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Cited by 36Viral Infections and Immunology ResearchCardiomyopathy and Myosin StudiesCardiac electrophysiology and arrhythmias

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Prognostic Role of Myocarditis-Like Episodes and Their Treatment in Patients With Pathogenic Desmoplakin Variants

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Gasperetti, Alessio, Muller, Steven A., Peretto, Giovanni, Asatryan, Babken, Protonotarios, Alexandros, Laredo, Mikael, Tarlet, Pierre-Yves, Syrris, Petros, Carrick, Richard T., Murray, Brittney, Tichnell, Crystal, Scheel, Paul, Gigli, Marta, Yap, Sing-Chien, Pilichou, Kalliopi, Basso, Cristina, Medo, Kristen, Jason, Max, Rossi, Valentina A., Saguner, Ardan M., Duru, Firat, Abrams, Dominic, Cadrin-Tourigny, Julia, Targetti, Mattia, Pieroni, Maurizio, Graziosi, Maddalena, Biagini, Elena, Charron, Philippe, Cox, Moniek, Casella, Michela, Tondo, Claudio, Ware, James S., Prasad, Sanjay K., Calò, Leonardo, Smith, Eric, Ingles, Jodie, Tandri, Harikrishna, Ader, Flavie, Peters, Stacey, Zentner, Dominica, Russo, Jeremy, Ravaro, Silvia, Crotti, Lia, Dittmann, Sven, Schulze-Bahr, Eric, Carruth, Eric D., Krysov, Vikki A., Parikh, Victoria N., Conte, Giulio, Wilde, Arthur, van Tintelen, J. Peter, te Riele, Anneline S.J.M., Sinagra, Gianfranco, Mestroni, Luisa, Taylor, Matthew, Merlo, Marco, Adamo, Luigi, Calkins, Hugh, Bauce, Barbara, Arbelo, Elena, Elliott, Perry M., Gandjbakhch, Estelle, Lakdawala, Neal, Helms, Adam, James, Cynthia A., Gilotra, Nisha A.·2025·Circulation
DOI 10.1161/circulationaha.125.073919PMC PMC13036385
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Abstract

BACKGROUND: Inflammatory, myocarditis-like episodes precede and are associated with higher risk of sustained ventricular arrhythmias and heart failure in patients with pathogenic or likely pathogenic desmoplakin (DSP) variants. Whether the recurrence and treatment of myocarditis-like episodes influence the outcomes in this population is unknown. This study aimed to assess the prognostic impact of the recurrence and treatment of myocarditis-like episodes in patients with pathogenic or likely pathogenic DSP variants. METHODS: The study was designed as an observational cohort study using all patients with pathogenic or likely pathogenic DSP variants in the DSP -ERADOS Network ( Desmopakin Specific Effort for a Rare Disease Outcome Study) enrolled at 31 institutions up until April 30, 2024. Survival from ventricular arrhythmia and heart failure end points and recurrent myocarditis-like episodes was evaluated for both first and recurrent documented myocarditis-like episodes and the impact of immunosuppressive treatment on outcomes assessed. RESULTS: A total of 1014 patients with pathogenic or likely pathogenic DSP variants (62.4% female; mean age, 40.4±17.4 years; 47.1% probands) were included. Among them, 177 individuals (17.5%) experienced ≥1 documented myocarditis-like episode, with 63 of them (35.6%) receiving immunosuppressive treatment. Immunosuppressive treatment of the first documented myocarditis-like episode was associated with reduced risk of ventricular arrhythmia and heart failure outcomes (hazard ratio, 0.18 [95% CI, 0.07–0.45]; P <0.001; and hazard ratio, 0.09 [95% CI, 0.02–0.39; P =0.001, respectively) over a median follow-up of 6.4 years (95% CI, 4.0–10.5). This risk was comparable to that of patients with no history of myocarditis-like episode but did not correlate with a reduction in recurrent episodes after completion of initial immunosuppression (hazard ratio, 0.85 [95% CI, 0.47–1.54]; P =0.599). In contrast, nonsteroidal anti-inflammatory drugs or colchicine treatment did not influence outcomes. CONCLUSIONS: Immunosuppressive treatment of myocarditis-like episodes in DSP patients was associated with improved combined ventricular arrhythmia or heart failure outcomes, supporting future prospective evaluation in DSP cohorts.

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  • The "hot phase" of desmosomal ACM occurs more frequently in young individuals and women and is characterized by chest pain, peaks in serum cTnT, and mildly reduced or preserved cardiac function.

    “The so-called “hot phase” of desmosomal ACM occurs more frequently in young individuals and women and is characterized by chest pain, peaks in serum cTnT, and mildly reduced or preserved cardiac function [56,57,58].”

