eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Cardiomyopathy, Dilated

Author: Poothirikovil Venugopalan, MBBS, MD, FRCP (Glasg), FRCPCH, Consulting Staff, Department of Child Health, University Hospital of Hartlepool, UK
Contributor Information and Disclosures

Updated: Nov 12, 2008

Introduction

Background

Idiopathic dilated cardiomyopathy (DCM) refers to congestive cardiac failure secondary to dilatation and systolic dysfunction (with or without diastolic dysfunction) of the ventricles (predominantly left) in the absence of congenital, valvular, or coronary artery disease or any systemic disease known to cause myocardial dysfunction. DCM is the most common type of heart muscle disease in children.

All 4 cardiac chambers are dilated and are sometimes hypertrophied. Dilation is more pronounced than hypertrophy, and the left ventricle is affected more often than the right ventricle. The cardiac valves are intrinsically normal, although the mitral and tricuspid valve rings are dilated, and the valve leaflets do not appose each other in systole, giving rise to varying degrees of mitral regurgitation, tricuspid regurgitation, or both. Persistent mitral regurgitation leads to thickening of the mitral valve leaflets, and, at times, distinguishing this thickening from other causes of mitral regurgitation is difficult. Thrombus formation (secondary to the low-flow cardiac output state) is often seen in the left ventricular apex and, at times, is seen in the atria. Occasionally, the right ventricle is preferentially involved in the cardiomyopathic process; this often indicates a familial basis.

Pathophysiology

Injury to the myocardial cell is the initiating factor that leads to cell death. If considerable cell loss occurs, the myocardium fails to generate enough contractile force to produce adequate cardiac output. This results in the activation of compensatory mechanisms, including the renin-angiotensin-aldosterone system, sympathetic stimulation, antidiuretic hormone production, release of atrial natriuretic peptide, tumor necrosis factor (TNF)-a, and mechanical factors, such as increased end-diastolic stretch on the ventricle. These compensatory mechanisms help to maintain cardiac output in the initial phase; however, as myocardial damage progresses, persistent and excessive activation can be detrimental to cardiac function, leading to overt congestive heart failure.

The theory that left ventricular noncompaction is an underlying factor in the development of DCM in young infants has received much attention.1

Over-stretching of the ventricles causes myocardial thinning, cavity dilation, secondary valvular regurgitation, and compromised myocardial perfusion. The resulting subendocardial ischemia perpetuates myocyte damage.

Myocardial remodeling is an important contributor to worsening heart failure. Lost myocyte cells are replaced with fibrous tissue, thereby decreasing the compliance of one or more ventricles and adversely affecting performance. Aldosterone, angiotensin II, catecholamines, endothelins, and mechanical factors, such as excessive myocardial stretch and ischemia, have been identified as mediators of remodeling.

Apoptosis is a process of programmed cell death and is now believed to play a role in the continuing loss of myocardial cells in chronic heart failure. Overloading of myocytes possibly triggers apoptosis without fibrosis.

Heightened peripheral vasoconstriction, abnormal and excessive remodeling of the peripheral vasculature, and abnormalities in endothelium-dependent vasodilation contribute to the progression of heart failure. Abnormal responses to muscarinic stimulation along with a defect in the endothelial nitric oxide pathway have been suggested as the potential underlying mechanisms.

Altered gene expressions resulting in calcium-handling abnormalities, downregulation of myosin or conversion to the less-active beta isoform, and abnormal beta-receptor signal transduction have all been identified at the molecular level in the chronically failing heart.

Frequency

United States

The reported incidence rate is 0.57 cases per 100,000 children.2

International

The incidence rate in Finland is 2.6 cases per 100,000 children.3  In the United Kingdom, the incidence rate is 0.87 cases per 100,000 individuals older than 16 years.4 No reliable figures are available for the rest of the world. Genetic causes account for more than 30% of DCM cases.

Mortality/Morbidity

Mortality and morbidity have greatly decreased because of advances in medical management. Studies from 1975-1990 reported 70% survival at 2 years and 52% survival at 11.5 years of follow-up.5,6,7,8,3,9 Studies from 1992-1997 document more than 85% survival at 5 years. 

In general, approximately one third of patients die from the disease, one third of patients continue to have chronic heart failure requiring therapy, and one third of patients experience improvement in their condition. Causes of death include heart failure, ventricular arrhythmias, and transplantation-related complications (less common). Studies confirm children with underlying muscle disorders with progressive dilatation of the heart and worsening heart failure have a worse prognosis compared with patients with idiopathic DCM.10

Sex

DCM is reportedly more common in boys than in girls, and some forms are clearly X-linked.2

Age

All age groups are affected. However, studies suggest that DCM is more common in infancy (age <1 y) than in children.2 Fetal presentation is uncommon.

