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In cardiac ion channel diseases, there are a part of primary electrical disorders which easily lead to sudden cardiac death (SCD) in the absence of structural heart diseases, accounting for approximately 30% of young SCD patients. These electrical disorders include congenital long QT syndrome, congenital short QT syndrome, Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. These diseases usually have low penetrance and lack typical clinical manifestation, greatly challenging clinical diagnosis. However, SCD is probably the first symptom of primary cardiac ion channel diseases, and therefore it is especially important to make early diagnosis. This paper comprehensively introduces the clinical manifestations and diagnostic criteria, genetic background, pathophysiologic mechanism, and therapy of the above four kinds of primary cardiac ion channel diseases, in order to provide a clue for prevention and control of severe arrhythmias and even SCD. [ABSTRACT FROM AUTHOR]

Drug treatment and/or implantable cardioverter defibrillator (ICD) implantation are the most widely accepted first-line therapies for channelopathic patients who have recurrent syncope, sustained ventricular tachycardia (VT), or documented ventricular fibrillation (VF), or are survivors of cardiac arrest. In recent years, there have been significant advances in mapping techniques and ablation technology, coupled with better understanding of the mechanisms of ventricular tachyarrhythmia in channelopathies. Catheter ablation has provided important insights into the role of the Purkinje network and the right ventricular outflow tract in the initiation and perpetuation of VT/VF, and has evolved as a promising treatment modality for ventricular tachyarrhythmia even in channelopathies. When patients are exposed to a high risk of sudden cardiac death or deterioration of their quality of life due to episodes of tachycardia and frequent ICD discharges, catheter ablation may be an effective treatment option to reduce the risk of sudden cardiac death and decrease the frequency of cardiac events. In this review, we summarize the current understanding of catheter ablation for VT/VF in patients with channelopathies including Brugada syndrome, idiopathic VF, long QT syndrome, and catecholaminergic polymorphic VT.

Drug treatment and/or implantable cardioverter defibrillator (ICD) implantation are the most widely accepted first-line therapies for channelopathic patients who have recurrent syncope, sustained ventricular tachycardia (VT), or documented ventricular fibrillation (VF), or are survivors of cardiac arrest. In recent years, there have been significant advances in mapping techniques and ablation technology, coupled with better understanding of the mechanisms of ventricular tachyarrhythmia in channelopathies. Catheter ablation has provided important insights into the role of the Purkinje network and the right ventricular outflow tract in the initiation and perpetuation of VT/VF, and has evolved as a promising treatment modality for ventricular tachyarrhythmia even in channelopathies. When patients are exposed to a high risk of sudden cardiac death or deterioration of their quality of life due to episodes of tachycardia and frequent ICD discharges, catheter ablation may be an effective treatment option to reduce the risk of sudden cardiac death and decrease the frequency of cardiac events. In this review, we summarize the current understanding of catheter ablation for VT/VF in patients with channelopathies including Brugada syndrome, idiopathic VF, long QT syndrome, and catecholaminergic polymorphic VT. [ABSTRACT FROM AUTHOR]

Sudden cardiac death ( SCD) has an enormous impact on those who are left behind, evoking strong feelings of anxiety and incomprehension because such a dramatic event was not anticipated. Moreover, over the last decade a prominent genetic contribution to the pathogenesis of SCD has been unveiled. As many inherited cardiac diseases show an autosomal dominant pattern of inheritance, the risk of carrying the same inherited predisposition is a real concern for the relatives. In this article, we discuss the major causes of primary electrical disorders, cardiomyopathies and thoracic aortic dissection and address issues in genotype-phenotype correlation, personalized management and cardiogenetic counselling. [ABSTRACT FROM AUTHOR]