TY - JOUR
T1 - Current understanding of fibrosis in genetic cardiomyopathies
AU - Eijgenraam, Tim R
AU - Silljé, Herman H W
AU - de Boer, Rudolf A
N1 - Copyright © 2019. Published by Elsevier Inc.
PY - 2020/8
Y1 - 2020/8
N2 - Myocardial fibrosis is the excessive deposition of extracellular matrix proteins, including collagens, in the heart. In cardiomyopathies, the formation of interstitial fibrosis and/or replacement fibrosis is almost always part of the pathological cardiac remodeling process. Different forms of cardiomyopathies show particular patterns of myocardial fibrosis that can be considered as distinctive hallmarks. Although formation of fibrosis is initially aimed to be a reparative mechanism, in the long term, on-going and excessive myocardial fibrosis may lead to arrhythmias and stiffening of the heart wall and subsequently to diastolic dysfunction. Ultimately, adverse remodeling with progressive myocardial fibrosis can lead to heart failure. Not surprisingly, the presence of fibrosis in cardiomyopathies, even when subtle, has consistently been associated with complications and adverse outcomes. In the last decade, non-invasive in vivo techniques for visualization of myocardial fibrosis have emerged, and have been increasingly used in research and in the clinic. In this review, we will describe the epidemiology, distribution, and role of myocardial fibrosis in genetic cardiomyopathies, including hypertrophic, dilated, arrhythmogenic, and non-compaction cardiomyopathy, and a few specific forms of genetic cardiomyopathies.
AB - Myocardial fibrosis is the excessive deposition of extracellular matrix proteins, including collagens, in the heart. In cardiomyopathies, the formation of interstitial fibrosis and/or replacement fibrosis is almost always part of the pathological cardiac remodeling process. Different forms of cardiomyopathies show particular patterns of myocardial fibrosis that can be considered as distinctive hallmarks. Although formation of fibrosis is initially aimed to be a reparative mechanism, in the long term, on-going and excessive myocardial fibrosis may lead to arrhythmias and stiffening of the heart wall and subsequently to diastolic dysfunction. Ultimately, adverse remodeling with progressive myocardial fibrosis can lead to heart failure. Not surprisingly, the presence of fibrosis in cardiomyopathies, even when subtle, has consistently been associated with complications and adverse outcomes. In the last decade, non-invasive in vivo techniques for visualization of myocardial fibrosis have emerged, and have been increasingly used in research and in the clinic. In this review, we will describe the epidemiology, distribution, and role of myocardial fibrosis in genetic cardiomyopathies, including hypertrophic, dilated, arrhythmogenic, and non-compaction cardiomyopathy, and a few specific forms of genetic cardiomyopathies.
KW - Fibrosis
KW - Remodeling
KW - Heart failure
KW - Cardiomyopathy
KW - Genetics
KW - Imaging
KW - CARDIAC MAGNETIC-RESONANCE
KW - VENTRICULAR NON-COMPACTION
KW - LATE GADOLINIUM ENHANCEMENT
KW - MYOCARDIAL FIBROSIS
KW - HYPERTROPHIC CARDIOMYOPATHY
KW - INTERSTITIAL FIBROSIS
KW - MUTATION CARRIERS
KW - TASK-FORCE
KW - DIAGNOSIS
KW - DISEASE
U2 - 10.1016/j.tcm.2019.09.003
DO - 10.1016/j.tcm.2019.09.003
M3 - Review article
C2 - 31585768
SN - 1050-1738
VL - 30
SP - 353
EP - 361
JO - TRENDS IN CARDIOVASCULAR MEDICINE
JF - TRENDS IN CARDIOVASCULAR MEDICINE
IS - 6
ER -