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).
Cohort (n=2,908)
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).
Hazard Ratio: 1.72
OBJECTIVES We aimed to determine whether long QT syndrome (LQTS) genotype has a differential effect on clinical course of disease in male and female children and adults after adjustment for QTc duration. BACKGROUND Genotype influences clinical course of the LQTS; however, data on the effect of age and gender on this association are limited. METHODS The LQTS genotype, QTc duration, and follow-up were determined in 243 cases of LQTS caused by the KCNQ1 potassium channel gene mutations (LQT1), 209 cases of LQTS caused by the HERG potassium channel gene mutations (LQT2), and 81 cases of LQTS caused by the SCN5A sodium channel gene mutation (LQT3) gene carriers. The probability of cardiac events (syncope, aborted cardiac arrest, or sudden death) was analyzed by genotype, gender, and age (children < or = 15 years and adults 16 to 40 years). In addition, the risk of sudden death and lethality of cardiac events were evaluated in 1,075 LQT1, 976 LQT2, and 324 LQT3 family members from families with known genotype. RESULTS During childhood, the risk of cardiac events was significantly higher in LQT1 males than in LQT1 females (hazard ratio HR = 1.72), whereas there was no significant gender-related difference in the risk of cardiac events among LQT2 and LQT3 carriers. During adulthood, LQT2 females (HR = 3.71) and LQT1 females (HR = 3.35) had a significantly higher risk of cardiac events than respective males. The lethality of cardiac events was highest in LQT3 males and females (19% and 18%), and higher in LQT1 and LQT2 males (5% and 6%) than in LQT1 and LQT2 females (2% for both). CONCLUSIONS; Age and gender have different, genotype-specific modulating effects on the probability of cardiac events and electrocardiographic presentation in LQT1 and LQT2 patients.
Zaręba et al. (Tue,) 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).
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