencies in other micronutrients such as selenium failure is associated with activation of the renineangiotensine
and thiamine can also result in a reduction in cardiac function, aldosterone system, an attempt by the body to maintain adequate
therefore these are also included on our investigation panel. systemic perfusion pressures in the face of falling cardiac output.
Anaemia, whether caused by dietary insufficiency or inherited Although this may have immediate beneficial effects, evidence in
haemoglobinopathies, can result in a high cardiac-output state. adults shows that the longer term effects may exacerbate
In some circumstances this increase in myocardial workload can myocardial damage and worsen heart failure. By reducing
result in DCM, and so simple tests such as a full blood count systemic vascular resistance, ACEi improve both stroke volume
should not be omitted. and cardiac output. Small-scale studies have shown that this can
An electrocardiogram (ECG) should be performed on all patients translate into clinical improvement, and that ACEi are safe in the
presenting with DCM. Resting ECG usually shows a sinus tachy- majority of children. Despite recommendations that all children
cardia with possible signs of atrial enlargement and ventricular with ventricular dysfunction should be started on ACEi therapy,
hypertrophy. A 24-hour ECG recording should also be performed, studies have shown that only 66e70% of suitable children
as arrhythmias may be a cause, as well as a consequence, of DCM. receive these drugs.
Sustained disturbances of rhythm may result in alterations of flow Diuretics are in widespread use in paediatric heart failure
dynamics through the heart, resulting in ventricular dysfunction patients, despite little trial evidence of either clinical improvement
and ultimately DCM. The alteration in cardiac morphology caused or long-term benefit. Guidelines for adult patients recommend the
by DCM can also result in secondary arrhythmias that may further use of diuretics to control fluid overload, and there are specific
lower cardiac output. In either case, prompt identification may lead long-term benefits of the use of the aldosterone-antagonist spi-
to anti-arrhythmic therapy that may improve ventricular function. ronolactone. The mechanism of this survival benefit is thought to
A further use of ECG in DCM is in the identification of anomalous be distinct from its diuretic effect, and for this reason we prefer-
origin of the left coronary artery from the pulmonary artery entially use spironolactone over other potassium-sparing diuretics
(ALCAPA), a rare condition that can often go unrecognized, even in heart failure patients in our institution.
until adulthood. In this condition, the myocardium perfused by the Beta-blocker use has become ubiquitous amongst adult heart
left coronary artery becomes increasingly ischaemic with resulting failure patients, but rates of use in children remain low. Similarly
wall motion abnormalities and eventually global hypokinesia. An to ACEi, the mechanism of beta-blockers involves counteracting
ECG may show deep Q waves in lead I and wide Q waves in lead the body’s natural adrenergic response to a poor cardiac-output
AVL, so helping the diagnosis. state, resulting in a reduction in cardiomyocyte damage. Large
ALCAPA can also be diagnosed on echocardiography, an scale multi-centre trials have shown significant improvements in
investigation that is perhaps the most important in DCM patients. mortality in adults. Unsurprisingly, the evidence base in children
Echocardiography forms the basis of the DCM diagnosis by is much more limited, but several smaller trials have shown
defining and quantifying the extent of left ventricular dilatation beneficial effects of beta-blockade on both echocardiographic
and dysfunction. In addition, echocardiography allows the parameters and heart failure symptoms. Despite this, one US
exclusion of structural abnormalities that can lead to DCM and study found that beta-blockers were only used in 6% of paedi-
are potentially correctable, such as ALCAPA, valve disease, and atric cases of DCM.
other congenital anomalies. Digoxin has positive inotropic and negative chronotropic
An array of other medical conditions can also lead to DCM. actions, and is used in heart failure with the aim of increasing
Thyroid function tests should be performed as both hyper- and cardiac output thus ameliorating symptoms. It has been shown to
hypothyroidism can lead to severe myocardial dysfunction. improve symptoms in adults, but there is no evidence of
Systemic autoimmune disease including rheumatoid arthritis and a reduction in mortality. There is even less evidence for its use in
systemic lupus erythematosus (SLE) can be associated with children, but despite this it is commonly prescribed. However
cardiomyopathy, and we screen for these with serum autoanti- despite the polarized opinions that digoxin produces amongst
bodies. Although unlikely in the paediatric setting, excess alcohol cardiologists the tide of opinion seems to be turning. Heart rate
and use of cocaine can both result in cardiomyopathy. There are reduction in cardiac failure is becoming more en vogue high-
also iatrogenic causes of DCM, especially anthracycline-based lighted by the beneficial effects that Ivabradine has had for
chemotherapy, and serial echocardiograms should be per- patients with ischaemic heart disease. Anecdotal evidence from
formed on all children at risk. 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
Management
dose for heart rate reduction enables oral therapy to be escalated
Medical therapy without the toxicity that may otherwise tarnish its reputation.
Compared to the adult literature, studies on children with heart Attention needs to be focussed on the potential for cardiac
failure of any cause are limited in both number and size, and arrhythmias in relation to high potassium levels when other
there are relatively little data on the effect of most medical agents causing hyperkalaemia are used in conjunction.
therapies on long-term outcomes such as mortality. Because of
this weak evidence base, the medical management strategy for Resynchronization
treating children with DCM has largely been drawn from expe- Biventricular pacing has been shown to improve outcomes in
rience in treating adults with heart failure. adult heart failure patients. By improving the synchronous
Angiotensin-converting enzyme inhibitors (ACEi) are now contraction of both the left ventricular free wall and inter-
widely used in the treatment of paediatric heart failure. Heart ventricular septum, so-called ‘resynchronization therapy’ aims

