UMEM Educational Pearls

Title: Long QT Syndrome (LQTS) Part I

Keywords: Long QT Syndrome (PubMed Search)

Posted: 11/11/2012 by Semhar Tewelde, MD
Click here to contact Semhar Tewelde, MD

Despite advancement of molecular genetics dx of congenital long QT syndrome is based on ECG & clinical characteristics

Typical presentation is that a child/young adult experienced unexplained syncope or sudden death during physical exertion or emotional agitation

Bazett formula (QTc = QT x RR^1/2) is the most widely used method for measuring QTc

A QTc interval longer than 440 ms has been considered prolonged

1st described was QT prolongation w/congenital bilateral neural deafness and SCD (Jervell and Lange-Nielsen syndrome)

The more common form is QT prolongation w/o deafness and SCD (Romano-Ward syndrome)

Beta-blockers remain the mainstay treatment

Implantable cardioverter-defibrillator (ICD) is an effective therapeutic option to reduce mortality

Congenital and Acquired Long QT Syndrome.Cardiology in Review. 12(4):222-34, 2004 Jul-Aug