Key result
Risk algorithms predict 5-year life-threatening arrhythmias in LQTS patients with a ~0.71 C-statistic.
Why the study?
Risk stratification in long QT syndrome patients is important for optimizing care and clinical decision making.
Population
1,509 phenotypic, 1,288 genotypic, and 1,481 external validation LQTS patients
Design
Registry-based cohort risk prediction model development and external validation study
Follow-up
Median 9.0 years in phenotypic and 9.8 years in genotypic cohorts
Authors
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May support ICD decisions in LQTS; hypothesis-generating pending prospective outcome trials.
Cohort (n=4,278)
Yes
Effect estimate: C-statistic 0.784 (phenotypic) and 0.785 (genotypic) (95% CI 0.740-0.827 and 0.721-0.849)
The developed risk prediction algorithms provide validated estimates of 5-year absolute risk for life-threatening arrhythmic events in LQTS patients, which can assist in clinical decision making regarding prophylactic ICD therapy.
Wang et al. (2022) conducted a cohort in Long QT syndrome (LQTS) (n=4,278). Risk prediction algorithms (phenotypic and genotypic models) was evaluated on First life-threatening arrhythmic event (aborted cardiac arrest, sudden cardiac death, or appropriate ICD shock) (C-statistic 0.784 (phenotypic) and 0.785 (genotypic), 95% CI 0.740-0.827 and 0.721-0.849). Risk prediction algorithms for phenotype- and genotype-positive LQTS patients demonstrated good discrimination for 5-year life-threatening arrhythmic events, with external validation C-statistics of 0.700 and 0.711.
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