Short QT syndrome

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.

Cardiac disease

(SQTS1) KCNH2 [HSA:3757] [KO:K04905]
(SQTS2) KCNQ1 [HSA:3784] [KO:K04926]
(SQTS3) KCNJ2 [HSA:3759] [KO:K04996]

QT interval <300-320 msec

Quinidine [DR:D08458] (still under evaluation)

PMID:15890322

Schimpf R, Wolpert C, Gaita F, Giustetto C, Borggrefe M

Short QT syndrome.

Cardiovasc Res 67:357-66 (2005)

PMID:19026859

Zareba W, Cygankiewicz I

Long QT syndrome and short QT syndrome.

Prog Cardiovasc Dis 51:264-78 (2008)

PMID:20126594

Crotti L, Taravelli E, Girardengo G, Schwartz PJ

Congenital short QT syndrome.

Indian Pacing Electrophysiol J 10:86-95 (2010)