Display options
Cite
Asatryan B, Asimaki A, Landstrom AP, et al. Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review. Circulation. 2021;144(20):1646-1655doi: 10.1161/CIRCULATIONAHA.121.055890.
Asatryan, B., Asimaki, A., Landstrom, A. P., Khanji, M. Y., Odening, K. E., Cooper, L. T., Marchlinski, F. E., Gelzer, A. R., Semsarian, C., Reichlin, T., Owens, A. T., & Chahal, C. A. A. (2021). Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review. Circulation, 144(20), 1646-1655. https://doi.org/10.1161/CIRCULATIONAHA.121.055890
Asatryan, Babken, et al. "Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review." Circulation vol. 144,20 (2021): 1646-1655. doi: https://doi.org/10.1161/CIRCULATIONAHA.121.055890
Asatryan B, Asimaki A, Landstrom AP, Khanji MY, Odening KE, Cooper LT, Marchlinski FE, Gelzer AR, Semsarian C, Reichlin T, Owens AT, Chahal CAA. Inflammation and Immune Response in Arrhythmogenic Cardiomyopathy: State-of-the-Art Review. Circulation. 2021 Nov 16;144(20):1646-1655. doi: 10.1161/CIRCULATIONAHA.121.055890. Epub 2021 Nov 15. PMID: 34780255.
Copy Download .nbib Format: NLM
Share it on

Circulation. 2021 Nov 16;144(20):1646-1655. doi: 10.1161/CIRCULATIONAHA.121.055890. Epub 2021 Nov 15.

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

Circulation

Babken Asatryan, Angeliki Asimaki, Andrew P Landstrom, Mohammed Y Khanji, Katja E Odening, Leslie T Cooper, Francis E Marchlinski, Anna R Gelzer, Christopher Semsarian, Tobias Reichlin, Anjali T Owens, C Anwar A Chahal

Affiliations

  1. Department of Cardiology, Inselspital, Bern University Hospital (B.A., K.E.O., T.R.), University of Bern, Switzerland.
  2. Cardiovascular and Clinical Academic Group, Molecular and Clinical Sciences Research Institute, St George's University of London, United Kingdom (A.A.).
  3. Division of Cardiology, Department of Pediatrics (A.P.L.), Duke University School of Medicine, Durham, NC.
  4. Department of Cell Biology (A.P.L.), Duke University School of Medicine, Durham, NC.
  5. Department of Cardiology, Barts Heart Centre, Barts Health NHS Trust, London, United Kingdom (M.Y.K., A.A.C.).
  6. NIHR Biomedical Research Unit, William Harvey Research Institute, Queen Mary University of London, United Kingdom (M.Y.K.).
  7. Department of Cardiology, Newham University Hospital, London, United Kingdom (M.Y.K.).
  8. Department of Physiology (K.E.O.), University of Bern, Switzerland.
  9. Cardiac Electrophysiology, Cardiovascular Division, Hospital of the University of Pennsylvania, Philadelphia (F.E.M., A.A.C.).
  10. Department of Clinical Sciences and Advanced Medicine, School of Veterinary Medicine, University of Pennsylvania, Philadelphia (A.R.G.).
  11. Agnes Ginges Centre for Molecular Cardiology at Centenary Institute (C.S.), The University of Sydney, New South Wales, Australia.
  12. Sydney Medical School Faculty of Medicine and Health (C.S.), The University of Sydney, New South Wales, Australia.
  13. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia (C.S.).
  14. Center for Inherited Cardiac Disease, Division of Cardiovascular Medicine, University of Pennsylvania, Perelman School of Medicine, Philadelphia (A.T.O.).
  15. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN (A.A.C.).
  16. WellSpan Center for Inherited Cardiovascular Diseases, WellSpan Health, Lancaster, PA (A.A.C.).

PMID: 34780255 DOI: 10.1161/CIRCULATIONAHA.121.055890

Abstract

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.

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

Publication Types

Grant support