Arrhythmogenic Cardiomyopathy

Moniek G.P.J. Cox; Anneline S.J.M. te Riele; Richard N.W. Hauer

doi:10.1007/978-3-030-45457-9_6

Arrhythmogenic Cardiomyopathy

Moniek G.P.J. Cox^*, Anneline S.J.M. te Riele, Richard N.W. Hauer

^*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceeding › Chapter › Academic › peer-review

Abstract

Arrhythmogenic Cardiomyopathy (ACM) is a progressive heart muscle disease characterized histologically by fibrofatty replacement of ventricular myocardium. It is relatively rare, affecting approximately 1 in 1000 to 1 in 5000 people. Patients typically present with ventricular arrhythmias between the second and fourth decades of life. In early stages, ventricular arrhythmias originating from the right ventricle (RV) are most prominent, whereas usually in later stages, structural and functional abnormalities occur, eventually leading to heart failure. In all stages, ACM can be the cause of sudden death. However, sudden death most frequently occurs in adolescence, mainly in athletes. From autopsy studies, it is known that massive amounts of fibrofatty tissue can replace large parts of normal myocardium even in young teenagers (Fig. 6.1).

Original language	English
Title of host publication	Clinical Cardiogenetics
Subtitle of host publication	Third Edition
Publisher	Springer International Publishing
Pages	99-114
Number of pages	16
ISBN (Electronic)	9783030454579
ISBN (Print)	9783030454562
DOIs	https://doi.org/10.1007/978-3-030-45457-9_6
Publication status	Published - 1 Jan 2020

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title = "Arrhythmogenic Cardiomyopathy",

abstract = "Arrhythmogenic Cardiomyopathy (ACM) is a progressive heart muscle disease characterized histologically by fibrofatty replacement of ventricular myocardium. It is relatively rare, affecting approximately 1 in 1000 to 1 in 5000 people. Patients typically present with ventricular arrhythmias between the second and fourth decades of life. In early stages, ventricular arrhythmias originating from the right ventricle (RV) are most prominent, whereas usually in later stages, structural and functional abnormalities occur, eventually leading to heart failure. In all stages, ACM can be the cause of sudden death. However, sudden death most frequently occurs in adolescence, mainly in athletes. From autopsy studies, it is known that massive amounts of fibrofatty tissue can replace large parts of normal myocardium even in young teenagers (Fig. 6.1).",

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N2 - Arrhythmogenic Cardiomyopathy (ACM) is a progressive heart muscle disease characterized histologically by fibrofatty replacement of ventricular myocardium. It is relatively rare, affecting approximately 1 in 1000 to 1 in 5000 people. Patients typically present with ventricular arrhythmias between the second and fourth decades of life. In early stages, ventricular arrhythmias originating from the right ventricle (RV) are most prominent, whereas usually in later stages, structural and functional abnormalities occur, eventually leading to heart failure. In all stages, ACM can be the cause of sudden death. However, sudden death most frequently occurs in adolescence, mainly in athletes. From autopsy studies, it is known that massive amounts of fibrofatty tissue can replace large parts of normal myocardium even in young teenagers (Fig. 6.1).

AB - Arrhythmogenic Cardiomyopathy (ACM) is a progressive heart muscle disease characterized histologically by fibrofatty replacement of ventricular myocardium. It is relatively rare, affecting approximately 1 in 1000 to 1 in 5000 people. Patients typically present with ventricular arrhythmias between the second and fourth decades of life. In early stages, ventricular arrhythmias originating from the right ventricle (RV) are most prominent, whereas usually in later stages, structural and functional abnormalities occur, eventually leading to heart failure. In all stages, ACM can be the cause of sudden death. However, sudden death most frequently occurs in adolescence, mainly in athletes. From autopsy studies, it is known that massive amounts of fibrofatty tissue can replace large parts of normal myocardium even in young teenagers (Fig. 6.1).

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Arrhythmogenic Cardiomyopathy

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