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

Cardiomyopathy, Dilated: Treatment & Medication

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

Treatment

Medical Care

  • Perform general supportive measures in patients with dilated cardiomyopathy (DCM) during acute-stage management, including endotracheal intubation and mechanical ventilation, vasoactive infusions, and fluid/acid-base management. Treat chest infections appropriately. Treat anemia appropriately.
  • Oxygen inhalation is of benefit only in the presence of hypoxia (as with pneumonia or pulmonary edema).
  • Carnitine supplements (100 mg/kg IV infusion over 30 min, followed by 100 mg/kg/d as continuous infusion for 24-72 h; 25-50 mg/kg/dose PO bid/tid, not to exceed 200 mg/kg/d) reverse the myocardial dysfunction in most patients affected by systemic carnitine deficiency.
  • Coenzyme Q10 has also been used in children with DCM, with variable results.17
  • Decreased serum levels of growth hormone (GH), which acts on cardiac myocytes primarily through insulinlike growth factor (IGF)-1, are associated with impaired myocardial growth and function, which can be improved with the restoration of GH/IGF-1 homeostasis. Based on this hypothesis and on observation of benefits in animal models, GH therapy has been used in children with DCM, but the results have not been conclusive.

Surgical Care

Cardiac transplantation is currently the optimal treatment for DCM-induced resistant chronic congestive heart failure in children.

  • Limiting factors include availability of a suitable donor, complications of rejection, and lifelong immunosuppression. Survival rates of as much as 92% at 5 years18 and 53% at 15 years have been reported.19
  • Palliative surgical measures are associated with significant mortality and morbidity rates despite advances. Resection of a large segment of the hypertrophied ventricular muscle (Batista procedure) and repair or replacement of mitral valve to minimize volume overload of left ventricle have been used as palliative measures. Cardiomyoplasty is the transposition of electrically transformed skeletal muscle to provide systolic and diastolic augmentation to the native heart.
  • Mitral valve repair has been found to be feasible in selected patients and helps reduce symptoms in most patients and helps reduce left ventricular dimensions in some patients; however, whether it can modify the natural history, especially the need for cardiac transplantation, is unclear.20
  • Implantable mechanical support devices, modified for use in infants and children, have been introduced to support the failing heart until a suitable donor is available for transplantation (bridge to transplant). The Berlin Heart EXOR pediatric has also been successfully used in several centres.21 Major limitations include infection, thromboembolism, disturbance from noise, and the need to frequently recharge batteries.
  • Cardiac resynchronization therapy using a biventricular pacemaker has been shown to be effective in adults with DCM. In addition, these devices are available with defibrillator backup for patients at risk for ventricular arrhythmias. They are used in children with DCM with early favorable results.22
  • Prolonged support of left ventricular function with these devices has shown restoration of the native cardiac function, enabling removal of the device in a few cases. This raises the possibility that mechanical intervention at an earlier stage in viral myocarditis and DCM might prevent the deterioration of cardiac function. Adult studies have documented myocardial recovery and successful explantation of the left ventricular assist system, even after they were on the device for a year. A lesser degree of fibrotic changes in the left ventricle could predict better chances of recovery.

Consultations

  • A multidisciplinary approach is a must for optimum management and should include the following:
    • Pediatric cardiologist
    • Pediatric cardiothoracic surgeon
    • Pharmacist
    • Dietitian and nutritionist
    • Pediatrician or family physician
    • Occupational therapist
    • Psychologist
    • School teacher
    • Specialist nurse

Diet

  • Dietary requirements are high because of the catabolic state, recurrent infections, increased muscle activity, and need for rapid growth.
  • Dietary intake may be inadequate consequent to the anorexia, dietary restrictions, malabsorption, diarrhea, and frequent exacerbations of heart failure.
  • Ensuring an appropriate and palatable diet is a challenge.
  • Temporary nasogastric tube feedings may be required for sick and severely anorectic children.
  • Powerful diuretics have largely obviated the need for stringent restrictions on salt and fluid intake.

