TY - JOUR
T1 - Congenital Long-QT Syndromes
T2 - A Clinical and Genetic Update From Infancy Through Adulthood
AU - Webster, Gregory
AU - Berul, Charles I.
PY - 2008/8
Y1 - 2008/8
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=58749096216&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=58749096216&partnerID=8YFLogxK
U2 - 10.1016/j.tcm.2008.11.002
DO - 10.1016/j.tcm.2008.11.002
M3 - Review article
C2 - 19185812
AN - SCOPUS:58749096216
SN - 1050-1738
VL - 18
SP - 216
EP - 224
JO - Trends in Cardiovascular Medicine
JF - Trends in Cardiovascular Medicine
IS - 6
ER -