title: Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young.
abstract: Hypertrophic cardiomyopathy (HCM) remains the leading cause of sudden cardiac death in the young. Early markers for HCM are important to identify individuals at risk. The aim of this study was to investigate novel serum biomarkers reflecting myocardial remodeling, microfibrosis, and vascular endotheliopathy in the early stages of familial HCM in young patients. Twenty-three HCM patients, 16 HCM-risk individuals, and 66 controls (median 15 years) underwent echocardiography and serum analysis for cathepsin S, endostatin, myostatin, type I collagen degradation marker (ICTP), matrix metalloproteinase (MMP)-9, vascular endothelial growth factor receptor (VEGFR)-1, and vascular and intercellular adhesion molecules (VCAM, ICAM). In a subset of the population, global myocardial perfusion was performed by magnetic resonance imaging. Cathepsin S (p = 0.0009), endostatin (p < 0.0001), MMP-9 (p = 0.008), and VCAM (p = 0.04) were increased in the HCM group and correlated to left ventricular mass index and mitral E/e' (p < 0.01). In the HCM-risk group, myostatin was decreased (p = 0.004), whereas ICAM was increased (p = 0.002). Global perfusion was decreased in the HCM group (p < 0.05) versus controls. Endostatin and mitral E/e' correlated inversely to myocardial perfusion (p ≤ 0.05). This is the first study demonstrating adverse changes in biomarkers reflecting myocardial matrix remodeling, microfibrosis, and vascular endotheliopathy in early stage of hypertrophic cardiomyopathy in the young.
authors.affiliation: Pediatric Heart Center, Skane University Hospital, Lund University, Lund, Sweden. eva.fernlund@gmail.com.|Department of Paediatrics, Linköping University Hospital, Linköping University, Linköping, Sweden. eva.fernlund@gmail.com.
authors.forename: E
authors.initials: E
authors.lastname: Fernlund
authors.affiliation: Department of Clinical Sciences, Lund University Hospital, Lund University, Lund, Sweden.
authors.forename: T
authors.initials: T
authors.lastname: Gyllenhammar
authors.affiliation: Department of Clinical Sciences, Lund University Hospital, Lund University, Lund, Sweden.
authors.forename: R
authors.initials: R
authors.lastname: Jablonowski
authors.affiliation: Department of Clinical Sciences, Lund University Hospital, Lund University, Lund, Sweden.
authors.forename: M
authors.initials: M
authors.lastname: Carlsson
authors.affiliation: Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Larsson
authors.affiliation: Department of Medical Sciences, Cardiovascular Epidemiology, Uppsala University, Uppsala, Sweden.|Department of Neurobiology, Division of Family Medicine and Primary Care, Care Science and Society, Karolinska Institutet, Huddinge, Sweden.
authors.forename: J
authors.initials: J
authors.lastname: Ärnlöv
authors.affiliation: Pediatric Heart Center, Skane University Hospital, Lund University, Lund, Sweden.
authors.forename: P
authors.initials: P
authors.lastname: Liuba
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D002312:Cardiomyopathy, Hypertrophic / Q000097:blood* / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003324:Coronary Artery Disease / Q000097:blood* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D004730:Endothelium, Vascular / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D005355:Fibrosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007249:Inflammation / Q000097:blood / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D008297:Male; D009206:Myocardium / Q000473:pathology*; D018487:Ventricular Dysfunction, Left / Q000097:blood* / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D020257:Ventricular Remodeling / Q000502:physiology*; D055815:Young Adult
chemical_list: D015415:Biomarkers
keywords: Biomarkers; Early stage; Hypertrophic cardiomyopathy; Myocardium; Risk