Activity

  • Enforced bed rest is impractical and probably unnecessary.
  • Often, restriction of physical activity is self-enforced.
  • Infants might need intravenous alimentation for relief from feeding activity.
  • Apart from the above, activity to the limit of tolerance should be allowed and encouraged.
  • In patients with chronic illness, regular graded exercise has been shown to improve effort tolerance and quality of life.
  • All feasible support should be provided for peer interaction and participation in normal life activities. Restricted life activities, frequent diagnostic and therapeutic interventions, and an uncertain prognosis make these children prone to psychological problems that may significantly influence prognosis and outcome. Among the described abnormalities are inhibition of emotions, marked anxiety, depressive reaction with loneliness, low self-esteem, feelings of inadequacy, emotional lability, impulsiveness, and weakness of self-identity.

Medication

Medical therapy in dilated cardiomyopathy (DCM) is largely directed at the symptoms and is aimed at the underlying heart failure. Diuretics, ACE inhibitors, and beta-blockers form the initial therapy. Diuretics may provide an improvement in symptoms, whereas ACE inhibitors appear to prolong survival. Beta-blockers also have a long-term positive effect on outcome. Intravenous infusions of vasoactive agents may be required in patients with resistant heart failure. Recently, the use of beta-blockers has become more extensive in childhood DCM drug therapy.

Antibiotics for endocarditis prophylaxis are administered to patients with certain cardiac conditions, such as DCM, before performing procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Diuretics

These agents are used to eliminate retained fluid and preload reduction.


Furosemide (Lasix)

This is the DOC. Inhibits reabsorption of fluid from the ascending loop of Henle in the renal tubule. With IV administration, has venodilator action and lowers preload even before diuresis sets in. DOC in acute heart failure and in exacerbations of chronic heart failure. Also used for long-term management of chronic heart failure.

Adult

40 mg PO bid
20-50 mg IV; repeat q6-8h

Pediatric

1-4 mg/kg PO qd or bid
1-4 mg/kg IV q8h

Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides (hearing loss of varying degrees may occur); anticoagulant activity of warfarin may be enhanced when concurrently administered; increased plasma lithium levels and toxicity are possible; risk of hypokalemia with concurrent administration of amiodarone and flecainide; sotalol enhances hypotension and risk of cardiac arrhythmia

Documented hypersensitivity; hepatic coma; anuria; state of severe electrolyte depletion

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Hypokalemia, hyponatremia and hypotension; aggravates diabetes mellitus, porphyria, and liver failure; use with caution in pregnancy and breastfeeding; may cause hyperuricemia; may produce deafness due to ototoxicity; administer oral dose with food or milk to decrease stomach upset


Spironolactone (Aldactone)

Potassium-sparing diuretic that acts on the distal convoluted tubule of the kidney as an aldosterone antagonist. Exhibits synergistic action with furosemide.

Adult

100-200 mg PO qd

Pediatric

0.5-1.5 mg/kg PO bid

May decrease effect of anticoagulants; ACE inhibitors, cyclosporine, potassium, and potassium-sparing diuretics may increase toxicity of spironolactone; may increase the risk of digoxin toxicity

Documented hypersensitivity; anuria, renal failure, hyperkalemia; Addison disease

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

GI upset; hyponatremia; hyperkalemia; hepatotoxicity; lethargy; confusion; impotence; gynecomastia; avoid salt substitutes or natural licorice

Angiotensin-converting enzyme Inhibitors

These drugs reduce afterload and decrease myocardial remodeling that worsens chronic heart failure.


Captopril (Capoten)

Accepted as an essential part of any antifailure therapy; provides symptomatic improvement and prolonged survival; prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

Adult

6.25-25 mg PO tid; not to exceed 150 mg tid

Pediatric

0.1-1 mg/kg PO tid

NSAIDs may reduce hypotensive effects; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics

Documented hypersensitivity; renal impairment; renal artery stenosis

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in renal impairment, valvular stenosis, or severe congestive heart failure; hypotension; tachycardia; renal failure; persistent dry cough has been reported in 5-20% of children


Enalapril (Vasotec)

ACE Inhibitor with prolonged duration of action PO; competitive inhibitor of ACE; reduces angiotensin II levels, decreasing aldosterone secretion.

