End stage of arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum.

Maartje Noorman; J.A. Groeneweg; A. Asimaki; S. Rizzo; M. Papegaaij; L. van Stuijvenberg; N. de Jonge; D. Dooijes; C. Basso; J.E. Saffitz; T.A.B. van Veen; A. Vink; RNW Hauer

doi:10.1016/j.hrthm.2012.10.029

End stage of arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum.

Maartje Noorman^*, J.A. Groeneweg, A. Asimaki, S. Rizzo, M. Papegaaij, L. van Stuijvenberg, N. de Jonge, D. Dooijes, C. Basso, J.E. Saffitz, T.A.B. van Veen, A. Vink, RNW Hauer

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

2 Citations (Scopus)

Abstract

Detailed histopathologic, immunohistochemical, and ultrastructural analysis of the heart of a 56-year-old woman with end-stage arrhythmogenic cardiomyopathy with a pathogenic plakophilin-2 mutation is described. The explanted heart revealed severe fibrofatty replacement of nearly the entire right ventricular free wall. The left ventricle was severely affected, and, most remarkable, there was massive involvement of the interventricular septum. Immunohistochemical and electron microscopic findings of intercalated disks revealed areas with a heterogeneous distribution of connexin43 and focal electron microscopic abnormalities among these regions. This case illustrates that arrhythmogenic cardiomyopathy is not limited to the right ventricle but involves the entire myocardium, including the interventricular septum. Copyright © 2013 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

Original language	English
Pages (from-to)	283-289
Number of pages	7
Journal	Heart Rhythm
Volume	10
Issue number	2
DOIs	https://doi.org/10.1016/j.hrthm.2012.10.029
Publication status	Published - 2013

Keywords

Arrhythmia
Arrhythmogenic right ventricular cardiomyopathy
Cardiac transplantation
Cardiomyopathy
Desmosome
Electrocardiogram
Electron microscopy
Gap junction
Genetics
Immunohistochemistry
RIGHT-VENTRICULAR CARDIOMYOPATHY
PLAKOPHILIN-2 MUTATIONS
PLAKOGLOBIN CAUSES
DYSPLASIA
DYSPLASIA/CARDIOMYOPATHY
SARCOIDOSIS
EXPRESSION
CONNEXIN43
PHENOTYPE
HEART

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10.1016/j.hrthm.2012.10.029

http://www.sciencedirect.com/science/article/pii/S1547527112012428

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Noorman, M., Groeneweg, J. A., Asimaki, A., Rizzo, S., Papegaaij, M., van Stuijvenberg, L., de Jonge, N., Dooijes, D., Basso, C., Saffitz, J. E., van Veen, T. A. B., Vink, A., & Hauer, RNW. (2013). End stage of arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum. Heart Rhythm, 10(2), 283-289. https://doi.org/10.1016/j.hrthm.2012.10.029

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title = "End stage of arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum.",

abstract = "Detailed histopathologic, immunohistochemical, and ultrastructural analysis of the heart of a 56-year-old woman with end-stage arrhythmogenic cardiomyopathy with a pathogenic plakophilin-2 mutation is described. The explanted heart revealed severe fibrofatty replacement of nearly the entire right ventricular free wall. The left ventricle was severely affected, and, most remarkable, there was massive involvement of the interventricular septum. Immunohistochemical and electron microscopic findings of intercalated disks revealed areas with a heterogeneous distribution of connexin43 and focal electron microscopic abnormalities among these regions. This case illustrates that arrhythmogenic cardiomyopathy is not limited to the right ventricle but involves the entire myocardium, including the interventricular septum. Copyright {\textcopyright} 2013 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.",

