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

Cardiomyopathy, Dilated: Differential Diagnoses & Workup

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

Differential Diagnoses

Anomalous Left Coronary Artery From the Pulmonary Artery
Myocarditis, Nonviral
Aortic Stenosis, Valvar
Myocarditis, Viral
Carnitine Deficiency
Coarctation of the Aorta
Myocardial Infarction in Childhood

Workup

Laboratory Studies

  • Full blood counts, erythrocyte sedimentation rate, and C-reactive proteins may show evidence of acute inflammation in patients with dilated cardiomyopathy (DCM) in the presence of active myocarditis.
  • Similarly, creatine kinase–myocardial fraction may be elevated.
    • Rising titers of specific viral-neutralizing antibodies in the serum and positive viral cultures from nasopharyngeal or stool swabs may suggest a viral etiology; however, this does not necessarily mean a cause-and-effect relationship.
    • Serum carnitine levels (total and free) are low when the disease is due to systemic carnitine deficiency.
  • ABG analysis reveals early stages of mild respiratory alkalosis and, later, mild hypoxemia secondary to pulmonary edema. In advanced disease, mixed acid-base disturbances with metabolic acidosis indicate the need for intravenous inotropes and ventilatory assistance.

Imaging Studies

  • Chest radiography
    • Chest radiography reveals cardiomegaly with a prominent left ventricular apex and prominent pulmonary artery segment.
    • Elevation of left main bronchus reflects dilation of the left atrium. This can result in compression of the left lower lobe bronchus when combined with a dilated pulmonary artery, leading to collapse of the left lower lobe of the lung.
    • Pulmonary venous congestion and frank pulmonary edema are often evident. When present, pleural effusion is better appreciated in the erect and lateral decubitus films.
    • Massive cardiomegaly resembling pericardial effusion is the hallmark of established disease.
    • Rarely, in fulminant cases, cardiomegaly may not be prominent because the ventricle has not had time to dilate despite the presence of features of pulmonary edema.
  • Echocardiography and Doppler studies
    • These form the basis for the diagnosis of dilated cardiomyopathy (DCM) in most patients. Marked dilation of the left ventricle with global hypokinesia is the hallmark of the disease. Left ventricular fractional shortening is usually less than 25% (ejection fraction <50%). Left ventricular walls are thin and areas of dyskinesis may be observed. The left atrium is also dilated, and mitral valve leaflets show sluggish movement; the anterior leaflet does not appose to the interventricular septum, giving an increased E point septal separation on the M-mode pictures. The M-mode also clearly reveals the limited excursions of the anterior and posterior leaflets during diastole.
    • Doppler studies show varying degrees of mitral regurgitation secondary to left ventricular dilation and possible papillary muscle dysfunction. Mitral regurgitation is more prominent in follow-up studies after commencing therapy when the cardiac output has improved. Left ventricular ejection parameters show decrease in peak velocity and peak acceleration, prolongation of the pre-ejection period, and decrease in ejection time. These flow measurements are dependent on loading conditions. The dilatation of the mitral valve ring and the altered shape of the left ventricle cavity, which leads to change in the direction of the papillary muscles, are used to explain the secondary mitral regurgitation seen in a large proportion of children with DCM. Tissue Doppler studies have recently been reported in children with DCM.
    • Parameters of diastolic dysfunction are not reliable in the presence of established systolic dysfunction and mitral regurgitation; however, they may be useful in the early stages of the disease. Diastolic dysfunction is not as typical or as pronounced as it is in hypertrophic cardiomyopathy.
    • More detailed evaluation of mechanical dyssynchrony and its association with clinical status in children with DCM is increasingly used in specialized centers in an attempt to predict outcome.16 The standard deviation of QRS to peak systolic velocity interval using tissue Doppler can be measured in 12 left ventricular segments as a dyssynchrony index (DI). DI reference ranges for children have not been established; the current adult-defined DI reference ranges are used to define dyssynchrony. Longitudinal function can be assessed by serial measurements of the mitral and tricuspid valve displacements in systole.
    • Long-standing cases show evidence of pulmonary hypertension in the form of right ventricular dilation and hypertrophy and tricuspid regurgitation. Tricuspid regurgitation and pulmonary regurgitation velocities give an estimate of the pulmonary artery systolic and diastolic pressures respectively. In severe cases, swirling echodensity (smoke or spontaneous echocardiographic contrast) can be observed along the outer ventricular wall, moving from the mitral valve towards the aortic valve. Occasionally, thrombi can be visualized in the left ventricular apex and in the left atrium. Pericardial effusion also may be present.
    • Echocardiography can exclude other heart diseases, both congenital and acquired. Cardiomyopathy secondary to severe aortic stenosis, coarctation of aorta or congenital mitral valve dysplasia, and anomalous left coronary artery arising from pulmonary artery (ALCAPA) are the major differential diagnoses. At times, identifying cardiomyopathy secondary to congenital mitral regurgitation (dysplastic mitral valve without stenosis) is difficult, but the abnormal anatomy of the mitral valve leaflets should help. The echo-dense papillary muscles and the dilated proximal right coronary artery and continuous retrograde flow of blood into the origin of pulmonary artery all direct the attention of the cardiologist to ALCAPA, a potentially treatable condition that mimics DCM.
  • Radionuclide imaging
    • First-pass test and multiple gated acquisition (MUGA) scans help to measure the left and right ventricular stroke volumes and cardiac outputs. They are also helpful in documenting dyskinetic segments in the ventricular walls. Although theoretically superior to echocardiographic measurements, their practical application is limited because of a lack of standardization and because of nonreproducibility, especially in children.
    • Thallium studies can identify areas of decreased myocardial perfusion, although this is seldom required.
    • Gallium citrate Ga 67 scintigraphy and indium In-111 altumomab pentetate antimyosin antibody cardiac imaging have been suggested to help identify ongoing inflammation noninvasively. They may be used to identify patients who might benefit from myocardial biopsy.

