Provocative Cases -> Teaching Case Reports D-PO01 - Featured Poster Session (ID 11) Poster

D-PO01-084 - Brugada Syndrome Due To Cardiac Sarcoidosis: Managing An Electrical Storm (ID 80)

 V. Rangaswamy: Nothing relevant to disclose.
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Background: Triggers for unmasking Brugada syndrome (BrS) are fever and drugs.
Objective: We report a case of ventricular fibrillation (VF) storm, in a patient with BrS diagnosed to have cardiac sarcoidosis (CS).
Results: A Seventeen-year boy presented with VF storm. His ECG showed dynamic type I Brugada pattern. Echocardiography was normal. VF storm responded to isoprenaline. Genetic test was negative for BrS. Cardiac MRI revealed myocardial edema in the ventricular septum with mid septal, right ventricular (RV) free wall scar. Positron emission tomography with 18-fluorodeoxy glucose (FDG PET) showed inflammation in interventricular septum and left ventricular papillary muscles with metabolically active mediastinal and right supraclavicular lymph nodes. He was treated with steroids and cilostazol. Repeat FDG-PET after 3 months showed resolution of inflammation. Following month, he presented with drug refractory VF storm triggered by short coupled premature ventricular contractions (PVCs). Endo-epicardial substrate map of RV showed scar, isolated late potentials and fractionated signals in endocardial and epicardial aspect of RV outflow tract, which were ablated. Trigger PVCs were also successfully ablated. Post ablation no tachycardia was induced with epicardial warm saline infusion. Type 1 Brugada ECG pattern subsided after ablation. There is no recurrence on follow-up of 3 months.
Conclusion: Clinical CS can present as BrS and VF storm despite adequate immunosuppressive therapy.