Introduction

Myocardial fibrosis can lead to heart failure and act as a substrate for cardiac arrhythmias. In dilated cardiomyopathy diffuse interstitial reactive fibrosis can be observed, whereas arrhythmogenic cardiomyopathy is characterized by fibrofatty replacement in predominantly the right ventricle. The p.Arg14del mutation in the phospholamban (PLN) gene has been associated with dilated cardiomyopathy and recently also with arrhythmogenic cardiomyopathy. Aim of the present study is to determine the exact pattern of fibrosis and fatty replacement in PLN p.Arg14del mutation positive patients, with a novel method for high resolution systematic digital histological quantification of fibrosis and fatty tissue in cardiac tissue. Transversal mid-ventricular slices (n = 8) from whole hearts were collected from patients with the PLN p.Arg14del mutation (age 48±16 years; 4 (50%) male). An in-house developed open source MATLAB script was used for digital analysis of Masson's trichrome stained slides (http://sourceforge.net/projects/fibroquant/). Slides were divided into trabecular, inner and outer compact myocardium. Per region the percentage of connective tissue, cardiomyocytes and fatty tissue was quantified. In PLN p.Arg14del mutation associated cardiomyopathy, myocardial fibrosis is predominantly present in the left posterolateral wall and to a lesser extent in the right ventricular wall, whereas fatty changes are more pronounced in the right ventricular wall. No difference in distribution pattern of fibrosis and adipocytes was observed between patients with a clinical predominantly dilated and arrhythmogenic cardiomyopathy phenotype. In the future, this novel method for quantifying fibrosis and fatty tissue can be used to assess cardiac fibrosis and fatty tissue in animal models and a broad range of human cardiomyopathies.

Publications

  1. High resolution systematic digital histological quantification of cardiac fibrosis and adipose tissue in phospholamban p.Arg14del mutation associated cardiomyopathy.
    Cite this
    Gho JM, van Es R, Stathonikos N, Harakalova M, te Rijdt WP, Suurmeijer AJ, van der Heijden JF, de Jonge N, Chamuleau SA, de Weger RA, Asselbergs FW, Vink A, 2014-01-01 - PloS one

Credits

  1. Johannes M I H Gho
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  2. René van Es
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  3. Nikolas Stathonikos
    Developer

    Department of Pathology, University Medical Center Utrecht

  4. Magdalena Harakalova
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  5. Wouter P te Rijdt
    Developer

    Department of Cardiology, University Medical Center Groningen

  6. Albert J H Suurmeijer
    Developer

    Department of Pathology, University Medical Center Groningen

  7. Jeroen F van der Heijden
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  8. Nicolaas de Jonge
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  9. Steven A J Chamuleau
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  10. Roel A de Weger
    Developer

    Department of Pathology, University Medical Center Utrecht

  11. Folkert W Asselbergs
    Developer

    Department of Cardiology, Division Heart and Lungs, United Kingdom of Great Britain and Northern Ireland

  12. Aryan Vink
    Investigator

    Department of Pathology, University Medical Center Utrecht

Community Ratings

UsabilityEfficiencyReliabilityRated By
0 user
Sign in to rate
Summary
AccessionBT003511
Tool TypeApplication
Category
PlatformsWindows
Technologies
User InterfaceTerminal Command Line
Download Count0
Submitted ByAryan Vink