Long QT syndrome: Difference between revisions

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[[File:Long_QT_syndrome_type_1.jpg|Long QT syndrome type 1|thumb]] '''Long QT syndrome''' ('''LQTS''') is a [[genetic disorder]] that affects the [[heart]]'s electrical activity. It is characterized by a prolonged [[QT interval]] on an [[electrocardiogram]] (ECG), which can lead to [[arrhythmias]], [[syncope]], and sudden [[cardiac arrest]].  
{{SI}}
 
{{Infobox medical condition
| name            = Long QT syndrome
| image          = [[File:Long_QT_syndrome_type_1.jpg|250px]]
| caption        = Electrocardiogram showing Long QT syndrome
| field          = [[Cardiology]]
| synonyms        = LQTS
| symptoms        = [[Syncope]], [[seizures]], [[sudden cardiac death]]
| complications  = [[Torsades de pointes]], [[ventricular fibrillation]]
| onset          = Congenital or acquired
| duration        = Lifelong
| causes          = Genetic mutations, certain medications, electrolyte imbalances
| risks          = Family history, certain medications, electrolyte disturbances
| diagnosis      = [[Electrocardiogram]], [[genetic testing]]
| differential    = [[Ventricular tachycardia]], [[Brugada syndrome]], [[catecholaminergic polymorphic ventricular tachycardia]]
| prevention      = Avoiding QT-prolonging drugs, managing electrolyte levels
| treatment      = [[Beta blockers]], [[implantable cardioverter-defibrillator]], [[left cardiac sympathetic denervation]]
| prognosis      = Variable, risk of sudden death
| frequency      = 1 in 2,000 people
}}
'''Long QT syndrome''' ('''LQTS''') is a [[genetic disorder]] that affects the [[heart]]'s electrical activity. It is characterized by a prolonged [[QT interval]] on an [[electrocardiogram]] (ECG), which can lead to [[arrhythmias]], [[syncope]], and sudden [[cardiac arrest]].  
==Pathophysiology==
==Pathophysiology==
Long QT syndrome is caused by mutations in genes that encode for [[ion channels]] in the heart. These ion channels are responsible for the flow of [[potassium]], [[sodium]], and [[calcium]] ions, which are crucial for the heart's electrical activity. The most commonly affected genes include [[KCNQ1]], [[KCNH2]], and [[SCN5A]]. Mutations in these genes can disrupt the normal flow of ions, leading to a prolonged QT interval.
Long QT syndrome is caused by mutations in genes that encode for [[ion channels]] in the heart. These ion channels are responsible for the flow of [[potassium]], [[sodium]], and [[calcium]] ions, which are crucial for the heart's electrical activity. The most commonly affected genes include [[KCNQ1]], [[KCNH2]], and [[SCN5A]]. Mutations in these genes can disrupt the normal flow of ions, leading to a prolonged QT interval.
==Types==
==Types==
There are several types of Long QT syndrome, classified based on the specific gene mutation:
There are several types of Long QT syndrome, classified based on the specific gene mutation:
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* '''LQT2''': Caused by mutations in the [[KCNH2]] gene.
* '''LQT2''': Caused by mutations in the [[KCNH2]] gene.
* '''LQT3''': Caused by mutations in the [[SCN5A]] gene.
* '''LQT3''': Caused by mutations in the [[SCN5A]] gene.
==Symptoms==
==Symptoms==
Individuals with Long QT syndrome may experience:
Individuals with Long QT syndrome may experience:
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* [[Seizures]]
* [[Seizures]]
* Sudden [[cardiac arrest]]
* Sudden [[cardiac arrest]]
==Diagnosis==
==Diagnosis==
Diagnosis of Long QT syndrome typically involves:
Diagnosis of Long QT syndrome typically involves:
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* [[Holter monitor]] to record heart activity over 24-48 hours.
* [[Holter monitor]] to record heart activity over 24-48 hours.
* [[Exercise stress test]] to observe heart activity during physical exertion.
* [[Exercise stress test]] to observe heart activity during physical exertion.
==Treatment==
==Treatment==
Treatment options for Long QT syndrome include:
Treatment options for Long QT syndrome include:
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* [[Implantable cardioverter-defibrillator]] (ICD) for individuals at high risk of sudden cardiac arrest.
* [[Implantable cardioverter-defibrillator]] (ICD) for individuals at high risk of sudden cardiac arrest.
* [[Lifestyle modifications]] such as avoiding strenuous exercise and certain medications that can prolong the QT interval.
* [[Lifestyle modifications]] such as avoiding strenuous exercise and certain medications that can prolong the QT interval.
==Prognosis==
==Prognosis==
With appropriate treatment and lifestyle modifications, individuals with Long QT syndrome can lead relatively normal lives. However, the risk of sudden cardiac arrest remains, particularly in untreated individuals.
With appropriate treatment and lifestyle modifications, individuals with Long QT syndrome can lead relatively normal lives. However, the risk of sudden cardiac arrest remains, particularly in untreated individuals.
 