Other Tests

  • Electrocardiography
    • ECG changes are usually nonspecific.
    • Some patients have sinus tachycardia, downward frontal plane QRS axis, left atrial enlargement, left ventricular hypertrophy, deep Q waves with ST segment depression, and tall T waves in leads I, aVL, V5, V6 (the latter reflect left ventricular volume overload).
    • In more advanced disease, right axis deviation, right atrial enlargement, and right ventricular hypertrophy are seen (because of pulmonary hypertension).
    • The main role of ECG is to detect evidence of myocardial ischemia (pathologic Q waves with ST elevation and T-wave inversion in leads I, aVL, V5, V6) that might point to anomalous coronary artery as the etiology of the cardiomyopathy. A segmental myocarditis may result in ECG features of myocardial infarction.
    • Cardiac arrhythmias, such as supraventricular/ventricular ectopy or tachycardia, may be revealed. These might indicate an underlying myocarditis or cardiomyopathy; however, if sustained, the arrhythmia may be the cause of the cardiomyopathy rather than the result (ie, tachycardia-mediated cardiomyopathy).

Procedures

  • Cardiac catheterization and angiography
    • Children with DCM are at a particular risk for complications during cardiac catheter studies and angiography. Procedures should be performed by experienced pediatric cardiologists and only when absolutely essential.
    • At present, preparation for cardiac transplant and need for myocardial biopsy are the main indications for performing the procedure.
    • Patients should be under optimum medical therapy and kept hemodynamically stable before and after catheterization. Careful observation is required during the procedure for ventricular arrhythmias and hemodynamic deterioration.
    • Echocardiography should be considered after catheterization to identify any pericardial effusion secondary to subclinical perforation of the myocardium, especially if a biopsy has also been performed.
    • Aortography may be performed to identify a coronary artery anatomy, and left ventricular angiography may be performed to assess mitral valve function. The number of biopsy specimens collected should be limited to the minimum required (usually 4-8).
    • Usual findings include elevated filling pressures in all the cardiac chambers (especially the left ventricle), elevated pulmonary wedge pressure, and reduced cardiac output and stroke volume. Mixed venous oxygen saturation and reduced arterial saturation reflect low cardiac output and pulmonary edema. Pulmonary and systemic vascular resistances are elevated. With end-stage disease, the peak systolic left ventricular and aortic pressures drop.
  • Myocardial biopsy
    • At present, preparation for cardiac transplant and post-transplant follow-up monitoring for rejection are the main indications for biopsy. If facilities are available, molecular or metabolic studies can be additional indications for academic and research purposes. Rarely, suspected metabolic diseases (isolated myocardial carnitine deficiency, rare forms of glycogen storage disease, fatty acid oxidation defects) or persistent myocarditis might require biopsy for confirmation.
    • Specimens should be subjected to both light and electron microscopy. PCR and metabolic studies should be performed when indicated.
    • The most important aspect is the availability of a sufficient level of expertise for interpretation of the findings.
    • PCR has been used to aid the detection of viral antigens in myocardial tissue in patients with DCM. Studies have revealed an association between viral antigens and DCM. However, a proportion of the studies gave negative results. A recently published meta-analysis of the studies on DCM gave an odds ratio of 3.8 to the association between presence of viral antigens in DCM. The results are influenced by factors like contamination from the reference strain used in the laboratory and choice of the controls. It also is not clear whether these positive cases among DCM actually represented acute myocarditis rather than DCM.
  • Diagnostic evaluation: Flow charts (Tables 3 and 4) guide evaluation of children with suspected DCM to arrive at a firm diagnosis.

