congenital long QT syndrome

GPTKB entity

Statements (52)
Predicate Object
gptkbp:instanceOf gptkb:arrhythmia
genetic disorder
gptkbp:affects heart
gptkbp:alsoKnownAs gptkb:LQTS
gptkbp:canBeAsymptomatic true
gptkbp:canBeDiagnosedIn childhood
adulthood
gptkbp:canBeFamilial true
gptkbp:canBeSporadic true
gptkbp:cause gptkb:torsades_de_pointes
gptkb:ventricular_tachycardia
sudden cardiac death
syncope
prolonged QT interval
gptkbp:diagnosedBy electrocardiogram
gptkbp:firstDescribed 1957
gptkbp:geneticTestingAvailable true
https://www.w3.org/2000/01/rdf-schema#label congenital long QT syndrome
gptkbp:ICD-10_code I45.81
gptkbp:inheritance Mendelian
gptkbp:inheritsFrom autosomal dominant
autosomal recessive
gptkbp:Jervell_and_Lange-Nielsen_syndrome associated with deafness
gptkbp:MeSH_ID gptkb:D017363
gptkbp:mortalityRisk increased without treatment
gptkbp:mutationAssociatedWith gptkb:KCNQ1
gptkb:KCNH2
gptkb:SCN5A
gptkbp:OMIM 192500
gptkbp:prevalence 1 in 2,000 to 1 in 7,000 people
gptkbp:prognosis variable
gptkbp:relatedTo gptkb:acquired_long_QT_syndrome
gptkbp:riskFactor family history
certain medications
gptkbp:Romano-Ward_syndrome not associated with deafness
gptkbp:subspecies gptkb:Jervell_and_Lange-Nielsen_syndrome
gptkb:Romano-Ward_syndrome
gptkbp:symptom fainting
seizures
palpitations
sudden death
gptkbp:treatment gptkb:implantable_cardioverter-defibrillator
beta blockers
lifestyle modification
gptkbp:triggeredBy exercise
sleep
emotional stress
sudden loud noises
gptkbp:bfsParent gptkb:ondansetron
gptkb:pimozide
gptkb:vandetanib
gptkbp:bfsLayer 6