    From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [57]

    The Results (Patient Population & Baseline; Myocarditis Episodes, page unavailable) describe patients with desmoplakin-associated episodes as young and frequently female, with mostly normal ventricular function during the first episode. The Introduction describes sudden chest pain and increased cardiac troponins. The paper does not establish that episodes are more frequent in women than in men, specify cTnT peaks, or extend these findings to all desmosomal ACM. (LLM, conf=0.87)

    Experimental — accuracy not yet measured

  • DGV-positive patients with myocarditis-like phenotypes are at increased risk of major ventricular arrhythmias and heart failure compared to DGV-negative patients.

    “DGV-positive patients with myocarditis-like phenotypes are at increased risk of major VAs and heart failure, as compared to DGV-negative patients [56,57,58].”

    From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [57]

    The Results (Follow Up and Long-Term Outcomes; page unavailable) report higher ventricular-arrhythmia and heart-failure hospitalization rates in DSP variant carriers with myocarditis-like episodes than in carriers without such episodes. But the Methods (Study population; page unavailable) say both groups carried pathogenic or likely pathogenic DSP variants. The paper does not support the claimed comparison with DGV-negative patients. (LLM, conf=0.90)

    Experimental — accuracy not yet measured

  • Direct pharmacologic inhibition of NFκB has not reached clinical application because of unacceptable off-target effects; colchicine was not effective in ACM.

    “Direct pharmacologic inhibition of NFκB has not reached clinical application because of unacceptable off-target effects; furthermore, colchicine, an indirect modulator of NFκB signaling, was not effective in ACM [57].”

    From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [57]

    The Discussion (page unavailable) reports no apparent effect of NSAIDs or colchicine on cardiac outcomes in patients with pathogenic DSP variants and myocarditis-like episodes. That supports a narrower finding than the claim that colchicine was ineffective in ACM generally. The paper passages do not address NFκB inhibition, off-target effects, or whether direct inhibitors have reached clinical use. (LLM, conf=0.86)

    Experimental — accuracy not yet measured

  • In ACM, IL-1 inhibition has been employed on an individualized basis, particularly in patients with DSP-related ACM with recurrent inflammatory flares, with promising effects.

    “In the setting of ACM, IL-1 inhibition has been employed on an individualized basis, particularly in patients with DSP-related ACM with recurrent inflammatory flares, with promising effects [45,57].”

    From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [57]

    The Methods (Definitions and Outcomes; page not provided) include an interleukin-1 receptor antagonist among treatments classified as immunosuppression for myocarditis-like episodes in patients with DSP variants. The Results and Discussion report promising outcomes for immunosuppression as a group, including treatment of recurrent episodes, but do not establish a benefit specific to IL-1 inhibition or describe its use as individualized. (LLM, conf=0.85)

    Experimental — accuracy not yet measured

  • Conventional immunosuppression in ACM populations appears safe, provided exclusion of clinical contraindications, including active myocardial viral infection.

    “Conventional immunosuppression in ACM populations appears to be safe, provided the exclusion of clinical contraindications, including active myocardial viral infection [45,57].”

    From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [57]

    The Results and Discussion report lower rates of ventricular arrhythmias and heart-failure hospitalization among immunosuppressed patients with pathogenic DSP variants and myocarditis-like episodes. They do not establish the safety of conventional immunosuppression across ACM populations or say that its safety depends on excluding contraindications such as active myocardial viral infection. The Discussion mentions a separate trial in virus-negative myocarditis, but does not make that safety claim. (LLM, conf=0.79)

    Experimental — accuracy not yet measured

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Citations

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Research works printed but not linked: 24

  1. 10.1161/circulationaha.119.044934 · DOI · Paper page
  2. 10.1093/europace/euab183 · DOI · Paper page
  3. 10.1016/j.hrthm.2022.04.015 · DOI
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Metadata