Table 3.  Diagnosis of Dilated Cardiomyopathy in Children - Step I: Diagnosis

Open table in new window

[ CLOSE WINDOW ]
Table
ApproachFindingsConclusion
Clinical suspicionInfants and young children: Shortness of breath, feeding difficulties, wheezing, failure to thrive, recurrent chest infections, hepatomegaly, cardiomegaly
Older children: Dyspnea, dependent edema, elevated jugular venous pressure, cardiomegaly		Probable heart disease with heart failure
Chest radiographyCardiomegaly, pulmonary plethora, prominent upper lobe veins, pulmonary edema, pleural effusion, collapsed left lower lobeHigh probability of heart failure with or without chest infection
ElectrocardiographyLow-voltage complexesPericardial effusion
Presence of Q waves and inversion of T waves in leads I, II, aVL, and V4 through V6 (anterolateral infarction pattern)Anomalous left coronary artery from pulmonary artery
Significant arrhythmiaDilated cardiomyopathy secondary to arrhythmia
Left ventricular or biventricular hypertrophy with or without left ventricular strain patternOften unhelpful
Doppler echocardiographic studiesSignificant congenital heart diseaseDiagnose primary disease
Significant pericardial effusion with satisfactory left ventricular ejection fractionDiagnose pericardial effusion
Left ventricular posterior wall hypokinesia with hyperechoic papillary muscles, retrograde continuous flow into proximal pulmonary arteryDiagnose anomalous left coronary artery from pulmonary artery
Dilated left ventricle (>95th percentile) with global hypokinesia (fractional shortening <25%, ejection fraction <50%), and no demonstrable structural heart diseaseDiagnose dilated cardiomyopathy
ApproachFindingsConclusion
Clinical suspicionInfants and young children: Shortness of breath, feeding difficulties, wheezing, failure to thrive, recurrent chest infections, hepatomegaly, cardiomegaly
Older children: Dyspnea, dependent edema, elevated jugular venous pressure, cardiomegaly		Probable heart disease with heart failure
Chest radiographyCardiomegaly, pulmonary plethora, prominent upper lobe veins, pulmonary edema, pleural effusion, collapsed left lower lobeHigh probability of heart failure with or without chest infection
ElectrocardiographyLow-voltage complexesPericardial effusion
Presence of Q waves and inversion of T waves in leads I, II, aVL, and V4 through V6 (anterolateral infarction pattern)Anomalous left coronary artery from pulmonary artery
Significant arrhythmiaDilated cardiomyopathy secondary to arrhythmia
Left ventricular or biventricular hypertrophy with or without left ventricular strain patternOften unhelpful
Doppler echocardiographic studiesSignificant congenital heart diseaseDiagnose primary disease
Significant pericardial effusion with satisfactory left ventricular ejection fractionDiagnose pericardial effusion
Left ventricular posterior wall hypokinesia with hyperechoic papillary muscles, retrograde continuous flow into proximal pulmonary arteryDiagnose anomalous left coronary artery from pulmonary artery
Dilated left ventricle (>95th percentile) with global hypokinesia (fractional shortening <25%, ejection fraction <50%), and no demonstrable structural heart diseaseDiagnose dilated cardiomyopathy

Table 4. Diagnosis of Dilated Cardiomyopathy in Children - Step II: Identification of Any Underlying Etiology

