Congenital Long-QT Syndromes: A Clinical and Genetic Update From Infancy Through Adulthood

Long-QT syndromes (LQTSs) have been described in all ages and are a significant cause of cardiovascular mortality, especially in structurally normal hearts. Abnormalities in transmembrane ion conduction channels and structural proteins produce these clinical syndromes, labeled LQT1-LQT12; however, genotype-positive patients still represent only about 70% of LQTSs. Future research will determine the etiology of the remaining cases, further risk-stratify the known genetic defects, improve current treatment options for these syndromes, and uncover novel therapies. (Trends Cardiovasc Med 2008;18:216-224) (c) 2008, Elsevier Inc.