ABSTRACT
Ion-channelopathy syndromes-LQTS, CPVT, BrS, and ERS-emerge as leading causes of sudden unexplained death/sudden arrhythmic deaths in children and young adults. While pharmacological treatment with beta-blockade is the mainstay for LQTS and CPVT, in some patients, who are not responsive to beta blockers or noncompliant to the drug, non-pharmacological approaches including ICD, LCSD, or catheter ablation of VT/VFib triggers become an important alternative and necessity for treatment of life-threatening arrhythmias. LCDS has emerged as an effective treatment modality for LQTS and CPVT either as an adjunct treatment with a beta blocker or a stand-alone treatment. Treatments of symptomatic BrS and ERS are even more challenging because of limited therapeutic options: ICD and quinidine were the only accepted treatments in the past. But both have drawbacks: ICD does not prevent VT/VFib recurrences and in many symptomatic patients, frequent ICD discharges for VFib recurrences are life-threatening for many patients. While quinidine is considered to be effective for BrS and ERS patients, universal accessibility in many parts of the world, unwanted adverse effects, and patient compliance preclude wide use in a long term in most patients. Fortunately, catheter ablation of both VFib triggers and substrates is now recognized as safe and effective treatment for BrS and ERS patients and increasingly utilized worldwide. In this chapter, we discuss the role of these non-pharmacological interventions including how to perform catheter ablation of BrS and ERS in details.
