Arrhythmias & electrophysiologyCohort study

Association Between Syncope Trigger Type and Risk of Subsequent Life-Threatening Events in Patients With Long QT Syndrome

JAMA CardiolPublished

  • Retrospective cohort study
  • multicentre
  • 5 international long QT syndrome (LQTS) registries

Authors' conclusion

In this study, trigger-specific syncope in LQTS patients was associated with differential risk of subsequent LTE and response to β-blocker therapy.

Study context

Population
Patients with genetically confirmed LQT1, LQT2 or LQT3 from a single LQTS-causative variant, enrolled July 1979 to July 2021
Participants
2,938 patients
Exposure
Adrenergic- vs nonadrenergic-triggered syncope
Outcome
First occurrence of a life-threatening event (LTE)

Main finding

As reported in the abstract

First LTE, by genotype
Hazard ratio (95% CI)P value
ADLQT1
7.61(4.18–14.20)
P < 0.001
non-ADLQT1
1.50(0.21–4.77)
P = 0.97
ADLQT2
3.07(1.66–5.67)
P ≤ 0.001
non-ADLQT2
3.45(1.96–6.06)
P ≤ 0.001
LQT3: LTE preceded by syncope
7 (12%)

Observational data: associations, not effects.

Further findings

First syncope and syncope preceding LTE, by genotype

LQT1: first syncope
365 (27%)
LQT1: syncope with adrenergic (AD) triggers
243 [67%]
LQT1: syncope preceded life-threatening events (LTEs)
43 (68%)
LQT2: first syncope
283 (26%)
LQT2: AD / non-AD triggers
106 (37%) / 177 (63%)
LQT2: syncope preceded LTEs
55 (56%)
LQT3 (n = 501): LTE preceded by syncope
7 (12%)

β-blockers after syncope (LQT1 and LQT2)

β-blockers associated with significant reduction in subsequent LTE risk
Breakthrough events significantly higher with selective vs nonselective agents

Original publication

Association Between Syncope Trigger Type and Risk of Subsequent Life-Threatening Events in Patients With Long QT Syndrome. JAMA Cardiol. Published 1 August 2023. doi:10.1001/jamacardio.2023.1951

Summarised from the published abstract; every number is checked against it