Trends in Cardiovascular Medicine
Volume 18, Issue 6 , Pages 216-224, August 2008

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

  • Gregory Webster
    • ,
  • Charles I. Berul

      Affiliations

    • Corresponding Author InformationAddress correspondence to: Charles I. Berul, MD, Senior Associate in Cardiology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115. Tel.: +617 355 6432; fax: +617 566 5671

Department of Cardiology, Children's Hospital Boston

Department of Pediatrics, Harvard Medical School

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.

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PII: S1050-1738(08)00125-4

doi:10.1016/j.tcm.2008.11.002

Trends in Cardiovascular Medicine
Volume 18, Issue 6 , Pages 216-224, August 2008

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