[Molecular basis of primary electrical heart diseases]

The last decade has seen rapid progress in our understanding of the molecular basis of arrhythmias, particularly concerning hereditary arrhythmia syndromes. This has led to significant improvement regarding differentiation, risk stratification and therapy in these patients and their families. However, there is mounting evidence that the knowledge obtained by studying these rare monogenic disorders will also enable us to dissect the molecular mechanisms underlying polygenetic and multi-factorial arrhythmias that are by far more common in clinical practice. The goal of this review is to give a brief overview of current knowledge on the molecular basis of primary electrical heart diseases. A focus is on the long QT syndrome.