Long QT syndrome

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Long QT syndrome type 1

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 | edit source]

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 | edit source]

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 | edit source]

Individuals with Long QT syndrome may experience:

Diagnosis[edit | edit source]

Diagnosis of Long QT syndrome typically involves:

Treatment[edit | edit source]

Treatment options for Long QT syndrome include:

Prognosis[edit | edit source]

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.

Related Pages[edit | edit source]

Categories[edit | edit source]


Cardiovascular disease A-Z

Most common cardiac diseases

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Contributors: Prab R. Tumpati, MD