Clinical

History

  • Onset is usually insidious but may be acute in as many as 25% of patients with dilated cardiomyopathy (DCM), especially if exacerbated by a complicating lower respiratory infection.
  • Cough, poor feeding, irritability, and shortness of breath are usually the initial presenting symptoms.
  • Pallor, sweating, easy fatigability, failure to gain weight, and decreased urine output may be observed.
  • Wheezing may be an important clinical sign, suggesting congestive heart failure manifestation in infants.
  • Chest pain, palpitations, orthopnea, hemoptysis, frothy sputum, sudden death, abdominal pain, syncope, and neurologic deficit are other symptoms at presentation (20%).
  • Cardiomegaly that is incidentally detected on a chest radiograph or an arrhythmia that is incidentally detected on an ECG may be the basis for initial cardiac referral.
  • Approximately 50% of patients with DCM have a history of preceding viral illness. A detailed family history for familial cardiomyopathy is revealing in as many as 25% of cases.

Physical

  • In a patient with established disease, features of congestive heart failure are dominant.

  • The infant or young child with the disease is often tachypneic, tachycardic with weak peripheral pulses, and has cool extremities and hepatomegaly. Blood pressure is low with a decreased pulse pressure. In extreme cases, patients may present in shock.

  • Older children may show dependent edema, elevated jugular venous pulses, and fine basal crepitations in the lungs.

  • Major cardiac findings include cardiomegaly, quiet precordium, tachycardia, gallop rhythm (S3 and/or S4), accentuated P-2, and murmurs of mitral and tricuspid regurgitation. Murmurs may be inconspicuous initially if the patient presents with acute heart failure.

  • Infants often present with predominantly respiratory signs and, in the absence of a precordial heave or prominent murmur, the underlying cardiac disease may remain undiagnosed until cardiomegaly is detected on a chest radiograph.

Causes

  • Various factors have been identified as causes of myocardial damage. These are presented in Table 1. However, in the vast majority of patients, no specific etiology is demonstrable (idiopathic). Systemic carnitine deficiency and anthracycline-induced cardiomyopathy are notable exceptions.
    • Table 1. Factors Identified as Causes of Myocardial Damage

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    Table
    Category Of FactorsSpecific Factors
    Viral infections (myocarditis)Coxsackievirus B, human immunodeficiency virus, echovirus, rubella, varicella, mumps, Ebstein-Barr virus, cytomegalovirus, measles, polio
    Bacterial infectionsDiphtheria, Mycoplasma, tuberculosis, Lyme disease, septicemia
    RickettsiaPsittacosis, Rocky Mountain spotted fever
    ParasitesToxoplasma, Toxocara, Cysticercus
    FungiHistoplasma, coccidioidomycoses, Actinomyces
    Neuromuscular disordersDuchenne or Becker muscular dystrophies, Friedreich ataxia, Kearns-Sayre syndrome, other muscular dystrophies
    Nutritional factorsKwashiorkor, pellagra, thiamine deficiency, selenium deficiency
    Collagen vascular diseasesRheumatic fever, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease
    Hematological diseasesThalassemia, sickle cell disease, iron deficiency anemia
    Coronary artery diseasesAnomalous left coronary artery from pulmonary artery, infarction
    DrugsAnthracycline, cyclophosphamide, chloroquine, iron overload
    Endocrine diseasesHypothyroidism, hyperthyroidism, hypoparathyroidism, pheochromocytoma, hypoglycemia
    Metabolic disordersGlycogen-storage diseases, carnitine deficiency, fatty acid oxidation defects, mucopolysaccharidoses
    Malformation syndromesCat-cry syndrome (5p-)
    Category Of FactorsSpecific Factors
    Viral infections (myocarditis)Coxsackievirus B, human immunodeficiency virus, echovirus, rubella, varicella, mumps, Ebstein-Barr virus, cytomegalovirus, measles, polio
    Bacterial infectionsDiphtheria, Mycoplasma, tuberculosis, Lyme disease, septicemia
    RickettsiaPsittacosis, Rocky Mountain spotted fever
    ParasitesToxoplasma, Toxocara, Cysticercus
    FungiHistoplasma, coccidioidomycoses, Actinomyces
    Neuromuscular disordersDuchenne or Becker muscular dystrophies, Friedreich ataxia, Kearns-Sayre syndrome, other muscular dystrophies
    Nutritional factorsKwashiorkor, pellagra, thiamine deficiency, selenium deficiency
    Collagen vascular diseasesRheumatic fever, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease
    Hematological diseasesThalassemia, sickle cell disease, iron deficiency anemia
    Coronary artery diseasesAnomalous left coronary artery from pulmonary artery, infarction
    DrugsAnthracycline, cyclophosphamide, chloroquine, iron overload
    Endocrine diseasesHypothyroidism, hyperthyroidism, hypoparathyroidism, pheochromocytoma, hypoglycemia
    Metabolic disordersGlycogen-storage diseases, carnitine deficiency, fatty acid oxidation defects, mucopolysaccharidoses
    Malformation syndromesCat-cry syndrome (5p-)
  • Three major factors have been implicated in the pathogenesis of myocardial damage in DCM: preceding viral myocarditis, autoimmunity, and underlying genetic predisposition.
    • Viral myocarditis
      • Epidemiologic, serologic, and molecular studies have detected evidence of enteroviral infection, in particular coxsackievirus B, in 20-25% of patients. Recent evidence implicates various other viruses. In fact, the most common associated viruses appear to vary over time.11,12 Currently, coxsackievirus B is likely a less common cause of DCM than in the past.
      • Currently, no methods can be used to distinguish cardiovirulent strains of enteroviruses from those that are not virulent. Furthermore, the presence of a virus a patient with DCM does not necessarily establish a causal relationship. Demonstration of viral DNA or RNA by polymerase chain reaction (PCR) is a more reliable method for revealing viral myocarditis. Unfortunately, obtaining myocardial tissue is invasive.
      • The exact mechanism of myocardial damage (rapid destruction or a long-term slowing of cardiomyocyte function) also remains unclear.
    • Autoimmunity
      • Animal studies have shown that DCM is an autoimmune disease in genetically predisposed strains of mice. 
      • Approximately 30-40% of adult patients with DCM have organ-specific and disease-specific autoantibodies. The absence of these antibodies in the remaining patients may be related to the stage of disease progression.
      • The notion that an insult such as viral myocarditis initiates an autoimmune process with superantigen-triggered immune responses, resulting in massive T-lymphocyte activation and myocardial damage, has been postulated.
    • Genetic predisposition
      • Genetic causes account for 25-50% of DCM cases.
      • The role of genetic factors is exemplified by the studies on familial DCM.13,14
      • Patients with familial DCM have an increased frequency of human leukocyte antigen (HLA)-DR4. The frequency of HLA-DQA1 0501 alleles has been reported to be significantly higher in patients with idiopathic DCM.15  
      • Autosomal dominant and recessive inheritance, X-linked transmission, and polygenic and mitochondrial inheritance have all been documented. Presently known DCM genetic loci are summarized in Table 2.

