Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review.

Asatryan, Babken; Asimaki, Angeliki; Landstrom, Andrew P; Khanji, Mohammed Y; Odening, Katja E; Cooper, Leslie T; Marchlinski, Francis E; Gelzer, Anna R; Semsarian, Christopher; Reichlin, Tobias; Owens, Anjali T; Chahal, C Anwar A (2021). Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review. Circulation, 144(20), pp. 1646-1655. American Heart Association 10.1161/CIRCULATIONAHA.121.055890

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Arrhythmogenic cardiomyopathy (ACM) is a primary disease of the myocardium, predominantly caused by genetic defects in proteins of the cardiac intercalated disc, particularly, desmosomes. Transmission is mostly autosomal dominant with incomplete penetrance. ACM also has wide phenotype variability, ranging from premature ventricular contractions to sudden cardiac death and heart failure. Among other drivers and modulators of phenotype, inflammation in response to viral infection and immune triggers have been postulated to be an aggravator of cardiac myocyte damage and necrosis. This theory is supported by multiple pieces of evidence, including the presence of inflammatory infiltrates in more than two-thirds of ACM hearts, detection of different cardiotropic viruses in sporadic cases of ACM, the fact that patients with ACM often fulfill the histological criteria of active myocarditis, and the abundance of anti-desmoglein-2, antiheart, and anti-intercalated disk autoantibodies in patients with arrhythmogenic right ventricular cardiomyopathy. In keeping with the frequent familial occurrence of ACM, it has been proposed that, in addition to genetic predisposition to progressive myocardial damage, a heritable susceptibility to viral infections and immune reactions may explain familial clustering of ACM. Moreover, considerable in vitro and in vivo evidence implicates activated inflammatory signaling in ACM. Although the role of inflammation/immune response in ACM is not entirely clear, inflammation as a driver of phenotype and a potential target for mechanism-based therapy warrants further research. This review discusses the present evidence supporting the role of inflammatory and immune responses in ACM pathogenesis and proposes opportunities for translational and clinical investigation.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology
04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Physiology

UniBE Contributor:

Asatryan, Babken, Odening, Katja Elisabeth, Reichlin, Tobias Roman

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1524-4539

Publisher:

American Heart Association

Language:

English

Submitter:

Nadia Biscozzo

Date Deposited:

21 Jan 2022 11:00

Last Modified:

05 Dec 2022 15:59

Publisher DOI:

10.1161/CIRCULATIONAHA.121.055890

PubMed ID:

34780255

Uncontrolled Keywords:

arrhythmias, cardiac arrhythmogenic right ventricular dysplasia autoimmunity death, sudden, cardiac genetics inflammation myocarditis

BORIS DOI:

10.48350/163284

URI:

https://boris.unibe.ch/id/eprint/163284

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