433 A PRO-ARRHYTHMIC DOUBLE HIT

Abstract A 42-year-old man went to the emergency room complaining about dyspnea and palpitations. He reported an episode of atrial fibrillation (AF) with symptoms dizziness 8 years before. had a family history ischemic cardiopathy, his father has suffered from AF since young age.Acute course: The ECG revealed at high ventricular frequency. echocardiography demonstrated mild reduction left function (EF 45%) diffuse hypokinesis, mitral valve prolapse (MVP) regurgitation. blood tests showed troponin peak 750 ng/L (n.v. 0–34 ng/L). suspect was acute coronary syndrome. Thus, he treated dual antiplatet therapy low doses beta-blockers, underwent coronarography, which no significant lesions. So, after transesophageal that excluded thrombosis, loading dose amiodarone, successful electrical cardioversion performed. discharge diagnosis disfunction. Three months later: came back because suffering some days progressive epigastric retrosternal pain radiating shoulders. were unchanged, troponin-I values 79 ng/L-74 ng/L. Therefore, cardiac magnetic resonance (CMR), normal biventricular dimensions (LVEF 64%, LVEDV 80 ml/mq), MVP annular disjunction (MAD) curling infero-lateral basal wall (Figure A). native T1, T2 ECV increased, there wide subepicardial late gadolinium enhancement (LGE). conclusion active myocarditis. Moreover, endomyocardial biopsy focal lymphocytic infiltration, PCRs for common cardiotropic viruses or bacteria negative. Six characterized by QRS voltages in limb leads B). CMR non-ischemic fibrosis ring-like pattern C). 24-hours Holter premature beats right bundle branch block/superior inferior axis morphology D), keeping MVP. next step genetic testing resulted positive Filamin-C (FLNC) mutation associated cardiomyopathy. final “Hot-phase Left Ventricular Arrhythmogenic Cardiomyopathy (ACM), paroxysmal AF”. Discussion: ACM is rare hereditary heart muscle disease, “hot phase” represents its uncommon clinical presentation. Mutations FLNC gene could be involved pathogenesis ACM. are few cases literature correlation between mutations may cross-sectional substrate both these pro-arrhythmic conditions.