keywords = "Arrhythmia, Arrhythmogenic right ventricular cardiomyopathy, Cardiac transplantation, Cardiomyopathy, Desmosome, Electrocardiogram, Electron microscopy, Gap junction, Genetics, Immunohistochemistry, RIGHT-VENTRICULAR CARDIOMYOPATHY, PLAKOPHILIN-2 MUTATIONS, PLAKOGLOBIN CAUSES, DYSPLASIA, DYSPLASIA/CARDIOMYOPATHY, SARCOIDOSIS, EXPRESSION, CONNEXIN43, PHENOTYPE, HEART",

author = "Maartje Noorman and J.A. Groeneweg and A. Asimaki and S. Rizzo and M. Papegaaij and {van Stuijvenberg}, L. and {de Jonge}, N. and D. Dooijes and C. Basso and J.E. Saffitz and {van Veen}, T.A.B. and A. Vink and RNW Hauer",

year = "2013",

doi = "10.1016/j.hrthm.2012.10.029",

language = "English",

volume = "10",

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AU - Noorman, Maartje

AU - Groeneweg, J.A.

AU - Asimaki, A.

AU - Rizzo, S.

AU - Papegaaij, M.

AU - van Stuijvenberg, L.

AU - de Jonge, N.

AU - Dooijes, D.

AU - Basso, C.

AU - Saffitz, J.E.

AU - van Veen, T.A.B.

AU - Vink, A.

AU - Hauer, RNW

PY - 2013

Y1 - 2013

N2 - Detailed histopathologic, immunohistochemical, and ultrastructural analysis of the heart of a 56-year-old woman with end-stage arrhythmogenic cardiomyopathy with a pathogenic plakophilin-2 mutation is described. The explanted heart revealed severe fibrofatty replacement of nearly the entire right ventricular free wall. The left ventricle was severely affected, and, most remarkable, there was massive involvement of the interventricular septum. Immunohistochemical and electron microscopic findings of intercalated disks revealed areas with a heterogeneous distribution of connexin43 and focal electron microscopic abnormalities among these regions. This case illustrates that arrhythmogenic cardiomyopathy is not limited to the right ventricle but involves the entire myocardium, including the interventricular septum. Copyright © 2013 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

AB - Detailed histopathologic, immunohistochemical, and ultrastructural analysis of the heart of a 56-year-old woman with end-stage arrhythmogenic cardiomyopathy with a pathogenic plakophilin-2 mutation is described. The explanted heart revealed severe fibrofatty replacement of nearly the entire right ventricular free wall. The left ventricle was severely affected, and, most remarkable, there was massive involvement of the interventricular septum. Immunohistochemical and electron microscopic findings of intercalated disks revealed areas with a heterogeneous distribution of connexin43 and focal electron microscopic abnormalities among these regions. This case illustrates that arrhythmogenic cardiomyopathy is not limited to the right ventricle but involves the entire myocardium, including the interventricular septum. Copyright © 2013 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

KW - Arrhythmia

KW - Arrhythmogenic right ventricular cardiomyopathy

KW - Cardiac transplantation

KW - Cardiomyopathy

KW - Desmosome

KW - Electrocardiogram

KW - Electron microscopy

KW - Gap junction

KW - Genetics

KW - Immunohistochemistry

KW - RIGHT-VENTRICULAR CARDIOMYOPATHY

KW - PLAKOPHILIN-2 MUTATIONS

KW - PLAKOGLOBIN CAUSES

KW - DYSPLASIA

KW - DYSPLASIA/CARDIOMYOPATHY

KW - SARCOIDOSIS

KW - EXPRESSION

KW - CONNEXIN43

KW - PHENOTYPE

KW - HEART

U2 - 10.1016/j.hrthm.2012.10.029

DO - 10.1016/j.hrthm.2012.10.029

M3 - Article

C2 - 23085127

SN - 1547-5271

VL - 10

SP - 283

EP - 289

JO - Heart Rhythm

JF - Heart Rhythm

IS - 2

ER -

End stage of arrhythmogenic cardiomyopathy with severe involvement of the interventricular septum.

Abstract

Keywords

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