Risk stratification for sudden cardiac death in primary electrical disorders.

Risk stratification is extremely important for primary electrical diseases because the low-risk patients are likely to remain asymptomatic through life whereas the high-risk patients often die suddenly, unless properly treated. This chapter addresses what is currently known about risk stratification for long QT syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia, and Brugada syndrome. It will become evident that the knowledge and current usefulness of risk stratification has major gaps between these four disorders. It is advanced, mature, and very useful for the clinical management of long QT syndrome; rather primitive and still insufficient for short QT syndrome and catecholaminergic polymorphic ventricular tachycardia; and partly adequate and partly frustratingly controversial for Brugada syndrome. The limited numerosity hampers risk stratification for short QT syndrome and catecholaminergic polymorphic ventricular tachycardia, whereas the divergent interpretations of the results of the electrophysiological study have so far prevented a uniform assessment for Brugada syndrome. Long QT syndrome provides a useful example of progress in risk stratification.