Open table in new window

[ CLOSE WINDOW ]
Table
ApproachFindingsConclusion
Clinical featuresPositive family historyGenetic cause for dilated cardiomyopathy
Acute or chronic encephalopathy, muscle weakness, hypotonia, growth retardation, recurrent vomiting, lethargyInborn error of metabolism involving energy production
Coarse or dysmorphic features, organomegaly, skeletal abnormalities, short stature, chronic encephalopathy, cherry-red spot in eyesStorage diseases
Skeletal muscle weakness without encephalopathyNeuromuscular disorders
Blood investigationsHigh blood urea nitrogen and creatinine levels, low calcium and magnesium levels, electrolyte disturbancesHelp in the initial management; occasionally point to a cause of dilated cardiomyopathy, especially in neonates
Elevated acute-phase reactants and cardiac enzyme levelsMyocarditis
Positive viral titersViral myocarditis
Low serum carnitine levelsSystemic carnitine deficiency
Hypoglycemia with low or no acidosis (ketosis)
1. High insulin levels, low free fatty acid
2. Low insulin levels, high free fatty acid		1. Infant of diabetic mother, nesidioblastosis
2. Defect in fatty acid oxidation or carnitine metabolism
Hypoglycemia with moderate or high acidosis (ketosis)
1. Low or normal lactate and abnormal urine and serum organic acid levels
1. High lactate		1. Organic (propionic, methylmalonic) acidemias, or β -ketothiolase deficiency
2. Glycogen storage disease, Bath and Sengers syndromes, pyruvate dehydrogenase deficiency, mitochondrial enzyme deficiency
Hyperammonemia with acidosisOrganic acidemias (as above)
Specific enzyme assayConfirms enzymatic defect
Absence of above physical and biochemical abnormalitiesPost myocarditis or idiopathic dilated cardiomyopathy
Cardiac catheterizationEvaluate hemodynamicsUseful to predict prognosis and evaluate for transplant
Coronary angiographyAbnormal origin of left coronary artery from pulmonary arteryAnomalous left coronary artery from pulmonary artery
Myocardial biopsyMyocyte hypertrophy and fibrosis without lymphocytic infiltrateDilated cardiomyopathy
Inflammatory cell infiltration, cell necrosisMyocarditis
Special stainsMitochondrial or infiltrative diseases
Molecular studies (on blood, fibroblasts, or myocardial cells)Nucleic acid hybridization studies
Polymerase chain reaction studies		Myocarditis
DNA mutation analysisIdentifies specific genetic defect
ApproachFindingsConclusion
Clinical featuresPositive family historyGenetic cause for dilated cardiomyopathy
Acute or chronic encephalopathy, muscle weakness, hypotonia, growth retardation, recurrent vomiting, lethargyInborn error of metabolism involving energy production
Coarse or dysmorphic features, organomegaly, skeletal abnormalities, short stature, chronic encephalopathy, cherry-red spot in eyesStorage diseases
Skeletal muscle weakness without encephalopathyNeuromuscular disorders
Blood investigationsHigh blood urea nitrogen and creatinine levels, low calcium and magnesium levels, electrolyte disturbancesHelp in the initial management; occasionally point to a cause of dilated cardiomyopathy, especially in neonates
Elevated acute-phase reactants and cardiac enzyme levelsMyocarditis
Positive viral titersViral myocarditis
Low serum carnitine levelsSystemic carnitine deficiency
Hypoglycemia with low or no acidosis (ketosis)
1. High insulin levels, low free fatty acid
2. Low insulin levels, high free fatty acid		1. Infant of diabetic mother, nesidioblastosis
2. Defect in fatty acid oxidation or carnitine metabolism
Hypoglycemia with moderate or high acidosis (ketosis)
1. Low or normal lactate and abnormal urine and serum organic acid levels
1. High lactate		1. Organic (propionic, methylmalonic) acidemias, or β -ketothiolase deficiency
2. Glycogen storage disease, Bath and Sengers syndromes, pyruvate dehydrogenase deficiency, mitochondrial enzyme deficiency
Hyperammonemia with acidosisOrganic acidemias (as above)
Specific enzyme assayConfirms enzymatic defect
Absence of above physical and biochemical abnormalitiesPost myocarditis or idiopathic dilated cardiomyopathy
Cardiac catheterizationEvaluate hemodynamicsUseful to predict prognosis and evaluate for transplant
Coronary angiographyAbnormal origin of left coronary artery from pulmonary arteryAnomalous left coronary artery from pulmonary artery
Myocardial biopsyMyocyte hypertrophy and fibrosis without lymphocytic infiltrateDilated cardiomyopathy
Inflammatory cell infiltration, cell necrosisMyocarditis
Special stainsMitochondrial or infiltrative diseases
Molecular studies (on blood, fibroblasts, or myocardial cells)Nucleic acid hybridization studies
Polymerase chain reaction studies		Myocarditis
DNA mutation analysisIdentifies specific genetic defect


Histologic Findings

  • Histologic features are nonspecific in most patients and include myocardial cell loss with varying degree of necrosis and fibrosis.
  • In presence of myocarditis, lymphocytic infiltration of varying degree is also present (Dallas criteria).

More on Cardiomyopathy, Dilated

Overview: Cardiomyopathy, Dilated
Differential Diagnoses & Workup: Cardiomyopathy, Dilated
Treatment & Medication: Cardiomyopathy, Dilated
Follow-up: Cardiomyopathy, Dilated
Multimedia: Cardiomyopathy, Dilated
References
[ CLOSE WINDOW ]

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].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

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, infection tuberculosis, lyme disease, septicemia, psittacosis, Rocky Mountain spotted fever, coccidioidomycoses, Duchenne muscular dystrophy, Becker muscular dystrophy, Friedreich ataxia, Kearns-Sayre syndrome, kwashiorkor, pellagra, thiamine deficiency, selenium deficiency, rheumatic fever, rheumatoid arthritis, systemiclupus 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

[ CLOSE WINDOW ]

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, 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 Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z 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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.