Arrhythmias & electrophysiology
Clinical Features, Long-Term Prognosis, and Clinical Management of Genotype-Negative Long QT Syndrome Patients
Authors' conclusion
Arrhythmic risk is similar between GEN+ and GEN- LQTS patients
Study context
- Population
- Patients clinically diagnosed with long QT syndrome, genetically screened in Japan and ItalyJapan 347, Italy 485 · genetically screened in Japan 347 · Italy 485
- Participants
- 832 participants
- Exposure
- Genotype-negative vs genotype-positive long QT syndrome (LQTS)
- GEN+n = 698reference
- GEN-n = 134
- Outcome
- Clinical outcome (cardiac events) during follow-up
- Follow-up
- Average 6 and 7 years
Methodological detailsJapan and Italy cohorts
- Japan and Italy cohorts
Main finding
As reported in the abstract
Clinical outcome · average follow-up 6 (Japan) and 7 (Italy) years
- GEN+ and GEN- showed a comparable clinical outcome in both cohorts
Observational data: associations, not effects.
Further findings
Japanese vs Italian patients at diagnosis and follow-up
Probands
Symptomatic
β-blockers
FH of LQTS
Cardiac events during follow-up
- Japanese vs Italian patients at diagnosis and follow-up: P < 0.001 for all comparisons
Japanese cohort, GEN- vs GEN+
- GEN- had more cardiac arrests, used less β-blockers and had much less family history (FH) of LQTS
Italian cohort, GEN- vs GEN+
- More homogeneous; only more LQTS FH among GEN+
QTc shortening during follow-up
- Similar between Japanese and Italian patients, irrespective of GEN+ or GEN- (close to 30 ms in all groups)
Original publication
Clinical Features, Long-Term Prognosis, and Clinical Management of Genotype-Negative Long QT Syndrome Patients. JACC Clin Electrophysiol. Published 9 October 2024. doi:10.
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