Inherited arrhythmia syndrome predisposing to sudden cardiac death

Figures

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  Figure 1. Distribution of inherited arrhythmias in probands (A) and their family members (B) in the Korean population [18]. ARVC, arrhythmogenic right ventricular cardiomyopathy; BrS, Brugada syndrome; CPVT, catecholaminergic polymorphic ventricular tachycardia; ERS, early repolarization syndrome; IVF, idiopathic ventricular fibrillation; LQTS, long QT syndrome; SQTS, short QT syndrome.

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  Figure 2. A diagnostic algorithm in inherited arrhythmia syndrome. LQTS, long QT syndrome; CPVT, catecholaminergic polymorphic ventricular tachycardia; SQTS, short QT syndrome; ARVC, arrhythmogenic right ventricular cardiomyopathy; ECG, electrocardiogram; CAG, coronary angiography; SA, signal averaged; EP, electrophysiological; QTc, corrected QT; VT, ventricular tachycardia; DDx, differential diagnosis; MRI, magnetic resonance image; CT, computed tomography; RV, right ventricle; VF, ventricular fibrillation. aBazett or Fridericia formula: standard and upper level (up to the 2nd or 3rd intercostal space), bEpinephrine for LQTS, CPVT: procainamide or flecainide for Brugada syndrome, cNext-generation sequencing panels for channelopathies and cardiomyopathies.

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  Figure 3. A 24-year-old female patient’s electrocardiographies showed marked prolongation of the QT interval and subsequent polymorphic ventricular tachycardia (PMVT) (top). Her genetic testing result was a positive genotype of potassium voltage-gated channel subfamily H member 2 (KCNH2). She was diagnosed with long QT syndrome (LQTS) type 2. The mechanism of lethal ventricular tachyarrhythmia in LQTS (bottom). PVC, premature ventricular contraction.

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  Figure 4. Electrocardiogram in a 26-year-old man showed early repolarization syndrome with prominent J waves (arrows) in inferolateral leads (top). Implantable cardioverter defibrillator examination demonstrated an episode of sudden onset ventricular fibrillation (bottom).