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Determining the Likelihood of Variant Pathogenicity Using Amino Acid-level Signal-to-Noise Analysis of Genetic Variation
Published on: January 16, 2019
Long-QT syndrome after age 40
Ilan Goldenberg1, Arthur J Moss, James Bradley
1Cardiology Division of the Department of Medicine, University of Rochester Medical Center, Rochester, NY 14642, USA. Ilan.Goldenberg@heart.rochester.edu
Circulation
|April 23, 2008
Summary
Congenital long-QT syndrome (LQTS) patients over 40 still face significant cardiac event risks. Factors like gender, recent syncope, and LQT3 genotype influence outcomes in this older population.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Previous research on congenital long-QT syndrome (LQTS) primarily focused on younger individuals.
- The clinical trajectory of LQTS in older populations remains understudied.
Purpose of the Study:
- To investigate the risk of life-threatening cardiac events in patients with LQTS aged 41 to 75.
- To identify factors influencing cardiac event risk in older LQTS patients.
Main Methods:
- Analysis of 2759 subjects from the International LQTS Registry, categorized by corrected QT interval (QTc).
- Assessment of risk for aborted cardiac arrest or death across different age ranges (41-60 and 61-75).
- Evaluation of gender differences, clinical history (syncope), and genetic mutations (LQT3 genotype) as risk factors.
Main Results:
- LQTS patients aged 41-60 had a significantly higher risk (HR 2.65) of cardiac events compared to unaffected individuals.
- The risk diminished in the 61-75 age group (HR 1.23), but remained elevated.
- Affected women had higher event rates (26%) than borderline (16%) and unaffected (12%) women; men's rates were similar across groups.
- Recent syncope (HR 9.92) and LQT3 genotype (HR 4.76) were significant predictors of adverse events.
Conclusions:
- Congenital long-QT syndrome patients aged over 40 years remain at a substantial risk for life-threatening cardiac events.
- The manifestation of LQTS in older adults is shaped by age-related factors, including gender, clinical history, and specific genetic mutations.
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