Adult

20-40 mg PO qd or divided bid

Pediatric

0.1-1 mg/kg/d PO; not to exceed 40 mg per day

NSAIDs may reduce hypotensive effects; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Caution in renal impairment, valvular stenosis, or severe congestive heart failure

Cardiac glycosides

These drugs provide improvement of symptoms with chronic administration. The role of cardiac glycosides is currently less clear than in an earlier era.


Digoxin (Lanoxin)

Improves myocardial contractility, reduces heart rate, and lowers sympathetic stimulation in chronic heart failure. Digoxin inhibits Na+ -K+ ATPase pump. Sodium preferentially exchanges with calcium, increasing the intracellular calcium and resulting in an increase in contractility.

Adult

Total digitalizing dose (TDD): 0.75-1.5 mg PO
50% of TDD initially; remaining 2 doses at 25% TDD q6-12h (1/2, 1/4, 1/4)
Maintenance dose: 0.125-0.5 mg PO qd

Pediatric

Total digitalizing dose (TDD):
Preterm infant: 20-30 mcg/kg PO
Term infant: 25-35 mcg/kg PO
1 month to 2 years: 35-60 mcg/kg PO
2-5 years: 30-40 mcg/kg PO
5-10 years: 20-35 mcg/kg PO
>10 years: Administer as in adults
50% of TDD initially; remaining 2 doses at 25% TDD q6-12h (1/2, 1/4, 1/4)
Maintenance dose:
Preterm infant: 5-7.5 mcg/kg PO divided bid
Term infant: 6-10 mcg/kg PO divided bid
1 month to 2 years: 10-15 mcg/kg PO divided bid
2-5 years: 7.5-10 mcg/kg PO divided bid
5-10 years: 5-10 mcg/kg divided bid
>10 years: Administer as in adults

Medications that may increase levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (including carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid

Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; and carotid sinus syndrome

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Hypokalemia may reduce positive inotropic effect of digitalis; once digitalized, rapid administration of IV calcium may produce serious arrhythmias; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis

Oral anticoagulant

These agents are administered to prevent recurrence of thromboembolic episodes of cardiac origin.


Warfarin (Coumadin)

Interferes with hepatic synthesis of vitamin K-dependent coagulation factors. Prevents thrombus formation within cardiac chambers and venous circulation. Tailor dose to maintain an INR of 2-3.

Adult

5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR

Pediatric

0.05-0.34 mg/kg/d PO; adjust dose according to desired INR

Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate; medications that may increase anticoagulant effects include oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac

Documented hypersensitivity; severe liver or kidney disease; open wounds or GI ulcers

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Do not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients with protein C or protein S deficiency are at risk of developing skin necrosis

Beta-adrenergic blocking agents

These agents block the beta-adrenergic receptor and are modulators of the autonomic system.


Propranolol (Inderal)

Inhibits both beta1- and beta2-adrenergic receptors. Nonselective adrenergic antagonist.

Adult

40-80 mg PO bid initially; increase to 160-320 mg/d (some patients require up to 640 mg/d)

Pediatric

1-4 mg/kg/d PO divided bid/tid

Coadministration with aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease effects; calcium channel blockers, cimetidine, loop diuretics, and MAOIs may increase toxicity; toxicity of hydralazine, haloperidol, benzodiazepines, and phenothiazines may increase

Documented hypersensitivity; uncompensated congestive heart failure; bradycardia; cardiogenic shock; AV conduction abnormalities

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Beta-adrenergic blockade may decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; withdraw drug slowly and closely monitor; gradually taper over 1-2 wk when discontinuing


Carvedilol (Coreg)

Nonselective beta-blocker with additional direct vasodilator action.

