Abstract
Dilated cardiomyopathy (DCM) refers to a group of conditions of diverse etiology in which both ventricles are enlarged with reduced contractility. Certain correctable conditions associated with ventricular dysfunction can masquerade as DCM. Most of them can be identified with relatively inexpensive and readily available tests. A typical diagnostic work-up for a child with DCM also includes a number of investigations to identify the underlying cause, some of which are expensive and sophisticated. The average center in the developing world often does not have the facilities to carry out these investigations. The results of many of these investigations typically do not translate into a specific management strategy that makes a difference to prognosis. A significant number of children with DCM will eventually develop end-stage heart failure that requires cardiac transplantation with or without bridging procedures. This is an unrealistic option for the developing world. The management strategy of childhood DCM in the developing world needs to be tailored to the resources available with in a manner such that the overall prognosis is not substantially affected.
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Fujioka S, Kitaura Y, Deguchi H, Kawamura K, Isomura T, Suma Het al. Evaluation of viral infection in the myocardium of patients with idiopathic dilated cardiomyopathy.A J Coll Cardiol 2000; 36:1920–1926.
Kasper ED, Agema WRP, Hutchins GMet al. The causes of dilated cardiomyopathy: A clinicopathologic review of 673 consecutive patients.J Am Coll Cardiol 1994; 23: 586–590.
Matitiau A, Perez-Atayde A, Sanders SPet al. Infantile dilated cardiomyopathy relationof outcome to left evntricular mechanics, hemodynamics and histology at the time of presentation.Circulation 1994; 90:1310.
Arola A, Jokinene E, Ruuskanen Oet al. Epidemiology of idiopathic cardiomyopathies in children and adolescents: a nationwide study in Finland.Am J Epidemiol 1997; 146: 385–393.
Daubeney PEF, Nugent A, Davis AM, Wilkinson JL, Weintraub RJ. On behalf of the National Australian Cardiomyopathy Study. Incidence and outcome of childhood cardiomyopathy in Australia: results of a ten-year population-based study [abstract].J Am Coll Cardiol 1999; 33 (suppl A): 496A.
Towbin JA. Pediatric myocardial disease.Pediatr Clin North Am 1999; 46: 289–312.
The Consensus trial study group: Effects of enalapril on mortality in severe congestive heart failure. Results of the Cooperative North Scandinavian Enalapril Survival Study.N Engl J Med 1987; 316:1429.
The SOLVD Investigators : the effect of enalapril on survival in patient with reduced left ventricular ejection fraction and congestive heart failure. NEng J Med 1991; 325–293.
Gersony WM. Major advances in pediatric cardiology in the 20th century: II.Therapeutics J Pediatr 2001; 139: 328–333.
Prabhu SS, Delvi BV. Treatable cardiomyopathies.Indian J Pediatr 2000; 67: 279–282.
Schwartz ML, Cox GF, Lin AEet al. Clinical Approach to Genetic Cardiomyopathy in Children.Circulation 1996; 94: 2021–2038.
Ichida F, Hamamichi Y, Miyawaki Tet al. Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background.J Am Coll Cardiol 1999; 34: 233–240.
Barth PG, Wanders RJA, Vreken P. X-linked cardioskeletal myopathy and neutropenia (Barth syndrome)-MIM 302060.J Pediatr 1999; 135: 273–276.
Nigro G, Comi LI, Politano Let al. The incidence and evoluation of cardiomyopathy in Duchenne muscular dystrophy.Int J Cardiol 1990; 26: 271–277.
Towbin JA, Hejtmancik F, Brink Pet al. X-linked cardiomyopathy (XLCM): Molecular genetic evidene of linkage to the Duchenne muscular dystrophy (dystrophin) gene at the Xp21 locus.Circulation 1993; 87 :854–1865.
Lauer B, Niederau C, Kuhl Uet al. Cardiac troponin T in patients with clinically suspected myocarditis.J Am Coll Cardiol 1997; 30(5): 1354–1359.
Kubo N, Morimoto S, Hiramitsu Set al. Feasibility of diagnosing chronic myocarditis by endomyocardial biopsy.Heart Vessels 1997; 12:167–170.
Pophal SG, Sigfusson G, Booth ELet al. Complications of endomyocardial biopsy in children.J Am Coll Cardiol 1999; 34: 2105–2110.
Narayan R, Menahem S, Chow CW, Dennett X. Endomyocardial biopsy in infants and children with cardiomyopathy.Clin Cardiol 1991; 14 (11): 903–907.
Webber SA, Boyle GJ, Jaffe R, Pickering RM, Beerman LB, Flicker FJ. Role of right ventricular endomyocardial biopsy in infants and children with suspected or possible myocarditis.Br Heart J 1994; 72(4): 360–363.
