Presentation with sudden cardiac death due to Long QT syndrome decreased by 22% per year from 2014 (p=0.026), with no deaths from 2019 to 2023 in a New Zealand national registry.
Observational (n=915)
Yes
A national LQTS registry and molecular autopsy program in New Zealand was associated with a significant decrease in presentations with resuscitated cardiac arrest and sudden cardiac death over a 24-year period.
p-value: p=0.026
BACKGROUND Population-based data on Long QT syndrome (LQTS) is lacking. New Zealand has a national LQTS registry, molecular autopsy program and standardised investigation of resuscitated cardiac arrest (RSCA). OBJECTIVE To describe trends in LQTS detection and presentation with RSCA/sudden cardiac death (SCD) over 24 years. METHODS Review of data on registry participants who underwent genetic testing for LQTS presenting between 2000 and 2023. RESULTS Among 915 diagnosed with LQTS, 378 probands, (mean QTc 512 +/- 52 ms presented with SCD (26/378, 7%), RSCA (82, 21%), syncope or seizure (122, 32%), incidental/other (148, 40%). Causative genetic variants were found in 165/378 probands (44%): 98 KCNQ1 (59 %), 41 KCNH2 (25%), 7 SCN5A (4%), and 19 other genes (12%). 593/915 participants (65%) were on therapy (beta blocker (565, 62%), implantable cardioverter defibrillator (134, 16%) and/or left sympathetic cardiac denervation (69, 8%)). Three likely arrhythmic deaths (0.3%), and 23 appropriate ICD shocks (3%) occurred during follow up (median 12 years). Detections of LQTS/population was 0.5/100,000 in 2000, 1.6/100,000 in 2009 and 0.4/100,000 in 2023. Presentation with RSCA decreased by 10%/year from 2007. Presentation with death decreased by 22%/year from 2014 (p=0.026), with no deaths from 2019 to 2023. CONCLUSIONS Decreasing rates of detection of LQTS in recent years suggests that most cases have been discovered in New Zealand. Decreasing cardiac arrest and sudden death due to LQTS is likely related to the success of the overall program.
Tarca et al. (Sun,) conducted a observational in Long QT syndrome (LQTS) (n=915). National LQTS registry and molecular autopsy program was evaluated on Trends in LQTS detection and presentation with RSCA/sudden cardiac death (SCD) over 24 years (p=0.026). Presentation with sudden cardiac death due to Long QT syndrome decreased by 22% per year from 2014 (p=0.026), with no deaths from 2019 to 2023 in a New Zealand national registry.
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