Arrhythmias & electrophysiologyCohort study

Long QT syndrome: importance of reassessing arrhythmic risk after treatment initiation

Eur Heart JPublished

  • Cohort study

Authors' conclusion

Beta-blockers often shorten QTc, thus changing risk scores and ICD indications for primary prevention. Yearly risk reassessment with therapy optimization leads to fewer ICD implants (3%) without increasing life-threatening events.

Study context

Population
LQT1/2/3 and genotype-negative long QT syndrome (LQTS) patients without aborted cardiac arrest (ACA) before diagnosis or cardiac events (CEs) below age 1
Participants
946 participants
Outcome
Appropriateness of adherence to M-FACT risk score suggestions (M-FACT ≥2, static or dynamic)

Main finding

As reported in the abstract

Persistent dynamic M-FACT ≥2 after further therapeutic optimization: patients with CEs (no ACA)
7 (14%) of 49
Remaining static M-FACT ≥2 patients on βB with CEs
0 of 57
New dynamic M-FACT ≥2 after therapeutic optimization: patients with CEs
3 (9%) of 32
Implantable cardioverter defibrillator (ICD) implanted among patients indicated by M-FACT ≥2
22/142 (15%)
Patients with ICD shocks
3

Observational data: associations, not effects.

Further findings

QTc ≥500 ms before vs on βB

QTc ≥500 ms reduced with βB (94% treated)
18% to 12% (P < .001)

Overall outcomes · 7 ± 6 years of follow-up

Deaths
0
CEs
4%
ACA
0.4%

Original publication

Long QT syndrome: importance of reassessing arrhythmic risk after treatment initiation. Eur Heart J. Published 16 May 2024. doi:10.1093/eurheartj/ehae289

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