Case report: desmoplakin cardiomyopathy presenting as an inflammatory cardiomyopathy with repeated sudden cardiac arrests.

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 cardiomyopathie

SUBMITTER: Massie E 

PROVIDER: S-EPMC11181931 | biostudies-literature | 2024 Apr

REPOSITORIES: biostudies-literature