Arrhythmias & electrophysiology

Characteristics, clinical course, and cardiac events of patients with previously asymptomatic long QT syndrome

Heart RhythmPublished retrospective analysis

Authors' conclusion

Patients with asymptomatic LQTS have an extremely low chance of having an LQTS-triggered cardiac event (<1%). Necessary treatment should account for sex, subtype, corrected QT interval, age, and quality of life and should adapt as situational risk factors arise.

Study context

Population
Patients with type 1, 2, or 3 long QT syndrome (LQTS) who were asymptomatic at the first evaluationasymptomatic at first evaluation, of 1547 patients with type 1, 2, or 3 LQTS
Participants
1,139 patients
Outcome
BCE
Follow-up
Average of 8 ± 7.5 years
Methodological detailssingle specialty center
  • Single specialty center

Main finding

As reported in the abstract

Patients who experienced a breakthrough cardiac event (BCE)
13 patients (1%)
Of those with a BCE, nonadherent to treatment at the time of an event
5 (38%)
Of the 8 patients with a true treatment-refractory BCE, female
7 (88%)
Of the 8 patients with a true treatment-refractory BCE, mean age at BCE
15 ± 6 years
Of the 8 treatment-refractory BCEs, syncope/seizure
4 (50%)
Of the 8 treatment-refractory BCEs, implantable cardioverter-defibrillator shock
3 (37.5%)
Of the 8 treatment-refractory BCEs, sudden cardiac arrest
1 (12.5%)

Observational data: associations, not effects.

Further findings

Characteristics of asymptomatic patients at first evaluation

Asymptomatic LQTS at first evaluation
1139 of 1547 patients (74%)
Women
n = 636 women [56%]
Mean age
31 ± 16 years
Mean corrected QT interval
462 ± 34
LQT1
55%
LQT2
33%
LQT3
12%

Treatment

Started on beta-blockers
755 patients (66%)
On sodium channel blockers
27 (2%)
Underwent left cardiac sympathetic denervation
11 (1%)
Received combination therapy
71 (6%)
Managed with an intentional nontherapy
275 qualified patients (24%)

Original publication

Characteristics, clinical course, and cardiac events of patients with previously asymptomatic long QT syndrome. Heart Rhythm. Published 30 June 2025. doi:10.1016/j.hrthm.2025.06.045

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