Arrhythmias & electrophysiology

Frequency of and outcomes associated with nonadherence to guideline-based recommendations for an implantable cardioverter-defibrillator in patients with congenital long QT syndrome

Heart RhythmPublished Retrospective cohort study

Authors' conclusion

With >1200 years of combined follow-up, the experience and evidence from our 2 LQTS specialty centers suggest that many patients who satisfy a recommendation for an ICD based on the latest 2022 European Society of Cardiology guidelines may not need one.

Study context

Population
Patients with LQT1, LQT2 or LQT3 meeting a class I or II 2022 ESC guideline-based implantable cardioverter-defibrillator (ICD) recommendation290 met an ICD recommendation
Participants
2,861 patients
Exposure
No ICD vs ICD
  • ICDn = 134
  • No ICDn = 156reference
Outcome
Frequency of guideline-based ICD recommendations and outcomes (BCEs, complications) with vs without ICD
Methodological details2 long QT syndrome (LQTS) specialty centres, >1200 years of combined follow-up
  • 2 LQTS specialty centres
  • >1200 years of combined follow-up

Main finding

As reported in the abstract

GroupValue≥1 appropriate VF-terminating ICD therapyNonlethal breakthrough cardiac events (BCEs)
ICD41%55 of 134 (41%)
No ICD4%6 of 156 (4%)

Value

ICD41%
No ICD4%

≥1 appropriate VF-terminating ICD therapy

ICD55 of 134 (41%)
No ICDNot reported in the abstract

Nonlethal breakthrough cardiac events (BCEs)

ICDNot reported in the abstract
No ICD6 of 156 (4%)
  • No ICD vs ICD: P < 0.001 — BCEs significantly lower without ICD
Met a guideline-based ICD recommendation
290 (≈10%)
Class I/level B indication
53 (18%)
Class I/level C indication
56 (19%)
Class IIa/level C indication
19 (7%)
Class IIb/level B indication
162 (56%)
Did not receive an ICD
156/290 [54%]

Observational data: associations, not effects.

Safety

ICD-related complications
10% · 13 of 134

Original publication

Frequency of and outcomes associated with nonadherence to guideline-based recommendations for an implantable cardioverter-defibrillator in patients with congenital long QT syndrome. Heart Rhythm. Published 2 October 2024. doi:10.1016/j.hrthm.2024.09.063

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

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