KEGG   DISEASE: Short QT syndromeHelp
Entry
H00725                      Disease                                

Name
Short QT syndrome
Description
Short QT syndrome (SQTS) is a cardiovascular disorder resulting from mutations in cardiac ion channels. The mutation of genes (KCNH2, KCNQ1, and KCNJ2) encoding for cardiac potassium channels plays a central role in SQTS. SQTS is characterized by constantly short QT interval associated with atrial fibrillation, syncopal episodes, and sudden cardiac death. The implantable cardioverter defibrillator (ICD) therapy in patients with a short QT syndrome has an increased risk due to possible T wave oversensing.
Category
Cardiovascular disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Cardiac diseases
   H00725  Short QT syndrome
Human diseases in ICD-11 classification [BR:br08403]
 11 Diseases of the circulatory system
  Cardiac arrhythmia
   BC65  Cardiac arrhythmia associated with genetic disorder
    H00725  Short QT syndrome
BRITE hierarchy
Gene
(SQTS1) KCNH2 [HSA:3757] [KO:K04905]
(SQTS2) KCNQ1 [HSA:3784] [KO:K04926]
(SQTS3) KCNJ2 [HSA:3759] [KO:K04996]
Other DBs
ICD-11: BC65.2
OMIM: 609620 609621 609622
Reference
  Authors
Schimpf R, Wolpert C, Gaita F, Giustetto C, Borggrefe M
  Title
Short QT syndrome.
  Journal
Cardiovasc Res 67:357-66 (2005)
DOI:10.1016/j.cardiores.2005.03.026
Reference
  Authors
Zareba W, Cygankiewicz I
  Title
Long QT syndrome and short QT syndrome.
  Journal
Prog Cardiovasc Dis 51:264-78 (2008)
DOI:10.1016/j.pcad.2008.10.006
Reference
  Authors
Crotti L, Taravelli E, Girardengo G, Schwartz PJ
  Title
Congenital short QT syndrome.
  Journal
Indian Pacing Electrophysiol J 10:86-95 (2010)

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