1078 recurrent flares of myocarditis: a challenging case

Abstract A 38-year-old woman with curly hair was admitted at the Emergency Department of our Hospital for typical thoracic pain associated with dyspnea and palpitations. Electrocardiogram showed sinus rhythm, 65 bpm, low voltages in the peripheral leads with fragmented QRS complex in the inferior leads and negative T-waves in anterior leads. Initial high-sensitivity cardiac troponin level was 46326 ng/L (n.v. < 16 ng/L). At presentation the patient was treated with Anakinra (anti-interleukin 1) and Metoprolol 200 mg/die because of a past medical history characterized by multiple myocarditis recurrences since 2012. Various therapies had been tested (Aspirin, Ibuprofen, Colchicine, Prednisone, Azathioprine), with a good efficacy of Mycophenolic acid, recently stopped to plan a pregnancy. Transthoracic echocardiography documented normal left ventricle (LV) ejection fraction and no alteration wall motion abnormalities; cardiac-MRI demonstrated LV late gadolinium enhancement with a non-ischemic subepicardial “ring-like” pattern. Endomyocardial biopsy was performed, and the ultrastructural analysis showed mitochondrial hyperplasia with focal swelling and marked alterations of intercalated disks. A high ventricular extrasystolic burden was registered at EKG monitoring. Gene sequencing revealed a pathogenetic variant of the gene coding for Desmoplakin. This is involved in the anchoring of intermediate filaments to desmosomal plaques and its pathogenetic variants are associated with an inflammatory and fibrotic LV predominant cardiomyopathy with an arrhythmogenic evolution (see Padua criteria). In the absence of approved therapies, in agreement with the limited available data and considering patient's history, Micofenolic acid was restarted. At 1-, 3- and 6-month follow-up the patient has remained asymptomatic with progressive declining in troponin levels after discharge.