        Table 2. Summary of Genetic Loci and Disease Genes for Familial Dilated Cardiomyopathy

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Table
Clinical PatternIdentified Genetic LociIdentified Disease Genes
Autosomal dominant (AD)10q21-10q23, 9q13-q22, 1q32, 15q14, 2q31, 1q11-21Actin, desmin, lamin A/C
AD with conduction defect1p1-1q1, 3p22-3p25 ...
X-linked (XL)Xp21Dystrophin
XL cardio-skeletal (Barth syndrome)Xq28 (gene G4.5)Tafazzin
Clinical PatternIdentified Genetic LociIdentified Disease Genes
Autosomal dominant (AD)10q21-10q23, 9q13-q22, 1q32, 15q14, 2q31, 1q11-21Actin, desmin, lamin A/C
AD with conduction defect1p1-1q1, 3p22-3p25 ...
X-linked (XL)Xp21Dystrophin
XL cardio-skeletal (Barth syndrome)Xq28 (gene G4.5)Tafazzin

      • Mutation screening of the exons that code for actin, β myosin heavy chain (MYH7 gene), cardiac troponin T (TNNT2 gene), phospholamban (PLN gene), titin, α β -crystallin, and the cardio-specific exon of metavinculin (VCL gene) could be helpful in detecting some forms of familial DCM.
  • Anthracyclines, which are widely used in the management of childhood malignancies, account for as many as 30% of cases of DCM in the United States and a lesser percentage in other countries.
    • Besides DCM, the other manifestations of anthracycline cardiotoxicity include restrictive cardiomyopathy (symptomatic and asymptomatic), asymptomatic left ventricular enlargement, and more subtle changes of cardiac function.
    • Early diagnosis requires periodic Doppler echocardiography studies during therapy and for several years after cessation of treatment. It also requires more widespread use of load-independent measurements of cardiac contractility (like stress velocity index), which incorporate measurements of contractility, afterload, and preload. 
    • Cardiotoxicity has 2 types: early onset and late onset. The early onset type may be acute nonprogressive or chronically progressive. 
      • Acute-onset type is defined as left ventricular dysfunction during or immediately following infusion of anthracycline and is attenuated by discontinuation of therapy. With use of low-dose regimens, this type is becoming rare. ECG changes include nonspecific ST segment and T wave changes, decreased QRS voltage, prolonged QT interval, and sinus tachycardia. Less commonly, ventricular, junctional, or supraventricular tachycardia or atrioventricular and bundle branch blocks. Blood levels of cardiac troponin T (cTnT) are a specific marker of this type of injury. 
      • Early onset chronically progressive toxicity presents within one year of completion of therapy and persists or progresses even after discontinuation of therapy. Clinical features are similar to any other type of cardiomyopathy and include ECG changes, left ventricular dysfunction, arrhythmias, reduced exercise-stress capacity, and even overt signs of heart failure. Blood levels of cTnT are elevated. Presence of early onset cardiotoxicity is believed to be a harbinger of poor patient outcome.
      • Late-onset toxicity clinically manifests after a latent period of one or more years following completion of therapy. This type of manifestation is presumably due to diminished left ventricular contractility and an inappropriately thin left ventricular wall, resulting in elevated wall stress and progressive left ventricular dysfunction. Myocyte loss underlies all these sequelae, and alteration of myocellular protein transcription by anthracyclines may contribute. Thus, the latent period is not latent at all, and more sensitive markers may be able to detect the changes earlier. Late-onset asymptomatic toxicity has also been reported, but more detailed questioning often reveals easy fatigability or dyspnea in many of these patients.
      • Risk factors contributing to the development of cardiotoxicity include the following: (1) the total cumulative dose of the drug received (20% mortality with cumulative dose >550 mg/m2, 65% frequency of subtle Echo changes with dose >400 mg/m2, and histologic evidence of toxicity with >240 mg/m2), (2) female sex (higher cellular concentrations because of higher body fat percentage), (3) young children, (4) rate of drug administration (maximum risk with doses >50 mg/m2/dose), (5) concomitant cardiac radiation exposure or use of amsacrine, (6) black race, and (7) trisomy 21.

