Modulating effects of age and gender on the clinical course of long QT syndrome by genotype
Key result
Childhood LQT1 linked to ~72% higher cardiac risk in males, shifting to females in adulthood.
- HR 1.72
- n=2,908
Why the study?
Genotype influences the clinical course of LQTS, but data on the modulating effects of age and gender on this association are limited.
Comparison
Clinical course and risk of cardiac events stratified by genotype, gender, and age
Design
Cohort study
Authors
Discussion
Loading...
Member takes
Implication
The risk of cardiac events in long QT syndrome is heavily modulated by the interaction between specific genotype, age, and gender, with males at higher risk in childhood (LQT1) and females at higher risk in adulthood (LQT1 and LQT2).
Key Points
- This research investigates how the genotype of long QT syndrome (LQTS) affects the clinical progression of the disease, particularly in relation to age and gender.
- Analyzed 243 cases with KCNQ1 mutations, 209 with HERG mutations, and 81 with SCN5A mutations.
- Evaluated cardiac event probabilities based on genotype, age, and gender in children and adults.
- Assessed the risk of sudden death and event lethality in 1,075 LQT1, 976 LQT2, and 324 LQT3 family members.
- Risk of cardiac events was significantly higher in childhood LQT1 males compared to females (HR = 1.72).
- LQT2 females (HR = 3.71) and LQT1 females (HR = 3.35) had a higher risk of cardiac events than males in adulthood.
- Lethality rates for cardiac events were highest in LQT3 males (19%) and females (18%), with lower rates in LQT1 and LQT2 males (5% and 6%) compared to females (2%).
Study Design
Type
Cohort (n=2,908)
Structured PICO
P
Population
2,908 patients and family members with known LQT1, LQT2, or LQT3 genotypes evaluated for the probability of cardiac events across childhood and adulthood.
O
Outcome
Probability of cardiac events (composite of syncope, aborted cardiac arrest, or sudden death)composite
Main Result
Hazard Ratio: 1.72
The risk of cardiac events in long QT syndrome is heavily modulated by the interaction between specific genotype, age, and gender, with males at higher risk in childhood (LQT1) and females at higher risk in adulthood (LQT1 and LQT2).
Cite This Study
Zaręba et al. (2003) conducted a cohort in Long QT syndrome (n=2,908). LQTS genotype, age, and gender vs. Different genders and age groups within genotypes was evaluated on Cardiac events (syncope, aborted cardiac arrest, or sudden death) (HR 1.72). During childhood, LQT1 males had a higher risk of cardiac events than females (HR 1.72), whereas in adulthood, LQT1 and LQT2 females had higher risks than males (HR 3.35 and 3.71, respectively).