==See also==
==Related Pages==
* [[Arrhythmia]]
* [[Arrhythmia]]
* [[Cardiac arrest]]
* [[Cardiac arrest]]
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* [[Ion channel]]
* [[Ion channel]]


==Categories==
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Genetic disorders]]
[[Category:Genetic disorders]]
[[Category:Electrophysiology]]
[[Category:Electrophysiology]]
{{Cardiology}}
{{Cardiology}}
{{medicine-stub}}
{{medicine-stub}}

Latest revision as of 04:09, 8 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD's medical weight loss NYC, sleep center NYC
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Long QT syndrome
File:Long QT syndrome type 1.jpg
Synonyms LQTS
Pronounce N/A
Specialty N/A
Symptoms Syncope, seizures, sudden cardiac death
Complications Torsades de pointes, ventricular fibrillation
Onset Congenital or acquired
Duration Lifelong
Types N/A
Causes Genetic mutations, certain medications, electrolyte imbalances
Risks Family history, certain medications, electrolyte disturbances
Diagnosis Electrocardiogram, genetic testing
Differential diagnosis Ventricular tachycardia, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia
Prevention Avoiding QT-prolonging drugs, managing electrolyte levels
Treatment Beta blockers, implantable cardioverter-defibrillator, left cardiac sympathetic denervation
Medication N/A
Prognosis Variable, risk of sudden death
Frequency 1 in 2,000 people
Deaths N/A


Long QT syndrome (LQTS) is a genetic disorder that affects the heart's electrical activity. It is characterized by a prolonged QT interval on an electrocardiogram (ECG), which can lead to arrhythmias, syncope, and sudden cardiac arrest.

Pathophysiology[edit]

Long QT syndrome is caused by mutations in genes that encode for ion channels in the heart. These ion channels are responsible for the flow of potassium, sodium, and calcium ions, which are crucial for the heart's electrical activity. The most commonly affected genes include KCNQ1, KCNH2, and SCN5A. Mutations in these genes can disrupt the normal flow of ions, leading to a prolonged QT interval.

Types[edit]

There are several types of Long QT syndrome, classified based on the specific gene mutation:

  • LQT1: Caused by mutations in the KCNQ1 gene.
  • LQT2: Caused by mutations in the KCNH2 gene.
  • LQT3: Caused by mutations in the SCN5A gene.

Symptoms[edit]

Individuals with Long QT syndrome may experience:

Diagnosis[edit]

Diagnosis of Long QT syndrome typically involves:

Treatment[edit]

Treatment options for Long QT syndrome include:

Prognosis[edit]

With appropriate treatment and lifestyle modifications, individuals with Long QT syndrome can lead relatively normal lives. However, the risk of sudden cardiac arrest remains, particularly in untreated individuals.

See also[edit]



Cardiovascular disease A-Z

Most common cardiac diseases

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