Mason JW, O’connel JB, Herskowitz Aet al. A clinical trial of immunosuppressive therapy for myocarditis.N Eng J Med 1995; 333: 269.
Camargo PR, Snitcowsky R, da Luz PLet al. Favorable effects 350 of immunosuppressive therapy in children with delated cardiomyopathy and active myocarditis.Pediatr Cardiol 1995; 16: 61–68.
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 1997; 16: 1248–1254.
Martin AB, Webber S, Fricker JFet al. Acute myocarditis. Rapid diagnosis by PCR in children.Circulation 1994; 90: 330–339.
Keeling PJ, Gang G, Smith Get al. Familial dilated cardiomyopathy in the United Kingdom.Br Heart j 1995; 73: 417–421.
Michels W, Moll PP, Miller FAet al. The frequency of familial dilated cardiomyopathy in a series of patients with idiopathic dilated cardiomyopathy.N Engl J Med 1992; 326: 77–82.
Mestroni L, Krajinovic M, Severini GMet al. Familial dilated cardiomyopathy.Br Heart J 1994; 72: 35–41.
Bengur AR, Beekman RH, Rocchini AP, Crowley DC, Schork MA, Rosenthal A. Acute hemodynamic effets of captopril in children with a congestive or restrictive cardiomyopathy.Circulation 1991; 83: 523–527.
Beta-blocker therapy in young children with congestive heart failure under consideration for heart transplantation.Am Heart J 1998; 136 : 19–21.
Bruns LA, Chrisant MK, Lamour JMet al. Carvedilol as therapy in pediatric heart failure: an initial multicenter experience.J Pediatr 2001; 138: 505–511.
Gachara N, Prabhakaran S, Srinivas S, Farzana F, Krishnan U, Shah MJ. Efficacy and safety of carvedilol in infants with dilated cardiomyopathy: a preliminary report.Indian Heart J 2001; 53(1): 74–78.
Karpati G, Carpenter S, Engel AGet al. The syndrome of systemic carnitine deficiency: clinical, morphologic, biochemica, and pathophysiologic features.Neurology 1975; 25:16–24.
Rebouche CJ, Engel AG. Carnitine metabolism and deficiency syndromes.Mayo Clin Proc. 1983; 58: 533–540.
Helton E, Darragh R, Francis Pet al. Metabolic Aspects of Myocardial Disease and a Role for L-Carnitine in the Treatment of Childhood Cardiomyopathy.Pediatrics 2000; 105:1260–1270.
Kothari SS, Sharma M. L-carnitine in children with idiopathic dilated cardiomyopathy.Indian Heart J 1998; 50: 59–61.
Drucker NA, Colan SD, Lewis ABet al. Gamma-globulin treatment of acute myocarditis in the pediatric population.Circulation 1994; 89: 252.
Batista RJ. Reduction ventriculoplasty.Z Kardiol 2001; 90:35–37.
Gradinac S, Jovanovic I, Dukic Met al. Partial left ventriculectomy in a two-year-old girl with dilated cardiomyopathy.J Heart lung Transplant 1999; 18: 610.
Hosenpud JD, Novick RJ, Breen TJ, Daily OP. The Registry of the International Society for Heart and Lung Transplantation: eleventh official report-1994.J Heart Lung Transplant 1994; 13: 561–570.
Taliercio CP, Seward JB, Driscoll DJet al. Idiopathic dilated cardiomyopathy in the young: Clinical profile and natural history.J Am Coll Cardiol 1985; 6: 1126–1131.
Wiles HB, McArthur PD, Taylor ABet al. Prognostic features of children with dilated cardiomyopathy.Am J Cardiol 1991; 68: 1372–1376.
Akagi T, Benson LN, Ligthfoot NEet al. Natural history of dilated cardiomyopathy in children.Am Heart J 1991; 121: 1502–1506.
Lewis AB, Chabot BS. Outcome of infants and childlren with dilated cardiomyopathy.Am J Cardiol 1991; 68: 365–369.
Bruch M, Siddiqui, Celermajar DSet al. Dilated cardiomyopathy in children determinants of outcome.Br Heart J 1994; 72: 246–250.
Kumar K, Thatia D, Saxena Aet al. Pediatrie dilated cardiomyopathy (DCM): prognosis in a developing nation is comparable to developed nations [abstract].J Am Coll Cardiol 1995; 27: 187A.
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Kumar, R.K. A practical approach for the diagnosis and management of dilated cardiomyopathy. Indian J Pediatr 69, 341–350 (2002). https://doi.org/10.1007/BF02723221
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DOI: https://doi.org/10.1007/BF02723221