Observational studyCirculation2025
Prognostic Role of Myocarditis-Like Episodes and Their Treatment in Patients With Pathogenic Desmoplakin Variants.
Abstract readObservational StudyMulticenter Study
In one paragraph
Observational study in Circulation, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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14citing papers in PubMed
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What it found
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Who cites it
14 citing papers in PubMed.
- Colchicine in patients with chronic inflammatory cardiomyopathy: rationale and design of the CMP-MYTHiC.ESC heart failure · 2026Trial
- Arrhythmogenic right ventricular cardiomyopathy.European heart journal · 2026Review
- Workup and management of rhythm disorders in myocarditis and inflammatory cardiomyopathy: a clinical consensus statement of the European Heart Rhythm Association and the Heart Failure Association of the ESC, the ESC Working Group on Myocardial & Pericardial Diseases, the European Association of Preventive Cardiology of the ESC, the Heart Rhythm Society, the Asian Pacific Heart Rhythm Society, and the Latin American Heart Rhythm Society.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2026Article
- Physical Activity in Arrhythmogenic Cardiomyopathy: From Trigger to Therapeutic Dilemma.Reviews in cardiovascular medicine · 2026Review
- Disease penetrance and phenotypic spectrum of desmoplakin variant carriers in the population.Heart rhythm · 2026Article
- Acute and Chronic Myocarditis in Men and Women.Current heart failure reports · 2026Review
- Genetic counselling implementation in dilated cardiomyopathy.European heart journal · 2026Review
- Review
- Metabolic Reprogramming-Driven Cardiovascular Immune Damage: From Glyco-Lipotoxicity and Epigenetic Memory to Multidimensional Cross-Organ Communication Networks.International journal of molecular sciences · 2026Review
- Multi-site lactate kinetics during moderate intensity resistance and endurance exercise in definitive and probable arrhythmogenic cardiomyopathy.International journal of cardiology. Heart & vasculature · 2026Article
- Insights Into the Natural History of Recurrent Myocarditis, A Multicenter International Study (Re-Myo Study).Journal of the American Heart Association · 2026Observational
- Review
- Electrical storm in pregnancy uncovering an arrhythmogenic cardiomyopathy: a case report.European heart journal. Case reports · 2026Article
- Recurrent myocarditis-like episodes in a patient with a rare variant in DES gene: an uncommon hot-phases cardiomyopathy.ESC heart failure · 2026Article
Corrections and comments
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Authors and funding
66 authors.
Alessio GasperettiDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0003-3432-070X
Steven A MullerDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0002-2674-8709
Giovanni PerettoMultidisciplinary Disease Unit for Myocarditis and Arrhythmogenic Cardiomyopathies, IRCCS San Raffaele Scientific Institute, Milan, Italy (G.P.).ORCID 0000-0003-1815-4000
Babken AsatryanDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0002-0050-5717
Alexandros ProtonotariosInherited Cardiovascular Diseases Unit, St Bartholomew's Hospital, UCL Institute of Cardiovascular Science, London, UK (A.P., P.S., P.M.E.).
Mikael LaredoSorbonne 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.).ORCID 0000-0002-7326-2656
Pierre-Yves TarletSorbonne 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.).
Petros SyrrisInherited Cardiovascular Diseases Unit, St Bartholomew's Hospital, UCL Institute of Cardiovascular Science, London, UK (A.P., P.S., P.M.E.).ORCID 0000-0002-2363-8758
Richard T CarrickDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0001-5589-1216
Brittney MurrayDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).
Crystal TichnellDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).
Paul ScheelDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).
Marta GigliCardiothoracovascular Department, Division of Cardiology, Azienda Sanitaria Universitaria Giuliano Isontina and University of Trieste, Italy (M.G., G.S., M.M.).
