title: Myocarditis in children with dilated cardiomyopathy: incidence and outcome after dual therapy immunosuppression.
abstract: BACKGROUND
  The true incidence and prognosis of myocarditis in children with acute dilated cardiomyopathy (DCM) at presentation remains uncertain. This study examines the incidence of lymphocytic myocarditis in a consecutive cohort of children with acute DCM at presentation and outcome after dual therapy immunosuppression with cyclosporine and steroids.
  
  
  METHODS
  Twenty-nine consecutive children with acute DCM underwent early endomyocardial biopsy. Children with "definite" myocarditis comprised group I (n = 9) and were treated with cyclosporine and prednisolone. Group II (n = 2) had "borderline" myocarditis, and group III (n = 18) nonspecific histologic findings. Outcome was assessed by echocardiographic measurement of left ventricular end-diastolic dimension and fractional shortening, with follow-up endomyocardial biopsy in group I subjects.
  
  
  RESULTS
  Myocardial inflammation with or without myocardial necrosis (groups I and II) was present in 38% of all cases. There were no initial clinical, electrocardiographic, or echocardiographic features to distinguish patients in group I from patients in group III. At presentation, the mean +/- SEM left ventricular end-diastolic dimension and fractional score-Z scores of group I patients were 4.6 +/- 1.7 and -5.1 +/- 0.8, respectively, compared with 0.8 +/- 0.3 and -0.9 +/- 0.4, respectively, at withdrawal of immunosuppression (p < 0.001 for both). Both of these parameters did not differ significantly from normal controls at least follow up. Two group I patients had a biopsy-proven relapse after withdrawal of therapy that responded to reinstitution of immunosuppression. At latest follow-up, all nine group I patients had regained normal left ventricular function compared with four of 18 group III patients (p < 0.001).
  
  
  CONCLUSIONS
  Lymphocytic myocarditis is frequent in children with dilated cardiomyopathy and cannot be predicted from noninvasive investigations. The use of cyclosporine and steroids is associated with a favorable outcome, and a controlled trial of dual therapy immunosuppression in children is therefore warranted.
authors.affiliation: Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
authors.forename: S
authors.initials: S
authors.lastname: Kleinert
authors.forename: R G
authors.initials: RG
authors.lastname: Weintraub
authors.forename: J L
authors.initials: JL
authors.lastname: Wilkinson
authors.forename: C W
authors.initials: CW
authors.lastname: Chow
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D002675:Child, Preschool; D018849:Controlled Clinical Trials as Topic; D016572:Cyclosporine / Q000627:therapeutic use; D003971:Diastole; D004452:Echocardiography; D004562:Electrocardiography; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D007223:Infant; D008214:Lymphocytes / Q000473:pathology; D008775:Methylprednisolone / Q000627:therapeutic use; D009200:Myocardial Contraction; D009205:Myocarditis / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D009206:Myocardium / Q000473:pathology; D009336:Necrosis; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012008:Recurrence; D016896:Treatment Outcome; D016277:Ventricular Function, Left
chemical_list: D000893:Anti-Inflammatory Agents; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone; D008775:Methylprednisolone