Cardiomyopathy in Children

Overview

Journal Curr Treat Options Cardiovasc Med

Specialty Cardiology & Vascular Diseases

Date 2010 Sep 18

PMID 20848351

Authors

S C Chen

Affiliations

Soon will be listed here.

Abstract

| Cardiomyopathy in children is a rare but serious disease leading to heart failure with a high mortality rate. Dilated cardiomyopathy (DCM) and hypertrophic cardio-myopathy (HCM) are more common than restrictive cardiomyopathy (RCM). Arrhythmogenic right ventricular dysplasia (ARVD) is extremely rare in pediatric age groups. Treatment should be targeted at the cause of the cardiomyopathy, if identified. Nutritional deficiencies, metabolic disorders, and inborn errors of metabolism should be corrected if possible. Immunosuppressive therapy may be considered in patients with lymphocytic myocarditis. Maximal medical therapy with inotropic drugs or cardiac glycosides, diuretic drugs, and angiotensin-converting enzyme inhibitors improve heart failure symptoms in patients with DCM. Beta-blocker therapy in adults with DCM is well established and is being evaluated in children with DCM. High-dosage beta-blocker or calcium channel blocker therapy improves the survival rate in children with HCM. The cautious use of diuretics and angiotensin-converting enzyme inhibitors in patients with RCM improves symptoms. Anticoagulation to prevent thromboembolism in patients with DCM and RCM is indicated. Antiarrhythmia therapy is needed in patients with DCM- or HCM-associated dysrhythmia. Cardiac transplant is indicated in patients with progressive deterioration.

References

Denfield S, Rosenthal G, Gajarski R, Bricker J, Schowengerdt K, Price J . Restrictive cardiomyopathies in childhood. Etiologies and natural history. Tex Heart Inst J. 1997; 24(1):38-44. PMC: 325396. View

Maron B, Shen W, Link M, EPSTEIN A, Almquist A, Daubert J . Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000; 342(6):365-73. DOI: 10.1056/NEJM200002103420601. View

Corrado D, Basso C, Thiene G, McKenna W, Davies M, Fontaliran F . Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997; 30(6):1512-20. DOI: 10.1016/s0735-1097(97)00332-x. View

Packer M, Bristow M, Cohn J, Colucci W, Fowler M, Gilbert E . The effect of carvedilol on morbidity and mortality in patients with chronic heart failure. U.S. Carvedilol Heart Failure Study Group. N Engl J Med. 1996; 334(21):1349-55. DOI: 10.1056/NEJM199605233342101. View

Kleinert S, Weintraub R, Wilkinson J, Chow C . Myocarditis in children with dilated cardiomyopathy: incidence and outcome after dual therapy immunosuppression. J Heart Lung Transplant. 1998; 16(12):1248-54. View

Kakkis E, Muenzer J, Tiller G, Waber L, Belmont J, Passage M . Enzyme-replacement therapy in mucopolysaccharidosis I. N Engl J Med. 2001; 344(3):182-8. DOI: 10.1056/NEJM200101183440304. View

Kushwaha S, Fallon J, Fuster V . Restrictive cardiomyopathy. N Engl J Med. 1997; 336(4):267-76. DOI: 10.1056/NEJM199701233360407. View

Bonne G, Carrier L, Richard P, Hainque B, Schwartz K . Familial hypertrophic cardiomyopathy: from mutations to functional defects. Circ Res. 1998; 83(6):580-93. DOI: 10.1161/01.res.83.6.580. View

Schwartz M, Cox G, Lin A, Korson M, Lacro R, Lipshultz S . Clinical approach to genetic cardiomyopathy in children. Circulation. 1996; 94(8):2021-38. DOI: 10.1161/01.cir.94.8.2021. View

10.

Peters C, Balthazor M, Shapiro E, King R, Kollman C, Hegland J . Outcome of unrelated donor bone marrow transplantation in 40 children with Hurler syndrome. Blood. 1996; 87(11):4894-902. View

11.

Cox G, Souri M, Aoyama T, Rockenmacher S, Varvogli L, Rohr F . Reversal of severe hypertrophic cardiomyopathy and excellent neuropsychologic outcome in very-long-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1998; 133(2):247-53. DOI: 10.1016/s0022-3476(98)70228-8. View

12.

Ostman-Smith I, Wettrell G, Riesenfeld T . A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose beta-adrenoceptor antagonist treatment. J Am Coll Cardiol. 1999; 34(6):1813-22. DOI: 10.1016/s0735-1097(99)00421-0. View

13.

Rothenberg M . Eosinophilia. N Engl J Med. 1998; 338(22):1592-600. DOI: 10.1056/NEJM199805283382206. View

14.

Nagueh S, Lakkis N, Middleton K, Killip D, Zoghbi W, Quinones M . Changes in left ventricular diastolic function 6 months after nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy. Circulation. 1999; 99(3):344-7. DOI: 10.1161/01.cir.99.3.344. View

15.

Yetman A, Hamilton R, Benson L, McCrindle B . Long-term outcome and prognostic determinants in children with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1998; 32(7):1943-50. DOI: 10.1016/s0735-1097(98)00493-8. View

16.

Rivenes S, Kearney D, Smith E, Towbin J, Denfield S . Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy. Circulation. 2000; 102(8):876-82. DOI: 10.1161/01.cir.102.8.876. View

17.

Ino T, Sherwood W, Benson L, Wilson G, Freedom R, Rowe R . Cardiac manifestations in disorders of fat and carnitine metabolism in infancy. J Am Coll Cardiol. 1988; 11(6):1301-8. DOI: 10.1016/0735-1097(88)90296-3. View

18.

Pierpont M, Breningstall G, Stanley C, Singh A . Familial carnitine transporter defect: A treatable cause of cardiomyopathy in children. Am Heart J. 2000; 139(2 Pt 3):S96-S106. DOI: 10.1067/mhj.2000.103921. View

19.

Camargo P, SNITCOWSKY R, Luz P, Mazzieri R, Higuchi M, Rati M . Favorable effects of immunosuppressive therapy in children with dilated cardiomyopathy and active myocarditis. Pediatr Cardiol. 1995; 16(2):61-8. DOI: 10.1007/BF00796819. View

20.

Towbin J . Pediatric myocardial disease. Pediatr Clin North Am. 1999; 46(2):289-312, ix. DOI: 10.1016/s0031-3955(05)70119-x. View