AI Article Synopsis
- Desmoplakin cardiomyopathy is a recently defined heart condition causing left ventricular dysfunction, often leading to inflammation, fibrosis, and arrhythmias.
- A case study highlights a 46-year-old woman with this condition, who experienced multiple cardiac arrests due to a new variant in the desmoplakin gene and showed little improvement after receiving immunosuppressive treatment.
- The condition should be included in the evaluation of inflammatory heart diseases, and while some patients may benefit from immunosuppressive therapy, its effectiveness remains largely unknown.
Article Abstract
Background: Desmoplakin cardiomyopathy has been recently classified as a non-dilated left ventricular cardiomyopathy, which is characterized by inflammatory-like episodes followed by left ventricular fibrosis/dysfunction and ventricular arrhythmias. Specific management is unclear.
Case Summary: We report a detailed case of a 46-year-old Caucasian woman presenting with repeated sudden cardiac arrests who was diagnosed with a new variant in the desmoplakin gene. Because the initial 18F-fluorodeoxyglucose positron emission tomography scan showed significant hypermetabolism, she was treated with immunosuppressors, with only minimal improvement on imaging.
Discussion: Desmoplakin cardiomyopathy should be considered in the differential diagnosis of inflammatory cardiomyopathies. Little is known about the use of immunosuppressive treatments, but it could be reasonable for some selected patients.
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|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11181931 | PMC |
| http://dx.doi.org/10.1093/ehjcr/ytae160 | DOI Listing |
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