Heart failure & cardiomyopathiesCohort study

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

CirculationPublished

  • Observational cohort study
  • multicentre

Authors' conclusion

Immunosuppressive treatment of myocarditis-like episodes in DSP patients was associated with improved combined ventricular arrhythmia or heart failure outcomes

Study context

Population
Patients with pathogenic or likely pathogenic desmoplakin (DSP) variants in the DSP-ERADOS Network, 31 institutions177 with myocarditis-like episode
Participants
1,014 patients
Exposure
Immunosuppressive treatment vs No immunosuppressive treatment
  • Immunosuppressive treatmentn = 63
Outcome
Ventricular arrhythmia and HF end points
Follow-up
Median 6.4 years

Main finding

As reported in the abstract

Immunosuppressive treatment vs No immunosuppressive treatmentHazard ratio (95% CI)P value
ventricular arrhythmia
0.18(0.07–0.45)
P < 0.001
HF
0.09(0.02–0.39)
P = 0.001

Observational data: associations, not effects.

Further findings

OutcomeHR (95% CI)P value
Recurrent myocarditis-like episodes after completion of initial immunosuppression0.85 (0.47–1.54)P = 0.599

NSAIDs or colchicine treatment

Nonsteroidal anti-inflammatory drugs or colchicine did not influence outcomes

Original publication

Prognostic Role of Myocarditis-Like Episodes and Their Treatment in Patients With Pathogenic Desmoplakin Variants. Circulation. Published 20 August 2025. doi:10.1161/circulationaha.125.073919

Summarised from the published abstract; every number is checked against it