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{{Infobox_Disease |
{{Infobox Disease |
   Name          = Short QT syndrome |
   Name          = Short QT syndrome |
   Image          = SinusRhythmLabels.png |
   Image          = SinusRhythmLabels.svg |
   Caption        = Schematic representation of normal ECG trace ''([[sinus rhythm]]),'' with waves, segments, and intervals labeled. |
   Caption        = Schematic representation of normal ECG trace ''([[sinus rhythm]]),'' with waves, segments, and intervals labeled. |
   DiseasesDB    = 11105 |
   DiseasesDB    = 11105 |
   ICD10          = |
   ICD10          = {{ICD10|R|94|3|r|94}}|
   ICD9          = |
   ICD9          = |
   ICDO          = |
   ICDO          = |
   OMIM          = |
   OMIM          = |
   MedlinePlus    = |
   MedlinePlus    = |
  eMedicineSubj  = |
  eMedicineTopic = |
   MeshID        = |
   MeshID        = |
}}
}}
{{SI}}
{{Short QT syndrome}}
{{WikiDoc Cardiology Network Infobox}}
{{CMG}} {{AE}}{{sumanthK}}
{{CMG}}


'''Associate Editor-In-Chief:''' {{CZ}}
{{SK}} SQTS; short QT; short QTc; QT interval shortening
==Overview==
'''Short QT syndrome''' is a [[genetics|genetic]] disease of the electrical system of the [[heart]].  It consists of a constellation of signs and symptoms, consisting of a short [[QT interval]] interval on [[EKG]] (≤ 300 ms) that doesn't significantly change with heart rate. Tall and peaked T waves are often present, and the heart is structurally normal.  Short QT syndrome appears to be inherited in an [[autosomal dominant]] pattern, and a few affected families have been identified.


==Symptoms and signs==
==[[Short_QT_syndrome_overview|Overview]]==


Individuals with short QT syndrome frequently complain of palpitations and may have [[syncope]] (loss of consciousness) that is unexplained. Mutations in the ''KCNH2'', ''KCNJ2'', and ''KCNQ1'' genes cause short QT syndrome. These genes provide instructions for making proteins that act as channels across the cell membrane. These channels transport positively charged atoms (ions) of potassium into and out of cells. In cardiac muscle, these ion channels play critical roles in maintaining the heart's normal rhythm. Mutations in the ''KCNH2'', ''KCNJ2'', or ''KCNQ1'' gene increase the activity of the channels, which changes the flow of potassium ions between cells. This disruption in ion transport alters the way the heart beats, leading to the abnormal heart rhythm characteristic of short QT syndrome. Short QT syndrome appears to have an autosomal dominant pattern of inheritance.
==[[Short_QT_syndrome_historical_perspective|Historical Perspective]]==


Due to the [[autosomal dominant]] inheritance pattern, most individuals will have family members with a history of unexplained or sudden death at a young age (even in [[infancy]]), palpitations, or [[atrial fibrillation]].
==[[Short QT syndrome classification|Classification]]==


Short QT syndrome is associated with an increased risk of sudden cardiac death, most likely due to [[ventricular fibrillation]].
==[[Short QT syndrome pathophysiology|Pathophysiology]]==


==Diagnosis==


The diagnosis of short QT syndrome consists of characteristic history and findings on [[EKG]] and electrophysiologic testing. There are currently no set guidelines for the diagnosis of short QT syndrome.
==[[Short QT syndrome causes|Causes]]==


<gallery>
==[[Short QT syndrome triggers|Triggers]]==
Image:autodominant2.jpg|Short QT syndrome is inherited in an [[autosomal dominant]] pattern.
</gallery>


===Electrocardiogam===
==[[Short QT syndrome differential diagnosis|Differentiating Short QT Syndrome from other Diseases]]==


The characteristic findings of short QT syndrome on [[EKG]] are a short QT interval, typically ≤ 300 ms, that doesn't significantly change with the heart rate.  Tall, peaked T waves may also be noted.  Individuals may also have an underlying [[atrium (anatomy)|atrial]] rhythm of [[atrial fibrillation]].
==[[Short QT syndrome epidemiology and demographics|Epidemiology and Demographics]]==


