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Cited by 504Cardiovascular Effects of ExerciseCardiomyopathy and Myosin StudiesCardiac electrophysiology and arrhythmias

Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy

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Smith, Eric D., Lakdawala, Neal K., Papoutsidakis, Nikolaos, Aubert, Gregory, Mazzanti, Andrea, McCanta, Anthony C., Agarwal, Prachi P., Arscott, Patricia +8 more·2020·Circulation
DOI 10.1161/circulationaha.119.044934PMC PMC7286080
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Abstract

Background: Mutations in desmoplakin ( DSP ), the primary force transducer between cardiac desmosomes and intermediate filaments, cause an arrhythmogenic form of cardiomyopathy that has been variably associated with arrhythmogenic right ventricular cardiomyopathy. Clinical correlates of DSP cardiomyopathy have been limited to small case series. Methods: Clinical and genetic data were collected on 107 patients with pathogenic DSP mutations and 81 patients with pathogenic plakophilin 2 ( PKP2 ) mutations as a comparison cohort. A composite outcome of severe ventricular arrhythmia was assessed. Results: DSP and PKP2 cohorts included similar proportions of probands (41% versus 42%) and patients with truncating mutations (98% versus 100%). Left ventricular (LV) predominant cardiomyopathy was exclusively present among patients with DSP (55% versus 0% for PKP2 , P <0.001), whereas right ventricular cardiomyopathy was present in only 14% of patients with DSP versus 40% for PKP2 ( P <0.001). Arrhythmogenic right ventricular cardiomyopathy diagnostic criteria had poor sensitivity for DSP cardiomyopathy. LV late gadolinium enhancement was present in a primarily subepicardial distribution in 40% of patients with DSP (23/57 with magnetic resonance images). LV late gadolinium enhancement occurred with normal LV systolic function in 35% (8/23) of patients with DSP . Episodes of acute myocardial injury (chest pain with troponin elevation and normal coronary angiography) occurred in 15% of patients with DSP and were strongly associated with LV late gadolinium enhancement (90%), even in cases of acute myocardial injury with normal ventricular function (4/5, 80% with late gadolinium enhancement). In 4 DSP cases with 18F-fluorodeoxyglucose positron emission tomography scans, acute LV myocardial injury was associated with myocardial inflammation misdiagnosed initially as cardiac sarcoidosis or myocarditis. Left ventricle ejection fraction <55% was strongly associated with severe ventricular arrhythmias for DSP cases ( P <0.001, sensitivity 85%, specificity 53%). Right ventricular ejection fraction <45% was associated with severe arrhythmias for PKP2 cases ( P <0.001) but was poorly associated for DSP cases ( P =0.8). Frequent premature ventricular contractions were common among patients with severe arrhythmias for both DSP (80%) and PKP2 (91%) groups ( P =non-significant). Conclusions: DSP cardiomyopathy is a distinct form of arrhythmogenic cardiomyopathy characterized by episodic myocardial injury, left ventricular fibrosis that precedes systolic dysfunction, and a high incidence of ventricular arrhythmias. A genotype-specific approach for diagnosis and risk stratification should be used.

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  • Desmoplakin cardiomyopathy can be characterized by episodic myocardial injury resembling myocarditis.

    “desmoplakin cardiomyopathy can be characterized by episodic myocardial injury resembling myocarditis.”

    From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [221]

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Authors
Smith, E. D., Lakdawala, N. K., Papoutsidakis, N., Aubert, G., Mazzanti, A., McCanta, A. C., Agarwal, P. P., Arscott, P., Dellefave-Castillo, L. M., Vorovich, E. E., Nutakki, K., Wilsbacher, L. D., Priori, S. G., Jacoby, D. L., McNally, E. M., & Helms, A. S.
Affiliations
  • University of Michigan (US)
  • Brigham and Women's Hospital (US)
  • Yale University (US)
  • Center for Genetic Medicine (G.A.), Feinberg School of Medicine, Northwestern University, Chicago, IL.
  • Istituti Clinici Scientifici Maugeri (IT)
  • Children's Hospital of Orange County (US)
  • Feinberg Cardiovascular Research Institute (L.M.D.-C., L.D.W., E.M.M.), Feinberg School of Medicine, Northwestern University, Chicago, IL.
  • Northwestern University (US)
ORCID
Venue
Circulation
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2020-06-09
Volume
141
Issue
23
Pages
1872-1884
ISSN
  • 0009-7322 (ISSN-L)
  • 1524-4539
DOI
10.1161/circulationaha.119.044934
PMC
PMC7286080
OpenAlex
W3022047361
Cited by
504
References
4
Citations by year
2026: 91 · 2025: 106 · 2024: 94 · 2023: 73 · 2022: 59 · 2021: 63 · 2020: 17 · 2018: 1
FWCI
32.80
Type
journal article
Language
en
Open access
Locations
  • Circulation (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
  • Deep Blue (University of Michigan) (submittedVersion)
  • University of Michigan Library
Funders
  • National Institutes of Health (UL1TR001422, UL1TR001863)
  • National Heart, Lung, and Blood Institute (K08HL130455, R01HL128075)
  • National Center for Advancing Translational Sciences
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
Keywords
arrhythmogenic right ventricular cardiomyopathy, late gadolinium enhancement, myocarditis, acute myocardial injury, ventricular arrhythmias, myocardial fibrosis, intermediate filaments
MeSH terms
Adult, Cardiomyopathy, Dilated, Female, Fibrosis, Humans, Inflammation, Male, Middle Aged, Mutation, Cardiomyopathies, Retrospective Studies, Arrhythmogenic Right Ventricular Dysplasia, Desmoplakins, Young Adult

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