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References
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References

  1. McMahon CJ, Pignatelli RH, Nagueh SF, et al. Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation. Heart. Jun 2007;93(6):676-81. [Medline].

  2. Towbin JA, Lowe AM, Colan SD, et al. Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA. Oct 18 2006;296(15):1867-76. [Medline].

  3. Arola A, Tuominen J, Ruuskanen O, Jokinen E. Idiopathic dilated cardiomyopathy in children: prognostic indicators and outcome. Pediatrics. Mar 1998;101(3 Pt 1):369-76. [Medline].

  4. [Best Evidence] Andrews RE, Fenton MJ, Ridout DA, Burch M. New-onset heart failure due to heart muscle disease in childhood: a prospective study in the United kingdom and Ireland. Circulation. Jan 1 2008;117(1):79-84. [Medline].

  5. Griffin ML, Hernandez A, Martin TC, et al. Dilated cardiomyopathy in infants and children. J Am Coll Cardiol. Jan 1988;11(1):139-44. [Medline].

  6. Akagi T, Benson LN, Lightfoot NE, et al. Natural history of dilated cardiomyopathy in children. Am Heart J. May 1991;121(5):1502-6. [Medline].

  7. Chen SC, Nouri S, Balfour I, Jureidini S, Appleton RS. Clinical profile of congestive cardiomyopathy in children. J Am Coll Cardiol. Jan 1990;15(1):189-93. [Medline].

  8. Venugopalan P, Houston AB, Agarwal AK. The outcome of idiopathic dilated cardiomyopathy and myocarditis in children from the west of Scotland. Int J Cardiol. Apr 2001;78(2):135-41. [Medline].

  9. Venugopalan P, Agarwal AK, Akinbami FO. Improved prognosis of heart failure due to idiopathic dilated cardiomyopathy in children. Int J Cardiol. Jul 1 1998;65(2):125-8. [Medline].

  10. Connuck DM, Sleeper LA, Colan SD, et al. Characteristics and outcomes of cardiomyopathy in children with Duchenne or Becker muscular dystrophy: a comparative study from the Pediatric Cardiomyopathy Registry. Am Heart J. Jun 2008;155(6):998-1005. [Medline].

  11. Bowles NE, Ni J, Kearney DL, et al. Detection of viruses in myocardial tissues by polymerase chain reaction. evidence of adenovirus as a common cause of myocarditis in children and adults. J Am Coll Cardiol. Aug 6 2003;42(3):466-72. [Medline].

  12. Fujioka S, Kitaura Y, Ukimura A, et al. Evaluation of viral infection in the myocardium of patients with idiopathic dilated cardiomyopathy. J Am Coll Cardiol. Nov 15 2000;36(6):1920-6. [Medline].

  13. Michels VV, Driscoll DJ, Miller FA, et al. Progression of familial and non-familial dilated cardiomyopathy: long term follow up. Heart. Jul 2003;89(7):757-61. [Medline].

  14. Michels VV, Olson TM, Miller FA, et al. Frequency of development of idiopathic dilated cardiomyopathy among relatives of patients with idiopathic dilated cardiomyopathy. Am J Cardiol. Jun 1 2003;91(11):1389-92. [Medline].

  15. Liu W, Li WM, Sun NL. Relationship between HLA-DQA1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy. Chin Med J (Engl). Oct 2004;117(10):1449-52. [Medline].

  16. Friedberg MK, Roche SL, Balasingam M, et al. Evaluation of mechanical dyssynchrony in children with idiopathic dilated cardiomyopathy and associated clinical outcomes. Am J Cardiol. Apr 15 2008;101(8):1191-5. [Medline].

  17. Senes M, Erbay AR, Yilmaz FM, et al. Coenzyme Q10 and high-sensitivity C-reactive protein in ischemic and idiopathic dilated cardiomyopathy. Clin Chem Lab Med. 2008;46(3):382-6. [Medline].

  18. Morales DL, Dreyer WJ, Denfield SW, et al. Over two decades of pediatric heart transplantation: how has survival changed?. J Thorac Cardiovasc Surg. Mar 2007;133(3):632-9. [Medline].

  19. Tjang YS, Stenlund H, Tenderich G, et al. Risk factor analysis in pediatric heart transplantation. J Heart Lung Transplant. Apr 2008;27(4):408-15. [Medline].

  20. Breinholt JP, Fraser CD, Dreyer WJ, et al. The efficacy of mitral valve surgery in children with dilated cardiomyopathy and severe mitral regurgitation. Pediatr Cardiol. Jan 2008;29(1):13-8. [Medline].

