ArticleJACC. Case reports2025
Desmoplakin Cardiomyopathy Presenting as Recurrent Myocarditis Treated With Immunosuppression.
Article in JACC. Case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- Colchicine in patients with chronic inflammatory cardiomyopathy: rationale and design of the CMP-MYTHiC.ESC heart failure · 2026Trial
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Authors and funding
7 authors.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundDesmoplakin (DSP) cardiomyopathy is a genetic cardiomyopathy which causes myocarditis, heart failure, and sudden death. CASE SUMMARY: A 42-year-old female presented with palmoplantar keratoderma, recurrent magnetic resonance imaging-confirmed myocarditis, and intense inflammation on cardiac positron emission tomography. Genetic testing confirmed DSP cardiomyopathy with a novel heterozygous DSP c.123C>G (p.Tyr41∗) truncating variant. Immunosuppression with prednisone and mycophenolate mofetil produced marked improvement on repeat positron emission tomography imaging. Predictive testing identified the same mutation in her 44-year-old sister. Cardiac magnetic resonance imaging revealed occult cardiomyopathy with severe myocardial fibrosis of similar phenotype. Both patients received heart failure therapies and were offered primary prevention implantable cardioverter defibrillator. DISCUSSION: A novel DSP truncating variant associated with inflammatory cardiomyopathy was identified. We propose a treatment algorithm to manage DSP cardiomyopathy incorporating predictive testing of relatives, immunosuppression of myocardial inflammation, risk stratification of sudden death, and initiation of heart failure therapies. TAKE-HOME MESSAGE: We present a novel DSP variant and algorithm to manage DSP cardiomyopathy.
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