PAEDIATRICS AND CHILD HEALTH 23:2 62 Ó 2012 Elsevier Ltd. All rights reserved.
 SYMPOSIUM: CARDIOVASCULAR

to optimize left ventricular function and maximize cardiac Burch M, Aurora P. Current status of paediatric heart, lung, and heart-lung
output. Paediatric DCM patients are known to have high rates of transplantation. Arch Dis Child 2004; 89: 386e9.
mechanical dyssynchrony, and so may benefit from pacemaker Cox GF. Diagnostic approaches to pediatric cardiomyopathy of metabolic
insertion. Results of clinical trials of resynchronization in DCM genetic etiologies and their relation to therapy. Prog Pediatr Cardiol
have so far been mixed, although it has found to have resulted in 2007; 24: 15e25.
marked clinical improvement for many patients. Dadlani GH, Harmon WG, Lipshultz SE. Dilated cardiomyopathy. In:
Chang AC, Towbin JA, eds. Heart failure in children and young adults.
Ventricular assist devices Philadelphia: Saunders Elsevier, 2006; 248e263.
Ventricular assist devices (VADs), such as the Berlin Heart Daubeney PE, Nugent AW, Chondros P, et al. Clinical features and
EXCOR device, are now part of routine clinical practice in many outcomes of childhood dilated cardiomyopathy: results from
centres. At our centre, DCM is the most common indication for a national population-based study. Circulation 2006; 114:
VAD insertion, and many patients have been successfully 2671e8.
bridged to cardiac transplant using the device. Recent data Harmon WG, Sleeper LA, Cuniberti L, et al. Treating children with idio-
indicate that worldwide around 69% of idiopathic cardiomyop- pathic dilated cardiomyopathy (from the pediatric cardiomyopathy
athy patients treated with a Berlin heart device were trans- registry). Am J Cardiol 2009; 104: 281e6.
planted, 24% died whilst on the device, and 8% were Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric
successfully weaned off the device after significant myocardial cardiomyopathy in two regions of the United States. N Engl J Med
recovery. Use of the Berlin Heart is not without risks however, 2003; 348: 1647e55.
and the patients are at greatly increased risk of haemorrhage Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of child-
(especially intracranial), thrombosis, and nosocomial infection. hood cardiomyopathy in Australia. N Engl J Med 2003; 348: 1639e46.
Richardson P, McKenna W, Bristow M, et al. Report of the 1995 world
Transplantation health organization/international society and federation of cardiology
The first paediatric heart transplant was performed over 40 years task force on the definition and classification of cardiomyopathies.
ago, and currently around 350 are performed each year world- Circulation 1996 Mar 1; 93: 841e2.
wide. DCM is the most common indication for paediatric heart Sharma MS, Forbess JM, Guleserian KJ. Ventricular assist device support in
transplant, and the outcome is favourable compared to trans- children and adolescents with heart failure: the children’s medical
plants performed for other indications. Analysis of our institu- center of Dallas experience. Artif Organs 2012; 36: 635e9.
tional data demonstrates that for those patients surviving beyond Silva JN, Canter CE. Current management of pediatric dilated cardiomy-
a year, median survival is approaching 20 years after transplant. opathy. Curr Opin Cardiol 2010; 25: 80e7.
Thomas DE, Wheeler R, Yousef ZR, Masani ND. The role of echocardiog-
Clinical approach to management raphy in guiding management in dilated cardiomyopathy. Eur J Echo-
Cardiomyopathy in children remains a rare clinical problem and cardiogr 2009; 10: iii15e21.
requires expertise to identify reversible causes amenable to Towbin JA, Lowe AM, Colan SD, et al. Incidence, causes, and outcomes of
treatment. Within our institution at Great Ormond Street dilated cardiomyopathy in children. JAMA 2006; 296: 1867e76.
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
Practice points
support and cardiac transplantation when required. The aim of
this integrated service is to focus expertise in one location and C DCM is uncommon, but is the most common disease of heart
support families as well as provide a research resource to
muscle in children, and the most common indication for
improve outcomes for this difficult group of patients. A cardiac transplantation
C The causes are varied, but potentially reversible, therefore all
patients with DCM should be extensively investigated at first
FURTHER READING presentation
Andrews RE, Fenton MJ, Ridout DA, Burch M. New-onset heart failure due C Treatment consists of medical therapy, cardiac resynchroniza-
to heart muscle disease in childhood: a prospective study in the tion (in selected cases), mechanical ventricular support, and
United Kingdom and Ireland. Circulation 2008; 117: 79e84. cardiac transplantation
Arora G, Friedman R. Cardiac resynchronisation therapy and heart failure. C Recent data suggest that the median life expectancy after
In: Chang AC, Towbin J, eds. Heart failure in children and young adults. a cardiac transplant is around 20 years
Philadelphia: Saunders Elsevier, 2006; 552e562.

PAEDIATRICS AND CHILD HEALTH 23:2 63 Ó 2012 Elsevier Ltd. All rights reserved.