  21. Malaisrie SC, Pelletier MP, Yun JJ, et al. Pneumatic paracorporeal ventricular assist device in infants and children: initial Stanford experience. J Heart Lung Transplant. Feb 2008;27(2):173-7. [Medline].

  22. Janousek J, Gebauer RA. Cardiac resynchronization therapy in pediatric and congenital heart disease. Pacing Clin Electrophysiol. Feb 2008;31 Suppl 1:S21-3. [Medline].

  23. Norkiene I, Ringaitiene D, Rucinskas K, et al. Intra-aortic balloon counterpulsation in decompensated cardiomyopathy patients: bridge to transplantation or assist device. Interact Cardiovasc Thorac Surg. Feb 2007;6(1):66-70. [Medline].

  24. John JB, Cron SG, Kung GC, Mott AR. Intracardiac thrombi in pediatric patients: presentation profiles and clinical outcomes. Pediatr Cardiol. May-Jun 2007;28(3):213-20. [Medline].

  25. Rhee EK, Canter CE, Basile S, Webber SA, Naftel DC. Sudden death prior to pediatric heart transplantation: would implantable defibrillators improve outcome?. J Heart Lung Transplant. May 2007;26(5):447-52. [Medline].

  26. Agostini D, Belin A, Amar MH, et al. Improvement of cardiac neuronal function after carvedilol treatment in dilated cardiomyopathy: a 123I-MIBG scintigraphic study. J Nucl Med. May 2000;41(5):845-51. [Medline].

  27. Al-Kiyumi WA, Venugopalan P. Antiphospholipid syndrome presenting as dilated cardiomyopathy in an 11-year-old boy. Acta Cardiol. Aug 2003;58(4):359-61. [Medline].

  28. Arbustini Eloisa AE, Diegoli M, Pasotti M, et al. Gene symbol: CMD1J. Disease: Dilated cardiomyopathy. Hum Genet. Jul 2005;117(2-3):297. [Medline].

  29. Bhagavan HN, Chopra RK. Potential role of ubiquinone (coenzyme Q10) in pediatric cardiomyopathy. Clin Nutr. Jun 2005;24(3):331-8. [Medline].

  30. Borggrefe M, Block M, Breithardt G. Identification and management of the high risk patient with dilated cardiomyopathy. Br Heart J. Dec 1994;72(6 Suppl):S42-5. [Medline].

  31. Brown CA, O'Connell JB. Implications of the Myocarditis Treatment Trial for clinical practice. Curr Opin Cardiol. May 1996;11(3):332-6. [Medline].

  32. Bruns LA, Chrisant MK, Lamour JM, et al. Carvedilol as therapy in pediatric heart failure: an initial multicenter experience. J Pediatr. Apr 2001;138(4):505-11. [Medline].

  33. Burch M, Siddiqi SA, Celermajer DS, et al. Dilated cardiomyopathy in children: determinants of outcome. Br Heart J. Sep 1994;72(3):246-50. [Medline].

  34. Caforio AL. Role of autoimmunity in dilated cardiomyopathy. Br Heart J. Dec 1994;72(6 Suppl):S30-4. [Medline].

  35. Chantranuwat C, Blakey JD, Kobashigawa JA, et al. Sudden, unexpected death in cardiac transplant recipients: an autopsy study. J Heart Lung Transplant. Jun 2004;23(6):683-9. [Medline].

  36. Chi NH, Huang SC, Yu HY, et al. Application of papillary muscle sling concept in an infant as a biological bridge to transplantation. Ann Thorac Surg. Jan 2005;79(1):337-9. [Medline].

  37. Cnota JF, Samson RA. Left bundle branch block in infants with dilated cardiomyopathy conveys a poor prognosis. Cardiol Young. Jan 1999;9(1):55-7. [Medline].

  38. Diogenes MS, Carvalho AC, Succi RC. Reversible cardiomyopathy subsequent to perinatal infection with the human immunodeficiency virus. Cardiol Young. Aug 2003;13(4):373-6. [Medline].

  39. Dubin AM, Berul CI, Bevilacqua LM, et al. The use of implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation. J Card Fail. Oct 2003;9(5):375-9. [Medline].

  40. El Belghiti MR, Aniba K, Bouskraoui M, Fadouach S. [Cerebral ischemic stroke revealing dilated idiopathic cardiomyopathy in a 10 month-old infant]. Arch Pediatr. Jul 2005;12(7):1166-7. [Medline].

  41. Elliott P. Cardiomyopathy. Diagnosis and management of dilated cardiomyopathy. Heart. Jul 2000;84(1):106-12. [Medline].

  42. Friedman RA, Moak JP, Garson A Jr. Clinical course of idiopathic dilated cardiomyopathy in children. J Am Coll Cardiol. Jul 1991;18(1):152-6. [Medline].

  43. Gagliardi MG, Bevilacqua M, Bassano C, et al. Long term follow up of children with myocarditis treated by immunosuppression and of children with dilated cardiomyopathy. Heart. Oct 2004;90(10):1167-71. [Medline].

  44. Giardini A, Formigari R, Bronzetti G, et al. Modulation of neurohormonal activity after treatment of children in heart failure with carvedilol. Cardiol Young. Aug 2003;13(4):333-6. [Medline].