===Electrophysiologic Studies===
==[[Short QT syndrome screening|Screening]]==


In the electrophysiology lab, individuals with short QT syndrome are noted to have short refractory periods, both in the [[atrium (anatomy)|atria]] as well as in the [[Ventricle (heart)|ventricles]].  Also, [[ventricular fibrillation]] is frequently induced on [[programmed stimulation]].
==[[Short QT syndrome natural history, complications and prognosis|Natural History, Complications, Prognosis]]==


==Etiology==
==[[Diagnosis]]==
[[Short QT syndrome diagnostic criteria|Diagnostic Criteria]] | [[Short QT syndrome history and symptoms|History and Symptoms]] | [[Short QT syndrome physical examination|Physical Examination]] |  [[Short QT syndrome laboratory findings|Laboratory Findings]] | [[Short QT syndrome electrocardiogram|Electrocardiogram]] | [[Short QT syndrome electrophysiologic studies|Electrophysiologic Studies]] |  [[Short QT syndrome genetic testing|Genetic Testing]]


The etiology of short QT syndrome is unclear at this time.  A current [[hypothesis]] is that short QT syndrome is due to increased activity of outward potassium currents in phase 2 and 3 of the [[cardiac action potential]].  This would cause a shortening of the plateau phase of the action potential (phase 2), causing a shortening of the overall action potential, leading to an overall shortening of refractory periods and the QT interval.
==[[Treatment]]==
[[Short QT syndrome AICD placement|AICD placement]] | [[Short QT syndrome medical therapy|Medical Therapy]]


In the families afflicted by short QT syndrome, two different [[missense]] [[mutation]]s have been described in the ''human ether-a-go-go [[gene]] ([[HERG]])''.  These mutations result in expression of the same amino acid change in the cardiac [[cardiac action potential|I<sub>Kr</sub> ion channel]].  This mutated I<sub>Kr</sub> has increased activity compared to the normal ion channel, and would theoretically explain the above hypothesis.
{{Electrocardiography}}
 
==Treatment==
 
Currently, the only effective treatment option for individuals with short QT syndrome is implantation of an [[implantable cardioverter-defibrillator]] (ICD).
 
A recent study has suggested that the use of certain [[antiarrhythmic agents]], particularly [[quinidine]], may be of benefit in individuals with short QT syndrome due to their effects on prolonging the action potential and by their action on the I<sub>K</sub> channels.<ref>{{cite journal | author=Gaita F, Giustetto C, Bianchi F, Schimpf R, Haissaguerre M, Calo L, Brugada R, Antzelevitch C, Borggrefe M, Wolpert C. | title=Short QT syndrome: pharmacological treatment. | journal=J Am Coll Cardiol | year=2004 | pages=1494-9 | volume=43 | issue=8 | id=PMID 15093889}}</ref>  While the use of these agents alone is not indicated at present, there may be benefit of adding these agents to individuals who have already had ICD implantation to reduce the number of arrhythmic events.


==See also==
* [[Long QT syndrome]]
==References==
<div class="references-small">
<references />
</div>
==Additional resource==
*[http://ghr.nlm.nih.gov/condition=shortqtsyndrome?wf=1 Short QT syndrome]
{{Electrocardiography}}
{{SIB}}
[[it:Sindrome del QT breve]]
[[it:Sindrome del QT breve]]
[[ru:Синдром короткого интервала QT]]
[[ru:Синдром короткого интервала QT]]


[[Category:Cardiology]]
[[Category:Cardiology]]
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[[Category:Genetic disorders]]
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[[Category:Syndromes]]
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Latest revision as of 18:49, 26 June 2020

Short QT syndrome
Schematic representation of normal ECG trace (sinus rhythm), with waves, segments, and intervals labeled.
ICD-10 R94.3
DiseasesDB 11105

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sumanth Khadke, MD[2]

Synonyms and keywords: SQTS; short QT; short QTc; QT interval shortening

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Triggers

Differentiating Short QT Syndrome from other Diseases

Epidemiology and Demographics

Screening

Natural History, Complications, Prognosis

Diagnosis

Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Electrophysiologic Studies | Genetic Testing

Treatment

AICD placement | Medical Therapy

it:Sindrome del QT breve


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