Deepanjan Bhattacharya¹, Bapi Barman¹, Sanjeev S Mukherjee¹, Rohit Bhowmick¹
1- AIIMS Kalyani, India
Abstract Body
Introduction: Electrical storm (ES) is a medical emergency characterized by at least 3 repetitive episodes of sustained ventricular arrhythmias (VAs) within a 24-h period. Cardiac sarcoidosis is an infiltrative cardiomyopathy that results from granulomatous inflammation of the myocardium and may present with high-grade conduction disease, ventricular arrhythmias, and right or left ventricular dysfunction. Case: A 7-year-old boy , presented with intermittent palpitations and chest tightness, beginning at the age of 6 years, with documented RBBB tachycardia with right axis deviation. He was evaluated elsewhere where he was diagnosed as cardiac sarcoidosis based on increased uptake on Ga-68 DOTA PET CT. Cardiac MRI showed good biventricular function with no scarring. He underwent radiofrequency ablation of left anterior fascicular VT, followed by pacemaker implantation for AV nodal disease. Whole genome analysis did not reveal any pathogenic mutation. Now he presented with palpitations with syncope and ECG showed wide complex tachycardia with RBBB and left axis deviation. After cardioversion, he was started on amiodarone, mexiletene and propranolol, with calcium and magnesium supplementation. In view of refractory electrical storm, pulse methylprednisolone was added, followed by bilateral stellate ganglion blockade with 0.5% Bupivacaine under USG guidance. Subsequently , he remained arrhythmia free, and was discharged on tapering steroids and oral anti-arrhythmic medication.
Conclusion: Electrical storm is a life-threatening emergency with mortality rates approaching 60%, with infiltrative cardiomyopathies including sarcoidosis being a differential diagnosis. Stellate ganglion block is an effective tool for management with an efficacy of almost 60%, especially in cases which are drug refractory .