BC65.0

ICD-11 MMS 2026-01

Long QT syndrome

Description

A congenital disorder of ventricular myocardial repolarization characterised by a prolonged QT interval on the electrocardiogram (ECG) that can lead to symptomatic ventricular arrhythmias and an increased risk of sudden cardiac death.

Index terms

  • Long QT syndrome
  • Congenital long QT syndrome
  • Familial long QT syndrome
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  • Long QT syndrome type 1
  • Long QT syndrome type 2
  • Long QT syndrome type 3
  • Long QT syndrome type 4
  • Long QT syndrome type 5
  • Long QT syndrome type 6
  • Long QT syndrome type 7
  • Andersen-Tawil syndrome
  • Cardiodysrythmic potassium-sensitive periodic paralysis
  • Potassium channel disorders
  • Long QT syndrome type 8
  • Timothy syndrome
  • Long QT syndrome, type 9
  • Long QT syndrome, type 10
  • Long QT syndrome, type 11
  • Long QT syndrome, type 12
  • Long QT syndrome, type 13
  • Long QT syndrome with hearing impairment
  • Jervell and Lange-Nielsen syndrome

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • BC65.1 – Brugada syndrome
  • BC65.2 – Short QT syndrome
  • BC65.3 – Early repolarisation syndrome
  • BC65.4 – Idiopathic ventricular fibrillation
  • BC65.5 – Catecholaminergic polymorphic ventricular tachycardia
  • BC65.Y – Other specified cardiac arrhythmia associated with genetic disorder
  • BC65.Z – Cardiac arrhythmia associated with genetic disorder, unspecified

Foundation entity

Long QT syndrome – http://id.who.int/icd/entity/1208831985

Part of: BC65: Cardiac arrhythmia associated with genetic disorder › 11: Diseases of the circulatory system