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Lang QT-tid-syndrom

Sist revidert:
Sist revidert av:
Definisjon:
Lang QT-tid syndrom (LQTS) defineres ved QT> 0,45s, sykehistorie med synkope og familiehistorie med LQTS. Arvelig ionekanaldefekt, men forekommer også i ervervet form.
Forekomst:
Forekomst anslått til 1 av 3000-5000 personer.
Symptomer:
Uforklarlig svimmelhet, synkoper eller plutselig død hos unge, tidligere friske personer.
Funn:
EKG-forandringer med korrigert QT > 0,45 sekund
Diagnostikk:
Elektrofysiologisk testing. Gentesting etter påvist forlenget QT-segment
Behandling:
Alle anbefales behandling med betablokker. Tilleggsbehandling med permanent pacemaker og defibrillator aktuelt. Ved ervervet LQTS unngå utløsende medikament eller ev. elektrolyttforstyrrelse.
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  2. Schwartz PJ, Ackerman MJ. Congenital long QT syndrome: Epidemiology and clinical manifestations. UpToDate, last updated May 23, 2019. UpToDate  
  3. Berul CI. Acquired long QT syndrome: Clinical manifestations, diagnosis, and management. UpToDate, last updated Feb 04, 2021. UpToDate  
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  8. Schwartz PJ, Ackerman MJ, Antzelevitch C, et al. Inherited cardiac arrhythmias. Nat Rev Dis Primers. 2020;6(1):58. Published 2020 Jul 16. PMID: 32678103 PubMed  
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  19. Hamang A, Solberg B, Bjorvatn C, Greve G, Øyen N. Genetic counseling in congenital long QT syndrome. Tidsskr Nor Legeforen 2009; 129: 1226-9. Tidsskrift for Den norske legeforening  
  20. Schwartz PJ, Crotti L, Insolia R. Long-QT syndrome: from genetics to management published correction appears in Circ Arrhythm Electrophysiol. 2012 Dec;5(6):e119-20. Circ Arrhythm Electrophysiol. 2012;5(4):868-877. PMID: 22895603 PubMed  
  21. Tisdale JE. Drug-induced QT interval prolongation and torsades de pointes: Role of the pharmacist in risk assessment, prevention and management. Can Pharm J (Ott). 2016;149(3):139-152. PMID: 27212965 PubMed  
  22. Tester DJ, Ackerman MJ. Sudden infant death syndrome: how significant are the cardiac channelopathies? Cardiovasc Res 2005; 67: 388-96. PubMed  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

  • Gottfried Greve, professor og seksjonsoverlege, Barneklinikken / Hjerteavdelingen, Haukeland Universitetssykehus og Universitetet i Bergen