title: Paediatric dilated cardiomyopathy: clinical profile and outcome. The experience of a tertiary centre for paediatric cardiology.
abstract: Dilated cardiomyopathy is the most common form of cardiomyopathy in the paediatric population and an important cause of heart transplantation in children. The clinical profile and course of dilated cardiomyopathy in children have been poorly characterised. A retrospective review of 61 patients (37 female; 24 male) diagnosed with dilated cardiomyopathy from January, 2005 to June, 2012 at a single institution was performed. The median age at diagnosis was 15 months. Heart failure was present in 83.6% of patients and 44.3% required intensive care. The most prevalent causes were idiopathic (47.5%), viral myocarditis (18.0%) and inherited metabolic diseases (11.5%). In viral myocarditis, Parvovirus B19 was the most common identified agent, in concurrence with the increasing incidence documented recently. Inherited metabolic diseases were responsible for 11.5% of dilated cardiomyopathy cases compared with the 4-6% described in the literature, which reinforces the importance of considering this aetiology in differential diagnosis of paediatric dilated cardiomyopathy. The overall mortality rate was 16.1% and five patients underwent heart transplantation. In our series, age at diagnosis and aetiology were the most important prognosis factors. We report no mortality in the five patients who underwent heart transplantation, after 2 years of follow-up.
authors.affiliation: 1Department of Paediatric Cardiology,Centro Hospitalar São João,Porto,Portugal.
authors.forename: Joana O
authors.initials: JO
authors.lastname: Miranda
authors.affiliation: 2Department of Pediatrics,Centro Hospitalar São João,Porto,Portugal.
authors.forename: Liane
authors.initials: L
authors.lastname: Costa
authors.affiliation: 3Department of Pediatrics,Metabolic Diseases Unit,Centro Hospitalar São João,Porto,Portugal.
authors.forename: Esmeralda
authors.initials: E
authors.lastname: Rodrigues
authors.affiliation: 3Department of Pediatrics,Metabolic Diseases Unit,Centro Hospitalar São João,Porto,Portugal.
authors.forename: Elisa L
authors.initials: EL
authors.lastname: Teles
authors.affiliation: 1Department of Paediatric Cardiology,Centro Hospitalar São João,Porto,Portugal.
authors.forename: Maria J
authors.initials: MJ
authors.lastname: Baptista
authors.affiliation: 1Department of Paediatric Cardiology,Centro Hospitalar São João,Porto,Portugal.
authors.forename: José C
authors.initials: JC
authors.lastname: Areias
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D002311:Cardiomyopathy, Dilated / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D002316:Cardiotonic Agents / Q000627:therapeutic use; D002675:Child, Preschool; D015331:Cohort Studies; D003422:Critical Care; D003586:Cytomegalovirus Infections / Q000150:complications*; D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D006333:Heart Failure / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007251:Influenza, Human / Q000150:complications*; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D009205:Myocarditis / Q000150:complications* / Q000821:virology; D010322:Parvoviridae Infections / Q000150:complications* / Q000821:virology; D016732:Parvovirus B19, Human; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D062606:Tertiary Care Centers
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D002121:Calcium Channel Blockers; D002316:Cardiotonic Agents; D004232:Diuretics; D010975:Platelet Aggregation Inhibitors
keywords: metabolic disease