Authors
Gasperetti, A., Muller, S. A., Peretto, G., Asatryan, B., Protonotarios, A., Laredo, M., Tarlet, P.-Y., Syrris, P., Carrick, R. T., Murray, B., Tichnell, C., Scheel, P., Gigli, M., Yap, S.-C., Pilichou, K., Basso, C., Medo, K., Jason, M., Rossi, V. A., Saguner, A. M., Duru, F., Abrams, D., Cadrin-Tourigny, J., Targetti, M., Pieroni, M., Graziosi, M., Biagini, E., Charron, P., Cox, M., Casella, M., Tondo, C., Ware, J. S., Prasad, S. K., Calò, L., Smith, E., Ingles, J., Tandri, H., Ader, F., Peters, S., Zentner, D., Russo, J., Ravaro, S., Crotti, L., Dittmann, S., Schulze-Bahr, E., Carruth, E. D., Krysov, V. A., Parikh, V. N., Conte, G., Wilde, A., van Tintelen, J. P., te Riele, A. S. J. M., Sinagra, G., Mestroni, L., Taylor, M., Merlo, M., Adamo, L., Calkins, H., Bauce, B., Arbelo, E., Elliott, P. M., Gandjbakhch, E., Lakdawala, N., Helms, A., James, C. A., & Gilotra, N. A.
Affiliations
  • Johns Hopkins University (US)
  • IRCCS Ospedale San Raffaele (IT)
  • St Bartholomew's Hospital (GB)
  • Sorbonne Université, AP-HP, IHU-ICAN, Groupe Hospitalier Pitié-Salpêtrière, Institut de Cardiologie, Paris, France (M.L., P.-Y.T., P.C., F.A., E.G.).
  • Sorbonne Université (FR)
  • University of Trieste (IT)
  • Erasmus MC (NL)
  • University of Padua (IT)
  • University of Colorado Anschutz (US)
  • University of Zurich (CH)
  • Department of Cardiology, Arrhythmia Unit, University Heart Center, University Hospital Zurich, Switzerland (V.A.R.., A.M.S., F.D.).
  • Boston Children's Hospital (US)
  • Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, QC, Canada (J.C.-T.).
  • Meyer Children's Hospital (IT)
  • IRCCS Azienda Ospedliero-Universitaria di Bologna Policlinico di Sant'Orsola (IT)
  • University Medical Center Groningen (NL)
  • Marche Polytechnic University (IT)
  • Centro Cardiologico Monzino (IT)
  • MRC London Institute of Medical Sciences (GB)
  • Policlinico Casilino (IT)
  • University of Michigan (US)
  • Garvan Institute of Medical Research (AU)
  • Vanderbilt University Medical Center (US)
  • The Royal Melbourne Hospital (AU)
  • IRCCS Istituto Auxologico Italiano (IT)
  • University Hospital Münster (DE)
  • Department of Cardiovascular Medicine, Institute for Genetics of Heart Diseases, University Hospital Münster, Germany (S.D., E.-S.B.).
  • Geisinger Health System (US)
  • Stanford Medicine (US)
  • Istituto Cardiocentro Ticino (CH)
  • Amsterdam UMC Location University of Amsterdam (NL)
  • University Medical Center Utrecht (NL)
  • Division of Cardiology, University Medical Center Utrecht, the Netherlands (S.A.M., J.P.v.T., A.S.J.M.t.R.).
  • Centro de Investigación en Red en Enfermedades Cardiovasculares (ES)
  • Inherited Cardiovascular Diseases Unit, St Bartholomew’s Hospital, UCL Institute of Cardiovascular Science, London, UK (A.P., P.S., P.M.E.).
  • Brigham and Women's Hospital (US)
Corresponding author
  • Muller, Steven A.
  • Tarlet, Pierre-Yves
  • Gigli, Marta
  • Yap, Sing-Chien
  • Pilichou, Kalliopi
  • Basso, Cristina
  • Graziosi, Maddalena
  • Biagini, Elena
  • Charron, Philippe
  • Ader, Flavie
  • Ravaro, Silvia
  • Crotti, Lia
  • Dittmann, Sven
  • Wilde, Arthur
  • van Tintelen, J. Peter
  • Sinagra, Gianfranco
  • Merlo, Marco
  • Bauce, Barbara
  • Arbelo, Elena
  • Gandjbakhch, Estelle
ORCID
Venue
Circulation
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2025-10-07
Volume
152
Issue
14
Pages
978-989
ISSN
  • 0009-7322 (ISSN-L)
  • 1524-4539
DOI
10.1161/circulationaha.125.073919
PMC
PMC13036385
OpenAlex
W4413338008
Cited by
36
References
4
Citations by year
2026: 31 · 2025: 5
FWCI
25.15
Type
journal article
Language
en
Open access
Locations
  • Circulation (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (publishedVersion)
  • EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, other-oa)
  • Pure Amsterdam UMC (submittedVersion, other-oa)
  • ArTS Archivio della ricerca di Trieste (University of Trieste https://www.units.it/) (submittedVersion)
  • BOA (University of Milano-Bicocca) (submittedVersion)
  • Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
  • UCL Discovery (University College London) (submittedVersion)
  • Florence Research (University of Florence) (submittedVersion)
  • Università Politecnica delle Marche (Università Politecnica delle Marche) (submittedVersion)
  • University of Groningen research database (University of Groningen / Centre for Information Technology) (submittedVersion, other-oa)
  • Padua Research Archive (University of Padova) (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich) (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich)
Funders
  • National Science Foundation
  • Johns Hopkins University
  • Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung
  • Schweizerische Herzstiftung
  • Baugarten Stiftung
  • National Heart, Lung, and Blood Institute (R01HL141901, T32HL007227)
Topics
  • Health Sciences › Medicine › Infectious Diseases › Viral Infections and Immunology Research
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
Keywords
desmoplakin, immunosuppressive therapy, ventricular arrhythmias, heart failure, prognosis, recurrent myocarditis, nonsteroidal anti-inflammatory drugs, colchicine, arrhythmia recurrence, survival outcomes, genotype-phenotype correlation
MeSH terms
Adult, Female, Heart Failure, Humans, Immunosuppressive Agents, Male, Middle Aged, Myocarditis, Prognosis, Recurrence, Desmoplakins

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