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Implantable cardioverter-defibrillators in patients with long QT syndrome: a multicentre study
Agnieszka Zienciuk-Krajka1, Maciej Sterliński, Artur Filipecki
1Dept. of Cardiology and Electrotherapy, Medical University of Gdańsk, Gdańsk, Poland. agzien@gumed.edu.pl.
Insights
Implantable cardioverter-defibrillator (ICD) therapy is effective for long QT syndrome (LQTS) patients, but complications are frequent. Careful programming may reduce some adverse events, but others persist.
Area of Science:
- Cardiology
- Electrophysiology
- Genetics
Background:
- Implantable cardioverter-defibrillator (ICD) therapy is established for preventing sudden cardiac death.
- Limited data exist on ICD outcomes in young, healthy long QT syndrome (LQTS) patients.
Purpose of the Study:
- To evaluate ICD therapy outcomes in LQTS patients.
- To identify risk factors for appropriate and inappropriate ICD discharges.
- To assess ICD-related complications in this population.
Main Methods:
- Retrospective analysis of 67 LQTS patients from 14 centers.
- Data collection included demographics, clinical status, and ICD therapy details.
- Median follow-up was 48 months.
Main Results:
- 58% of patients received ICD therapy during follow-up.
- Risk factors for appropriate therapy included syncope, prior pacemaker use, single-chamber ICD, and noncompliance.
- 57% of patients experienced at least one complication; no predictors for inappropriate shocks were found.
Conclusions:
- ICD therapy is effective in nearly half of LQTS patients.
- High rates of early and late complications necessitate careful patient selection and management.
- Modern programming may reduce some adverse events, but not all.
Background:
Implantable cardioverter-defibrillator (ICD) therapy has been proven effective in the prevention of sudden cardiac death, but data on outcomes of ICD therapy in the young and otherwise healthy patients with long QT syndrome (LQTS) are limited.
Aim:
We sought to collect data on appropriate and inappropriate ICD discharges, risk factors, and ICD-related complications.
Methods:
All LQTS patients implanted with an ICD in 14 centres were investigated. Demographic, clinical, and ICD therapy data were collected.
Results:
The study included 67 patients (88% female). Median age at ICD implantation was 31 years (12-77 years). ICD indication was based on resuscitated cardiac arrest in 46 patients, syncope in 18 patients, and malignant family history in three patients. During a median follow-up of 48 months, 39 (58%) patients received one or more ICD therapies. Time to first appropriate discharge was up to 55 months. Inappropriate therapies were triggered by fast sinus rhythm, atrial fibrillation, and T-wave oversensing. No predictors of inappropriate shocks were identified. Risk factors for appropriate ICD therapy were: (1) recurrent syncope despite b-blocker treatment before ICD implantation, (2) pacemaker therapy before ICD implantation, (3) single-chamber ICD, and (4) noncompliance to b-blockers. In 38 (57%) patients, at least one complication occurred.
Conclusions:
ICD therapy is effective in nearly half the patient population; however, the rates of early and late complica-tions are high. Although the number of unnecessary ICD shocks and reimplantation procedures may be lowered by modern programming and increased longevity of newer ICD generators, other adverse events are less likely to be reduced.
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