Sing-Chien YapDepartment of Cardiology, Thorax Center, Cardiovascular Institute, Erasmus MC, Rotterdam, the Netherlands (S.-C.Y.).ORCID 0000-0003-4520-2725
Kalliopi PilichouDepartment of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Italy (K.P., C.B., B.B.).ORCID 0000-0003-3646-0669
Cristina BassoDepartment of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Italy (K.P., C.B., B.B.).ORCID 0000-0002-0195-9753
Kristen MedoUniversity of Colorado Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora (K.M., M.J., L.M., M.T.).ORCID 0009-0007-9785-4668
Max JasonUniversity of Colorado Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora (K.M., M.J., L.M., M.T.).ORCID 0009-0003-1492-2276
Valentina A RossiDepartment of Cardiology, Arrhythmia Unit, University Heart Center, University Hospital Zurich, Switzerland (V.A.R.., A.M.S., F.D.).ORCID 0000-0001-9235-6597
Ardan M SagunerDepartment of Cardiology, Arrhythmia Unit, University Heart Center, University Hospital Zurich, Switzerland (V.A.R.., A.M.S., F.D.).ORCID 0000-0003-1896-0803
Firat DuruDepartment of Cardiology, Arrhythmia Unit, University Heart Center, University Hospital Zurich, Switzerland (V.A.R.., A.M.S., F.D.).ORCID 0000-0002-4748-0158
Dominic AbramsCenter for Cardiovascular Genetics, Boston Children's Hospital and Harvard Medical School, MA (D.A.).ORCID 0000-0001-5266-2805
Julia Cadrin-TourignyCardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal, QC, Canada (J.C.-T.).ORCID 0000-0002-7489-8744
Mattia TargettiUniversity of Colorado Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora (K.M., M.J., L.M., M.T.).ORCID 0000-0001-9213-8565
Maurizio PieroniDepartment of Experimental and Clinical Medicine, University of Florence, Meyer Children Hospital and Careggi University Hospital, Italy (M.T., M.P.).ORCID 0000-0002-6830-4292
Maddalena GraziosiCardiac Thoracic and Vascular Department, Cardiology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Italy (M.G., E.B.).
Elena BiaginiCardiac Thoracic and Vascular Department, Cardiology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Italy (M.G., E.B.).ORCID 0000-0003-0295-0440
Philippe CharronSorbonne 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.).ORCID 0000-0002-1782-1118
Moniek CoxDepartment of Cardiology, University Medical Centre Groningen, the Netherlands (M. Cox).ORCID 0000-0003-2825-8428
Michela CasellaCardiology and Arrhythmology Clinic, University Hospital "Ospedali Riuniti," Department of Clinical, Special and Dental Sciences, Marche Polytechnic University, Ancona, Italy (M. Casella).ORCID 0000-0002-5322-1742
Claudio TondoCentro Cardiologico Monzino, IRCCS, Department of Clinical Electrophysiology and Cardiac Pacing, Milan, Italy (C. Tondo).ORCID 0000-0002-8500-8313
James S WareNational Heart and Lung Institute and MRC London Institute of Medical Sciences, Imperial College London, UK (J.S.W., S.K.P.).ORCID 0000-0002-6110-5880
Sanjay K PrasadNational Heart and Lung Institute and MRC London Institute of Medical Sciences, Imperial College London, UK (J.S.W., S.K.P.).ORCID 0000-0002-5637-0727
Leonardo CalòDepartment of Cardiology, Policlinico Casilino, Rome, Italy (L. Calò).ORCID 0000-0002-6062-5286
Eric SmithDepartment of Internal Medicine, Division of Cardiovascular Medicine, University of Michigan, Ann Arbor (E.S., A.H.).ORCID 0000-0002-5915-9492
Jodie InglesGenomics and Inherited Disease Program, Garvan Institute of Medical Research, and UNSW Sydney, Australia (J.I.).ORCID 0000-0002-4846-7676
Harikrishna TandriDepartment of Medicine, Division of Cardiology, Vanderbilt University Medical Center, Nashville, TN (H.T.).ORCID 0000-0003-2651-4352
Flavie AderSorbonne 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.).ORCID 0000-0001-7891-3385
Stacey PetersRoyal Melbourne Hospital, Melbourne, Victoria, Australia (S.P., D.Z., J.R.).ORCID 0000-0002-3593-3228
Dominica ZentnerRoyal Melbourne Hospital, Melbourne, Victoria, Australia (S.P., D.Z., J.R.).ORCID 0000-0003-0437-1581
Jeremy RussoRoyal Melbourne Hospital, Melbourne, Victoria, Australia (S.P., D.Z., J.R.).
Silvia RavaroIRCCS, Istituto Auxologico Italiano, Department of Cardiology, Cardiomyopathy Unit, Milan, Italy (S.R., L. Crotti).
Lia CrottiIRCCS, Istituto Auxologico Italiano, Department of Cardiology, Cardiomyopathy Unit, Milan, Italy (S.R., L. Crotti).ORCID 0000-0001-8739-6527
Sven DittmannDepartment of Cardiovascular Medicine, Institute for Genetics of Heart Diseases, University Hospital Münster, Germany (S.D., E.-S.B.).ORCID 0000-0001-5829-3676
Eric Schulze-BahrDepartment of Cardiovascular Medicine, Institute for Genetics of Heart Diseases, University Hospital Münster, Germany (S.D., E.-S.B.).
