Channelopathies in children and adults

In the last decade, pediatric cardiologists have witnessed a revolution in the knowledge of the pathophysiology of rare arrhythmias. The identification of the molecular basis of several hereditary arrhythmia syndromes has been instrumental in this development. Within 12 years the number of causal genes has increased from two in 1995 to at least 24 early 2007 (Table I). Based on this knowledge, established treatment strategies in the 1990s have been modified during the most recent years. This leads to timely and tailored treatment of (asymptomatic) gene carriers, both through personalized lifestyle advices and pharmacologically. At the same time and of equal importance, unaffected family members (noncarriers) can be reassured.