Predicting the long-QT genotype from clinical data: from sense to science.

In the past 3 decades, the congenital long-QT syndrome (LQTS) has emerged as an important paradigm for understanding arrhythmogenesis. An understanding of the electrophysiological basis of arrhythmias in LQTS has now merged with new molecular genetics, solving some problems and raising new ones both in clinical management and in basic arrhythmia mechanisms (for review, see Roden and Spooner1). In this scientific evolution, the international LQT registry has proved to be of paramount importance. Since 1979, data from this registry have proved to be of great value for the diagnosis, prognosis, and management of LQT patients and their relatives, and in more recent years, data from the registry represent a reliable source for attempts to correlate phenotype with genotype and vice versa.