title: [Dilated cardiomyopathy in childhood].
abstract: In childhood, dilative cardiomyopathy (DCM) has a prevalence of 2.6 patients in 100,000 inhabitants. Manifestation age is, in 75%, the first two years of life. There are no specific symptoms. Diagnosis is often made, when congestive heart failure occurs. Despite intensive therapy with digitalis, diuretics and ACE inhibitors, DCM is the main indication for heart transplantation. The prognosis is critical: 11 studies with approximately 450 children showed a mean 1-year survival rate of 75% and a 5-year survival rate of 60%. In most cases etiology of DCM remains unclear ("idiopathic"). In the second position (approximately 40%) is inflammatory pathogenesis. While familiar DCM is manifested mainly during the 3rd and 4th decade, specific cardiomyopathy is often found during infancy: inborn errors of metabolism, neuromuscular diseases or malformation syndromes are the causes of a cardiomyopathy, which can appear as dilative or hypertrophic cardiomyopathy. A causal therapy exists only in a few cases.
authors.affiliation: Abt. Pädiatrische Kardiologie Universitäts-Kinderklinik Hufelandstr, 55 45122 Essen. achim.schmaltz@uni-essen.de
authors.forename: A A
authors.initials: AA
authors.lastname: Schmaltz
mesh_terms: D002311:Cardiomyopathy, Dilated / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D002317:Cardiovascular Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006333:Heart Failure / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D015996:Survival Rate
chemical_list: D002317:Cardiovascular Agents