Adult

25 mg PO bid; not to exceed 50 mg bid

Pediatric

0.08 mg/kg PO qd initially; increase as tolerated over 12 wk; not to exceed 0.5 mg/kg/d

Coadministration with rifampin may decrease effects; calcium channel blockers, cimetidine, loop diuretics, and MAOIs may increase toxicity

Documented hypersensitivity; uncompensated congestive heart failure; bradycardia; cardiogenic shock; AV conduction abnormalities

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Beta-adrenergic blockade may decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; withdraw drug slowly and closely monitor; gradually taper over 1-2 wk when discontinuing


Metoprolol (Lopressor, Toprol XL)

Selective beta-1 adrenergic receptor blocker that decreases automaticity of contractions.

Adult

Extended release tablets (Toprol XL):
NYHA Class II heart failure: 25 mg PO qd initially
More severe heart failure: 12.5 mg PO qd
May double the dose q2wk as tolerated; not to exceed 200 mg/d

Pediatric

0.1 mg/kg/dose PO bid; dose may be increased as patient tolerates

Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels of metoprolol, possibly resulting in decreased pharmacologic effects; toxicity of metoprolol may increase with coadministration of sparfloxacin, phenothiazines, astemizole, calcium channel blockers, quinidine, flecainide, and contraceptives; metoprolol may increase toxicity of digoxin, flecainide, clonidine, epinephrine, nifedipine, prazosin, verapamil, and lidocaine

Documented hypersensitivity; uncompensated congestive heart failure, bradycardia, asthma, cardiogenic shock, and AV conduction abnormalities

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Beta-adrenergic blockade may reduce signs and symptoms of acute hypoglycemia and may decrease clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; monitor patient closely and slowly withdraw the drug; during IV administration, carefully monitor blood pressure, heart rate, and ECG

Vasoactive infusions

These agents are used in resistant cases as intravenous infusions and are stimulators of beta1-adrenergic receptors in the myocardium. They are also useful for periodic home inotropic therapy in end-stage disease, in which cardiac transplant is not feasible, to improve the quality of life. However, studies have shown increased mortality related to arrhythmogenic potential.


Dobutamine hydrochloride (Dobutrex)

Synthetic catecholamine with potent cardiac-stimulating properties; in addition, has direct vasodilating action on peripheral blood vessels; infusion with or without additional dopamine infusion in renal dose would be appropriate therapy for cardiogenic shock secondary to dilated cardiomyopathy.

Adult

0.5 mcg/kg/min IV infusion initially; titrate to effect; not to exceed 40 mcg/kg/min

Pediatric

Administer as in adults

Beta-adrenergic blockers antagonize effects; general anesthetics may increase toxicity

Documented hypersensitivity; idiopathic hypertrophic subaortic stenosis; atrial fibrillation or flutter

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Close monitoring of heart rate, blood pressure and ECG are advisable during infusion; hypovolemic state should be corrected before use

Phosphodiesterase enzyme inhibitors

These agents elicit positive inotropic and vasodilatory effects.


Milrinone (Primacor)

Bipyridine with positive inotrope and vasodilator activity. Little chronotropic activity observed. Different in mode of action from both digitalis glycosides and catecholamines. Selectively inhibits phosphodiesterase type III (PDE III) in cardiac and smooth vascular muscle, resulting in reduced afterload, reduced preload, and increased inotropy. Not FDA-approved for pediatric patients, although often considered DOC in pediatric patients in ICU setting.

Adult

50 mcg/kg IV loading dose over 10 min followed by continuous IV infusion at 0.375-0.75 mcg/kg/min

Pediatric

Limited data exist; administer as in adults

Incompatible with furosemide when administered within same IV (forms precipitates)

Documented hypersensitivity to milrinone, any component, or inamrinone

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Monitor fluids, electrolyte changes and renal function during therapy; excessive diuresis may increase potassium loss and predispose patients who are digitized to arrhythmias; important to correct hypokalemia with potassium supplementation prior to treatment; patients showing excessive decreases in blood pressure should have infusion rates slowed or stopped; previous vigorous diuretic therapy has caused significant decreases in cardiac filling pressure, cautiously administer milrinone and monitor blood pressure, heart rate, and clinical symptomatology

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, 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

[ 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, 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.

 
 
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.