Prophylactic left thoracic sympathectomy to prevent electrical storms in CPVT patients needing ICD placement

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT), a life threatening arrhythmia induced by sympathetic stimulation in susceptible individuals is often refractory to antiarrhythmic agents. First line of treatment, beta-blockers can be ineffective in up to 50% with implantable cardioverter-defibrillator (ICD) placement for refractory cases. Paradoxically ICD can be arryhthmogenic from shock-associated sympathetic stimulation, initiating more shocks and " electrical storms" This has led to the use of more effective beta blockade offered by left sympathectomy, now performed by minimally invasive video assisted thoracoscopic surgery (VATS). Sympathectomy has been traditionally performed long after ICD placement, after the patient has experienced multiple shocks, thus necessitating two procedures. We report simultaneous ICD insertion and thoracoscopic sympathectomy in a 10 year-old boy with CPVT, and suggest it as a better approach than sequential procedures. To our knowledge this is first such reported case.