Eric D CarruthDepartment of Genomic Health, Geisinger, Danville, PA (E.D.C.).ORCID 0000-0001-8184-8676
Vikki A KrysovDepartment of Medicine, Division of Cardiovascular Medicine, Stanford Center for Inherited Cardiovascular Disease, Stanford University School of Medicine, CA (V.A.K., V.N.P.).ORCID 0009-0007-5026-0475
Victoria N ParikhDepartment of Medicine, Division of Cardiovascular Medicine, Stanford Center for Inherited Cardiovascular Disease, Stanford University School of Medicine, CA (V.A.K., V.N.P.).ORCID 0000-0002-5138-5559
Giulio ConteFondazione Cardiocentro Ticino, Department of Cardiology, Lugano, Switzerland (G.C.).ORCID 0000-0003-2248-3456
Arthur WildeAmsterdam UMC location University of Amsterdam, Department of Cardiology, the Netherlands (A.W.).ORCID 0000-0002-0528-0852
J Peter van TintelenDivision of Cardiology, University Medical Center Utrecht, the Netherlands (S.A.M., J.P.v.T., A.S.J.M.t.R.).ORCID 0000-0003-3854-6749
Anneline S J M Te RieleDivision of Cardiology, University Medical Center Utrecht, the Netherlands (S.A.M., J.P.v.T., A.S.J.M.t.R.).ORCID 0000-0002-4694-4790
Gianfranco SinagraCardiothoracovascular Department, Division of Cardiology, Azienda Sanitaria Universitaria Giuliano Isontina and University of Trieste, Italy (M.G., G.S., M.M.).ORCID 0000-0003-2700-8478
Luisa MestroniUniversity of Colorado Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora (K.M., M.J., L.M., M.T.).ORCID 0000-0003-1116-2286
Matthew TaylorUniversity of Colorado Cardiovascular Institute, University of Colorado Anschutz Medical Campus, Aurora (K.M., M.J., L.M., M.T.).ORCID 0000-0001-9043-0810
Marco MerloCardiothoracovascular Department, Division of Cardiology, Azienda Sanitaria Universitaria Giuliano Isontina and University of Trieste, Italy (M.G., G.S., M.M.).ORCID 0000-0002-1022-3131
Luigi AdamoDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).
Hugh CalkinsDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0002-9262-9433
Barbara BauceDepartment of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Italy (K.P., C.B., B.B.).ORCID 0000-0001-7357-293X
Elena ArbeloCardiology Department, Arrhythmia Section, Hospital Clínic, Universitat de Barcelona, Spain (E.A.).ORCID 0000-0003-0424-6393
Perry M ElliottInherited Cardiovascular Diseases Unit, St Bartholomew's Hospital, UCL Institute of Cardiovascular Science, London, UK (A.P., P.S., P.M.E.).ORCID 0000-0003-3383-3984
Estelle GandjbakhchSorbonne 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.).ORCID 0000-0002-4846-5021
Neal LakdawalaCenter for Advanced Heart Disease, Brigham and Women's Hospital Cardiovascular Medicine, Boston, MA (N.L.).ORCID 0000-0001-6458-5421
Adam HelmsDepartment of Internal Medicine, Division of Cardiovascular Medicine, University of Michigan, Ann Arbor (E.S., A.H.).ORCID 0000-0002-0234-6430
Cynthia A JamesDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0001-8040-4600
Nisha A GilotraDepartment of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (A.G., S.A.M., B.A., R.T.C., B.M., C. Tichnell, P.S., L.A., H.C., C.A.J., N.A.G.).ORCID 0000-0002-4511-8008
Funding
PATHOPHYSIOLOGY OF MYOCARDIAL DISEASEST32HL007227 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI Chulan Kwon, WENDY S POST · 1985 to 2026
$21.2MA Precision Medicine Approach to Arrhythmogenic CardiomyopathyR01HL141901 · NHLBI · GEISINGER CLINIC · PI CARRUTH, ERIC DAVID · 2019 to 2023
$3.1MNHLBI NIH HHS R01 HL141901NHLBI NIH HHS T32 HL007227
Abstract
backgroundInflammatory, 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.
methodsThe study was designed as an observational cohort study using all patients with pathogenic or likely pathogenic DSP variants in the
resultsA 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];
conclusionsImmunosuppressive treatment of myocarditis-like episodes in
Indexed as
DesmoplakinsImmunosuppressive AgentsMyocarditisAdultFemaleHeart FailureHumansMaleMiddle AgedPrognosisRecurrenceDesmoplakinsDSP protein, humanImmunosuppressive Agentsarrhythmias, cardiaccardiomyopathiesdeath, sudden, cardiacgeneticsinflammationmyocarditisrisk assessment
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