Management of Recurrent Myocarditis Due to Desmoplakin Cardiomyopathy: Diagnostic and Therapeutic Challenges

Overview

Journal JACC Case Rep

Specialty Cardiology & Vascular Diseases

Date 2022 Jan 17

PMID 35036946

Authors

Malik Ghawanmeh

Blanca Simon Frances

Ajay Kerai

Parth Patel

Jonathan Du

Preetham Kumar

Affiliations

Soon will be listed here.

Abstract

We present the case of a 25-year-old woman with desmoplakin cardiomyopathy-related myocarditis. Her high-sensitivity troponin and symptoms improved with pulse steroid therapy and mycophenolate mofetil. The literature lacks data to effectively guide the management of recurrent myocarditis in desmoplakin cardiomyopathy. ().

Citing Articles

Shaping cardiac diagnostics: The role of myocardial tissue mapping in unraveling ring-like fibrosis.

Gil K, Truong V, Rajpal S, Zareba K Cardiol J. 2025; 32(1):62-72.

PMID: 39912881 PMC: 11870005. DOI: 10.5603/cj.101260.

Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies.

Gasperetti A, Carrick R, Muller S, Murray B, Adamo L, Bauce B Curr Cardiol Rep. 2025; 27(1):12.

PMID: 39786454 DOI: 10.1007/s11886-024-02183-7.

The Desmoplakin Phenotype Spectrum: Is the Inflammation the "Fil Rouge" Linking Myocarditis, Arrhythmogenic Cardiomyopathy, and Uncommon Autoinflammatory Systemic Disease?.

DElia S, Caputo A, Natale F, Pezzullo E, Limongelli G, Golino P Genes (Basel). 2024; 15(9).

PMID: 39336825 PMC: 11431300. DOI: 10.3390/genes15091234.

Case report: desmoplakin cardiomyopathy presenting as an inflammatory cardiomyopathy with repeated sudden cardiac arrests.

Massie E, Dominati A, Suchet S, Carballo D, Hervier E, Fokstuen S Eur Heart J Case Rep. 2024; 8(4):ytae160.

PMID: 38887779 PMC: 11181931. DOI: 10.1093/ehjcr/ytae160.

MRI Findings in Desmoplakin-related Arrhythmogenic Left Ventricular Cardiomyopathy in a Pediatric Patient: A Case Report.

Morcos G, Vashist S, Aktay R Radiol Cardiothorac Imaging. 2023; 5(2):e220209.

PMID: 37124635 PMC: 10141448. DOI: 10.1148/ryct.220209.

References

Lopez-Ayala J, Gomez-Milanes I, Sanchez Munoz J, Ruiz-Espejo F, Ortiz M, Gonzalez-Carrillo J . Desmoplakin truncations and arrhythmogenic left ventricular cardiomyopathy: characterizing a phenotype. Europace. 2014; 16(12):1838-46. DOI: 10.1093/europace/euu128. View

Smith E, Lakdawala N, Papoutsidakis N, Aubert G, Mazzanti A, McCanta A . Desmoplakin Cardiomyopathy, a Fibrotic and Inflammatory Form of Cardiomyopathy Distinct From Typical Dilated or Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation. 2020; 141(23):1872-1884. PMC: 7286080. DOI: 10.1161/CIRCULATIONAHA.119.044934. View

Favre B, Begre N, Marsili L, van Tintelen J, Borradori L . Desmoplakin Gene Variants and Risk for Arrhythmogenic Cardiomyopathy. Circ Genom Precis Med. 2018; 11(9):e002241. DOI: 10.1161/CIRCGEN.118.002241. View

Tschope C, Ammirati E, Bozkurt B, Caforio A, Cooper L, Felix S . Myocarditis and inflammatory cardiomyopathy: current evidence and future directions. Nat Rev Cardiol. 2020; 18(3):169-193. PMC: 7548534. DOI: 10.1038/s41569-020-00435-x. View

Reichl K, Kreykes S, Martin C, Shenoy C . Desmoplakin Variant-Associated Arrhythmogenic Cardiomyopathy Presenting as Acute Myocarditis. Circ Genom Precis Med. 2018; 11(12):e002373. PMC: 6300059. DOI: 10.1161/CIRCGEN.118.002373. View