  45. Gradinac S, Jovanovic I, Dukic M, et al. Partial left ventriculectomy in a two-year-old girl with dilated cardiomyopathy. J Heart Lung Transplant. Apr 1999;18(4):381-3. [Medline].

  46. Grenier MA, Lipshultz SE. Epidemiology of anthracycline cardiotoxicity in children and adults. Semin Oncol. Aug 1998;25(4 Suppl 10):72-85. [Medline].

  47. Groetzner J, Reichart B, Roemer U, et al. Cardiac transplantation in pediatric patients: fifteen-year experience of a single center. Ann Thorac Surg. Jan 2005;79(1):53-60; discussion 61. [Medline].

  48. Guntheroth WG. Improved outcomes of pediatric dilated cardiomyopathy and heart transplantation. J Am Coll Cardiol. May 17 2005;45(10):1733-4; author reply 1734. [Medline].

  49. Horenstein MS, Ross RD, Singh TP, Epstein ML. Carvedilol reverses elevated pulmonary vascular resistance in a child with dilated cardiomyopathy. Pediatr Cardiol. Jan-Feb 2002;23(1):100-2. [Medline].

  50. Karkkainen S, Helio T, Miettinen R, et al. A novel mutation, Ser143Pro, in the lamin A/C gene is common in finnish patients with familial dilated cardiomyopathy. Eur Heart J. May 2004;25(10):885-93. [Medline].

  51. Karkkainen S, Miettinen R, Tuomainen P, et al. A novel mutation, Arg71Thr, in the delta-sarcoglycan gene is associated with dilated cardiomyopathy. J Mol Med. Dec 2003;81(12):795-800. [Medline].

  52. Kinali M, Olpin SE, Clayton PT, et al. Diagnostic difficulties in a case of primary systemic carnitine deficiency with idiopathic dilated cardiomyopathy. Eur J Paediatr Neurol. 2004;8(4):217-9. [Medline].

  53. Kleinert S, Weintraub RG, Wilkinson JL, Chow CW. Myocarditis in children with dilated cardiomyopathy: incidence and outcome after dual therapy immunosuppression. J Heart Lung Transplant. Dec 1997;16(12):1248-54. [Medline].

  54. Kothari SS, Dhopeshwarkar RA, Saxena A, Juneja R. Dilated cardiomyopathy in Indian children. Indian Heart J. Mar-Apr 2003;55(2):147-51. [Medline].

  55. Krell MJ, Kline EM, Bates ER, et al. Intermittent, ambulatory dobutamine infusions in patients with severe congestive heart failure. Am Heart J. Oct 1986;112(4):787-91. [Medline].

  56. Laer S, Mir TS, Behn F, et al. Carvedilol therapy in pediatric patients with congestive heart failure: a study investigating clinical and pharmacokinetic parameters. Am Heart J. May 2002;143(5):916-22. [Medline].

  57. Lancet. Carnitine deficiency. Lancet. Mar 17 1990;335(8690):631-3. [Medline].

  58. Leprince P, Bonnet N, Rama A, et al. Bridge to transplantation with the Jarvik-7 (CardioWest) total artificial heart: a single-center 15-year experience. J Heart Lung Transplant. Dec 2003;22(12):1296-303. [Medline].

  59. Lev D, Nissenkorn A, Leshinsky-Silver E, et al. Clinical presentations of mitochondrial cardiomyopathies. Pediatr Cardiol. Sep-Oct 2004;25(5):443-50. [Medline].

  60. Lewis AB. Late recovery of ventricular function in children with idiopathic dilated cardiomyopathy. Am Heart J. Aug 1999;138(2 Pt 1):334-8. [Medline].

  61. Lewis AB, Chabot M. Outcome of infants and children with dilated cardiomyopathy. Am J Cardiol. Aug 1 1991;68(4):365-9. [Medline].

  62. Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med. Apr 24 2003;348(17):1647-55. [Medline].

  63. Liu W, Li WM, Sun NL. HLA-DQA1, -DQB1 polymorphism and genetic susceptibility to idiopathic dilated cardiomyopathy in Hans of northern China. Ann Hum Genet. Jul 2005;69(Pt 4):382-8. [Medline].

  64. Luppi P, Rudert WA, Zanone MM, et al. Idiopathic dilated cardiomyopathy: a superantigen-driven autoimmune disease. Circulation. Aug 25 1998;98(8):777-85. [Medline].

  65. Mason JW, O'Connell JB, Herskowitz A, et al. A clinical trial of immunosuppressive therapy for myocarditis. The Myocarditis Treatment Trial Investigators. N Engl J Med. Aug 3 1995;333(5):269-75. [Medline].

  66. Matsumiya G, Monta O, Fukushima N, et al. Who would be a candidate for bridge to recovery during prolonged mechanical left ventricular support in idiopathic dilated cardiomyopathy?. J Thorac Cardiovasc Surg. Sep 2005;130(3):699-704. [Medline].

