ABSTRACT
The discovery of the genetic basis of inherited arrhythmogenic syndromes over the past two decades has led to significant advancement in our understanding of diseases with predisposition towards sudden cardiac death (SCD) in patients with “normal” hearts. This chapter focuses on the current understanding of what are now distinct and well-understood genetically determined disorders/syndromes, which predispose patients to ventricular arrhythmias. It focuses on Long QT Syndrome (LQTS), Short QT Syndrome (SQTS), Brugada Syndrome, Catecholaminergic Polymorphic Ventricular Tachycardia, and Idiopathic Ventricular Fibrillation. Clinical presentation, diagnosis, and treatment techniques are described for each of the diseases. All patients with LQTS should avoid QT-prolonging drugs. Lifestyle modifications should include correction of factors that may cause QT prolongation such as imbalanced diets, avoidance of strenuous exercises. Implantation of defibrillators is recommended in patients with SQTS who are survivors of a cardiac arrest or have a documented spontaneous sustained Ventricular Tachycardia/Ventricular Fibrillation.
