We present a case of recurrent myocardial inflammation in a young female patient with arrhythmogenic cardiomyopathy (AC). After comprehensive assessment, inclusive of cardiac magnetic resonance, endomyocardial biopsy, and genetic testing, a diagnosis of a flare in the context of desmoplakin (DSP)-related AC was made. Treatment with mycophenolic acid (MPA) was resumed after previous clinical efficacy with consistent freedom from recurrences at 24-month follow-up. This case highlights the potential of MPA as a novel therapeutic approach for DSP-related AC and explores its pathophysiological rationale.
Mycophenolic Acid for Desmoplakin-Related Cardiomyopathy: A Possible New Arrow in the Quiver
Angelini, FilippoFirst
;Ravera, Francesco;Gobello, Giulia;Manai, Rossella;Bocchino, Pier Paolo;Barreca, Antonella;Deaglio, Silvia;Pidello, Stefano;De Ferrari, Gaetano Maria;Dusi, Veronica
Last
2024-01-01
Abstract
We present a case of recurrent myocardial inflammation in a young female patient with arrhythmogenic cardiomyopathy (AC). After comprehensive assessment, inclusive of cardiac magnetic resonance, endomyocardial biopsy, and genetic testing, a diagnosis of a flare in the context of desmoplakin (DSP)-related AC was made. Treatment with mycophenolic acid (MPA) was resumed after previous clinical efficacy with consistent freedom from recurrences at 24-month follow-up. This case highlights the potential of MPA as a novel therapeutic approach for DSP-related AC and explores its pathophysiological rationale.
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