  67. Maunoury C, Acar P, Sidi D. Use of 123I-MIBG scintigraphy to assess the impact of carvedilol on cardiac adrenergic neuronal function in childhood dilated cardiomyopathy. Eur J Nucl Med Mol Imaging. Dec 2003;30(12):1651-6. [Medline].

  68. McElhinney DB, Colan SD, Moran AM. Recombinant human growth hormone treatment for dilated cardiomyopathy in children. Pediatrics. Oct 2004;114(4):e452-8. [Medline].

  69. McMahon CJ, Nagueh SF, Eapen RS, et al. Echocardiographic predictors of adverse clinical events in children with dilated cardiomyopathy: a prospective clinical study. Heart. Aug 2004;90(8):908-15. [Medline].

  70. McNamara DM, Rosenblum WD, Janosko KM, et al. Intravenous immune globulin in the therapy of myocarditis and acute cardiomyopathy. Circulation. Jun 3 1997;95(11):2476-8. [Medline].

  71. Mogensen J, Murphy RT, Shaw T, et al. Severe disease expression of cardiac troponin C and T mutations in patients with idiopathic dilated cardiomyopathy. J Am Coll Cardiol. Nov 16 2004;44(10):2033-40. [Medline].

  72. Narula J, Haider N, Virmani R, et al. Apoptosis in myocytes in end-stage heart failure. N Engl J Med. Oct 17 1996;335(16):1182-9. [Medline].

  73. Nugent AW, Daubeney PE, Chondros P, et al. The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. Apr 24 2003;348(17):1639-46. [Medline].

  74. Nurnberg JH, Butter C, Abdul-Khaliq H, et al. Successful cardiac resynchronization therapy in a 9-year-old boy with dilated cardiomyopathy. Z Kardiol. Jan 2005;94(1):44-8. [Medline].

  75. O'Connell JB, Moore CK, Waterer HC. Treatment of end stage dilated cardiomyopathy. Br Heart J. Dec 1994;72(6 Suppl):S52-6. [Medline].

  76. Paraskevaidis IA, Adamopoulos S, Tsiapras D, et al. Prognostic usefulness of echocardiographic dobutamine in younger (14 to 25 years) and older (40 to 55 years) patients with idiopathic dilated cardiomyopathy. Am J Cardiol. Jan 15 2004;93(2):251-5. [Medline].

  77. Park OY, Ahn Y, Park WS, et al. Rapid progression from hypertrophic cardiomyopathy to heart failure in a patient with Becker's muscular dystrophy. Eur J Heart Fail. Jun 2005;7(4):684-8. [Medline].

  78. Pittenger B, Gill EA, Holcslaw TL, Bristow MR. Relation of dose of carvedilol to reduction in QT dispersion in patients with mild to moderate heart failure secondary to ischemic or to idiopathic dilated cardiomyopathy. Am J Cardiol. Dec 1 2004;94(11):1459-62. [Medline].

  79. Price JF, Towbin JA, Denfield SW, et al. Arginine vasopressin levels are elevated and correlate with functional status in infants and children with congestive heart failure. Circulation. Jun 1 2004;109(21):2550-3. [Medline].

  80. Quek SC, Hia CP, Yip WC. A surgically correctable cause of dilated cardiomyopathy in infancy. Cardiol Young. Feb 2005;15(1):54-5. [Medline].

  81. Ratnapalan S, Griffiths K, Costei AM, et al. Digoxin-carvedilol interactions in children. J Pediatr. May 2003;142(5):572-4. [Medline].

  82. Rose AG, Park SJ. Pathology in patients with ventricular assist devices: a study of 21 autopsies, 24 ventricular apical core biopsies and 24 explanted hearts. Cardiovasc Pathol. Jan-Feb 2005;14(1):19-23. [Medline].

  83. Rusconi P, Gomez-Marin O, Rossique-Gonzalez M, et al. Carvedilol in children with cardiomyopathy: 3-year experience at a single institution. J Heart Lung Transplant. Jul 2004;23(7):832-8. [Medline].

  84. Salzberg S, Lachat M, Zund G, et al. Left ventricular assist device as bridge to heart transplantation--lessons learned with the MicroMed DeBakey axial blood flow pump. Eur J Cardiothorac Surg. Jul 2003;24(1):113-8. [Medline].

  85. Satoh M, Nakamura M, Saitoh H, et al. Tumor necrosis factor-alpha-converting enzyme and tumor necrosis factor- alpha in human dilated cardiomyopathy. Circulation. Jun 29 1999;99(25):3260-5. [Medline].

  86. Sebillon P, Bouchier C, Bidot LD, et al. Expanding the phenotype of LMNA mutations in dilated cardiomyopathy and functional consequences of these mutations. J Med Genet. Aug 2003;40(8):560-7. [Medline].

  87. Shaddy RE, Tani LY, Gidding SS, et al. Beta-blocker treatment of dilated cardiomyopathy with congestive heart failure in children: a multi-institutional experience. J Heart Lung Transplant. Mar 1999;18(3):269-74. [Medline].

  88. Siu BL, Niimura H, Osborne JA, et al. Familial dilated cardiomyopathy locus maps to chromosome 2q31. Circulation. Mar 2 1999;99(8):1022-6. [Medline].

