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Congenital Long QT Syndromes

Inherited arrhythmia syndromes caused by genetic ion channel defects with QT prolongation and sudden death risk.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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What is Congenital Long QT Syndromes?

Congenital long QT syndromes (LQTS) are inherited disorders caused by mutations in genes encoding cardiac ion channels, characterized by prolongation of the QT interval on the ECG and susceptibility to life-threatening ventricular arrhythmias (torsades de pointes).

Three subtypes predominate: LQT1 (KCNQ1 gene, triggered by exercise), LQT2 (KCNH2 gene, triggered by emotional stress and sudden noise) and LQT3 (SCN5A gene, occurring during sleep). Autosomal dominant (Romano-Ward) and recessive (Jervell-Lange-Nielsen) forms exist.

Diagnosis uses corrected QT interval (QTc), the Schwartz score and genetic testing. QTc >470 ms (male) or >480 ms (female) is considered prolonged.

Beta-blockers are first-line therapy; high-risk patients may need ICD implantation or left cardiac sympathetic denervation (LCSD).

Symptoms

Syncope, especially triggered by exercise, emotion or sudden noise
Palpitation episodes
Seizure-like episodes due to cerebral hypoperfusion from arrhythmia
Sudden cardiac arrest (may be first presentation)
Family history of unexplained sudden death
Most carriers are asymptomatic

Risk Factors

Family history of long QT syndrome or early sudden death
Use of QT-prolonging drugs (macrolide antibiotics, antiarrhythmics)
Electrolyte imbalance (hypokalemia, hypomagnesemia)
Female sex (higher risk in LQT2)
Events during exercise (LQT1) or sleep (LQT3)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Exercise- or emotion-triggered syncope, especially in children and young adults
  • Unexplained early sudden death in the family
  • QT prolongation detected on ECG

Treatment Methods

01
Beta-blocker therapy (preferably nadolol or propranolol)
02
Avoidance of QT-prolonging drugs (see www.crediblemeds.org)
03
ICD implantation in high-risk patients
04
Left cardiac sympathetic denervation (LCSD)
05
Subtype-specific lifestyle advice (e.g., swimming restriction in LQT1)
06
Family screening and genetic counseling

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.