title: Natural History of Dilated Cardiomyopathy in Children.
abstract: The long-term progression of idiopathic dilated cardiomyopathy (DCM) in pediatric patients compared with adult patients has not been previously characterized. In this study, we compared outcome and long-term progression of pediatric and adult DCM populations.
  
  
  
  Between 1988 and 2014, 927 DCM patients were consecutively enrolled. The pediatric population (aged <18 years at enrollment) included 47 participants (5.1%). At presentation, the pediatric population compared with adult patients had a significantly increased occurrence of familial forms (P=0.03), shorter duration of heart failure (P=0.04), lower systolic blood pressure (P=0.01), decreased presence of left bundle-branch block (P=0.001), and increased left ventricular ejection fraction (P=0.03). Despite these baseline differences, long-term longitudinal trends of New York Heart Association class III to IV, left ventricular dimensions, left ventricular ejection fraction, and restrictive filling pattern were similar between the 2 populations. Regarding survival analysis, because of the size difference between the 2 populations, we compared the pediatric population with a sample of adult patients randomly matched using the above-mentioned baseline differences in a 3:1 ratio (141 adult versus 47 pediatric patients). During a median follow-up of 110 months, survival free from heart transplantation was significantly lower among pediatric patients compared with adults (P<0.001). Furthermore, pediatric age (ie, <18 years) was found to be associated with an increasing risk of both death from pump failure and life-threatening arrhythmias.
  
  
  
  Despite the pediatric DCM population having higher baseline left ventricular ejection fraction and similar long-term echocardiographic progression compared with the adult DCM population, the pediatric DCM patients had worse cardiovascular prognosis.
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Puggia
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy marco.merlo79@gmail.com.
authors.forename: Marco
authors.initials: M
authors.lastname: Merlo
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy Cardiovascular Center, Azienda per i Servizi Sanitari N°1, Trieste, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Barbati
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics Program, University of Colorado Denver Anschutz Medical Campus, Aurora, CO.
authors.forename: Teisha J
authors.initials: TJ
authors.lastname: Rowland
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Stolfo
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Gigli
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Ramani
authors.affiliation: Cardiovascular Center, Azienda per i Servizi Sanitari N°1, Trieste, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Lenarda
authors.affiliation: Cardiovascular Institute and Adult Medical Genetics Program, University of Colorado Denver Anschutz Medical Campus, Aurora, CO.
authors.forename: Luisa
authors.initials: L
authors.lastname: Mestroni
authors.affiliation: Cardiovascular Department, Azienda Ospedaliero-Universitaria "Ospedali Riuniti" of Trieste, Trieste, Italy.
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Sinagra
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001145:Arrhythmias, Cardiac / Q000453:epidemiology; D001794:Blood Pressure; D002037:Bundle-Branch Block / Q000453:epidemiology; D002311:Cardiomyopathy, Dilated / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy; D002648:Child; D016757:Death, Sudden, Cardiac / Q000453:epidemiology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000453:epidemiology*; D016027:Heart Transplantation; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries; D013318:Stroke Volume; D013997:Time Factors
keywords: cardiomyopathy; death; dilated; echocardiography; heart failure; pediatrics; sudden