title: Procollagen type III amino-terminal propeptide: a serum biomarker of left ventricular remodelling in paediatric dilated cardiomyopathy.
abstract: BACKGROUND
  Procollagen type III amino-terminal propeptide is a collagen III cleavage product released in blood. The serum levels of this propeptide in adults with dilated cardiomyopathy are associated with cardiac remodelling and prognosis. The utility of procollagen type III amino-terminal propeptide as a biomarker in paediatric dilated cardiomyopathy is unknown.
  
  
  METHODS
  This was a prospective, longitudinal study of children with dilated cardiomyopathy and changes in procollagen type III amino-terminal propeptide. The serum level of propeptide was measured serially, compared with paediatric normal values, and correlated with clinical status and left ventricular size and function on echocardiograms and cardiac magnetic resonance imaging.
  
  
  RESULTS
  Procollagen type III amino-terminal propeptide was measured serially in 149 samples from 39 patients, age 9.0±6.4 years, followed up for 16.8±16.3 months. Procollagen type III amino-terminal propeptide in dilated cardiomyopathy was higher than in normal children. On multivariate analyses, procollagen type III amino-terminal propeptide had a positive correlation with left ventricular dilation, left ventricular end-diastolic diameter index (p<0.0001), and left ventricular end-diastolic diameter Z-score (p=0.0003), and a negative correlation with shortening fraction changes over time (p=0.001). Patients with myocarditis (n=12) had higher procollagen type III amino-terminal propeptide values than those with idiopathic dilated cardiomyopathy (n=20).
  
  
  CONCLUSIONS
  Procollagen type III amino-terminal propeptide increases with left ventricular dilation and decreases with improvement in systolic function in paediatric dilated cardiomyopathy, indicating a role as a biomarker of cardiac remodelling in children. The diagnostic utility of procollagen type III amino-terminal propeptide to differentiate myocarditis from idiopathic dilated cardiomyopathy warrants further investigation.
authors.affiliation: 1Department of Pediatrics,Division of Cardiology,Lucille Packard Children's Hospital,Stanford University School of Medicine,Palo Alto,United States of America.
authors.forename: Beth D
authors.initials: BD
authors.lastname: Kaufman
authors.affiliation: 2Clinical Trials Office,Children's Hospital of Philadelphia,Philadelphia,United States of America.
authors.forename: Nancy
authors.initials: N
authors.lastname: Videon
authors.affiliation: 3Department of Biostatistics and Data Management,Children's Hospital of Philadelphia,Westat,Philadelphia,United States of America.
authors.forename: Xuemei
authors.initials: X
authors.lastname: Zhang
authors.affiliation: 4Department of Pediatrics,Division of Cardiology,Children's Hospital of Philadelphia,Perelman School of Medicine,University of Pennsylvania,Philadelphia,United States of America.
authors.forename: Matthew A
authors.initials: MA
authors.lastname: Harris
authors.affiliation: 4Department of Pediatrics,Division of Cardiology,Children's Hospital of Philadelphia,Perelman School of Medicine,University of Pennsylvania,Philadelphia,United States of America.
authors.forename: Robert E
authors.initials: RE
authors.lastname: Shaddy
authors.affiliation: 4Department of Pediatrics,Division of Cardiology,Children's Hospital of Philadelphia,Perelman School of Medicine,University of Pennsylvania,Philadelphia,United States of America.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Goldmuntz
mesh_terms: D000293:Adolescent; D015415:Biomarkers; D002311:Cardiomyopathy, Dilated / Q000097:blood* / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009205:Myocarditis / Q000097:blood; D010446:Peptide Fragments / Q000097:blood*; D011347:Procollagen / Q000097:blood*; D011446:Prospective Studies; D014463:Ultrasonography; D020257:Ventricular Remodeling
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide
keywords: myocarditis