  89. Sliwa K, Norton GR, Kone N, et al. Impact of initiating carvedilol before angiotensin-converting enzyme inhibitor therapy on cardiac function in newly diagnosed heart failure. J Am Coll Cardiol. Nov 2 2004;44(9):1825-30. [Medline].

  90. Soongswang J, Durongpisitkul K, Nana A, et al. Cardiac troponin T: a marker in the diagnosis of acute myocarditis in children. Pediatr Cardiol. Jan-Feb 2005;26(1):45-9. [Medline].

  91. Stefanelli CB, Rosenthal A, Borisov AB, et al. Novel troponin T mutation in familial dilated cardiomyopathy with gender-dependant severity. Mol Genet Metab. Sep-Oct 2004;83(1-2):188-96. [Medline].

  92. Suma H, Isomura T, Horii T, Buckberg G. Role of site selection for left ventriculoplasty to treat idiopathic dilated cardiomyopathy. Heart Fail Rev. Oct 2004;9(4):329-36. [Medline].

  93. Takahashi T, Saegusa S, Sumino H, et al. Adiponectin, T-cadherin and tumour necrosis factor-alpha in damaged cardiomyocytes from autopsy specimens. J Int Med Res. Mar-Apr 2005;33(2):236-44. [Medline].

  94. Tsirka AE, Trinkaus K, Chen SC, et al. Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation. J Am Coll Cardiol. Jul 21 2004;44(2):391-7. [Medline].

  95. Venugopalan P, Agarwal AK. Plasma catecholamine levels parallel severity of heart failure and have prognostic value in children with dilated cardiomyopathy. Eur J Heart Fail. Oct 2003;5(5):655-8. [Medline].

  96. Venugopalan P, Agarwal AK, Akinbami FO, et al. Improved prognosis of heart failure due to idiopathic dilated cardiomyopathy in children. Int J Cardiol. Jul 1 1998;65(2):125-8. [Medline].

  97. Venugopalan P, Agarwal AK, Worthing EA. Chronic cardiac failure in children due to dilated cardiomyopathy: diagnostic approach, pathophysiology and management [published correction appears in Eur J Pediatr. 2000;160(4):266]. Eur J Pediatr. Nov 2000;159(11):803-10. [Medline].

  98. Vijayalakshmi IB, Yavagal ST, Prabhudev N. Role of echocardiography in assessing the mechanism and effect of ramipril on functional mitral regurgitation in dilated cardiomyopathy. Echocardiography. Apr 2005;22(4):289-95. [Medline].

  99. Villard E, Duboscq-Bidot L, Charron P, et al. Mutation screening in dilated cardiomyopathy: prominent role of the beta myosin heavy chain gene. Eur Heart J. Apr 2005;26(8):794-803. [Medline].

  100. Weng KP, Lin CC, Huang SH, Hsieh KS. Idiopathic dilated cardiomyopathy in children: a single medical center's experience. J Chin Med Assoc. Aug 2005;68(8):368-72. [Medline].

  101. Werner B, Przybylski A, Kucinska B, et al. Implantable cardioverter-defibrillators in children. Kardiol Pol. Mar 2004;60(3):239-46. [Medline].

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Further Reading

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Keywords

dilated cardiomyopathy, DCM, congestive cardiomyopathy, idiopathic dilated cardiomyopathy, idiopathic cardiomyopathy, congestive cardiac failure, cardiac failure, heart failure, enlargement of the heart muscle, heart disease, global hypokinesia, fatigue, mitral regurgitation, tricuspid regurgitation, subendocardial ischemia,  ventricular arrhythmia, orthopnea, hemoptysis, frothy sputum, syncope, cardiomegaly, arrhythmia,  coxsackievirus B, human immunodeficiency, echovirus, rubella, varicella, mumps, Ebstein-Barr virus, cytomegalovirus, measles, poliovirus, diphtheria, Mycoplasma infection , tuberculosis, lyme disease, septicemia, psittacosis, Rocky Mountain spotted fever, Toxoplasma, Toxocara, Cysticercus, Histoplasma, coccidioidomycoses, Actinomyces, Duchenne muscular dystrophy, Becker muscular dystrophy, Friedreich ataxia, Kearns-Sayre syndrome, kwashiorkor, pellagra, thiamine deficiency, selenium deficiency, rheumatic fever, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease, thalassemia, sickle cell disease, iron deficiency anemia, anomalous left coronary artery from pulmonary artery, infarction, anthracycline, cyclophosphamide, chloroquine, iron overload, hypothyroidism, hyperthyroidism, hypoparathyroidism, pheochromocytoma, hypoglycemia, glycogen storage diseases, carnitine deficiency, fatty acid oxidation defects, mucopolysaccharidoses, Cat-cry syndrome

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Contributor Information and Disclosures

Author

Poothirikovil Venugopalan, MBBS, MD, FRCP (Glasg), FRCPCH, Consulting Staff, Department of Child Health, University Hospital of Hartlepool, UK
Poothirikovil Venugopalan, MBBS, MD, FRCP (Glasg), FRCPCH is a member of the following medical societies: British Cardiac Society and Royal College of Physicians and Surgeons of Glasgow
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, Heart Rhythm Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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