ArticleKorean circulation journal2022
The Outcome of Long QT Syndrome, a Korean Single Center Study.
Article in Korean circulation journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 4 citations in OpenAlex.
- Case Report: Nadolol-mexiletine combination therapy for refractory neonatal long QT syndrome: a case series beyond sodium channelopathies.Frontiers in pediatrics · 2026Article
- Management of Long QT Syndrome: A Systematic Review.Cureus · 2024Review
- Clinical and Genetic Features of Korean Inherited Arrhythmia Probands.Korean circulation journal · 2023Article
- Multicenter Cohort Analysis Unveil Inherited Arrhythmia in Korea.Korean circulation journal · 2023Article
- The Outcome of Long QT Syndrome: What is the Optimal Therapy?Korean circulation journal · 2022Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesAlthough long QT syndrome (LQTS) is a potentially life-threatening inherited cardiac channelopathy, studies documenting the long-term clinical data of Korean patients with LQTS are scarce.
methodsThis retrospective cohort study included 105 patients with LQTS (48 women; 45.7%) from a single tertiary center. The clinical outcomes were analyzed for the rate of freedom from breakthrough cardiac events (BCEs), additional treatment needed, and death.
resultsLQTS was diagnosed at a median age of 11 (range, 0.003-80) years. Genetic testing was performed on 90 patients (yield, 71.1%). The proportions of genetically confirmed patients with LQTS types 1, 2, 3, and others were 34.4%, 12.2%, 12.2%, and 12.2%, respectively. In the symptomatic group (n=70), aborted cardiac arrest was observed in 30% of the patients. Treatments included medications in 60 patients (85.7%), implantable cardioverter-defibrillators in 27 (38.6%; median age, 17 years; range, 2-79 years), and left cardiac sympathetic denervation surgery in 7 (10%; median age, 13 years; range, 2-34). The 10-year BCE-free survival rate was 73.2%. By genotype, significant differences were observed in BCEs despite medication (p<0.001). The 10-year BCE-free survival rate was the highest in patients with LQTS type 1 (81.8%) and the lowest in those with multiple LQTS-associated mutations (LQTM). All patients with LQTS survived, except for one patient who had LQTM.
conclusionsGood long-term outcomes can be achieved by using recently developed genetically tailored management strategies for patients with LQTS.
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