Long QT Syndrome is a genetic disorder characterized by a prolonged QT interval on electrocardiogram that can cause dangerous arrhythmias and sudden cardiac death. Symptoms include unexplained fainting, seizures, or sudden death, especially with exercise or emotions. Treatment involves beta blockers, implantable cardioverter defibrillators, or left stellate ganglionectomy depending on risk level and genotype. Ongoing research seeks to better understand genotype-phenotype relationships and develop mutation-specific therapies.

1. The Long QT Syndrome Overview and Management Edited by A.Kharazi M.D Cardiac electrophysiologist

3. Long QT Syndrome Background and the Risk of Sudden Cardiac Death

8. LQTS: Identification

10. Syncope Slow Onset Abrupt Onset Abrupt Onset Slow Offset Abrupt Offset Slow Offset Seizure disorder Hyperventilation Hypoglycemia Obstructive Arrhythmic Vascular Aortic Stenosis, HCM, Myxoma Brady Tachy Vasovagal, Orthostatic Hypertension

12. Holter ECG Recording in LQTS Patient with Syncope ( representative strips of ECG recording, part 1 of 2)

13. Holter ECG Recording in LQTS Patient with Syncope (representative strips of ECG recording, part 2 of 2)

15. LQTS ECG Patterns Circ 1992;85[Suppl I]:I140-I144

16. Additional LQTS ECG Patterns Circ 1992;85[Suppl I]:I140-I144

19. QTc Interval and Risk AJC 1993;72:21B QTc Risk for Cardiac Event

21. Probability of Cardiac Event in LQTS Circ 1991;84:1136-1144 Probands Affected Undetermined Unaffected

23. Occurrence of Gene-Specific Triggers Circ 2001;103:89-95 Percent

24. Basis for the Long QT Syndrome JCE 1999;10:1664-1683

26. T-wave Morphology in LQTS by Genotype Moss AJ, et al. Circulation 1995;92:2929-2934

28. Therapies Available and Current Management

32. LQTS: Cardiac Events Before and After  -blockers Probands AFM † (n=581) (n=288) Risk exposure, yrs 5.2 4.5 (pre- and post-  B) Pre-  B Post-  B Pre-  B Post  B Pts with events 462 194* 92 49* Number events 1671 623* 245 138* Events/pt 3.0 1.1* 0.9 0.5* Events/pt/year 1.0 0.3* 0.3 0.15* Circ 2000;101:616-623 † Affected Family Member * P<0.01 vs. pre-  -blocker

34. Probability of Cardiac Event Circ 2000;101:616-623

35. Cumulative Probability of LQTS- Related Death w/ ß-blockers Circ 2000;101:616-623

43. Ongoing Research

45. Case Studies