Background

Use of oral contraceptives (OCs) may modulate the clinical course of women with congenital long QT syndrome (LQTS). The safety of OC use by sex hormone content has not been assessed in women with LQTS.

Objective

We aimed to evaluate the association of OCs with the risk of cardiac events (CEs) in women with LQTS.

Methods

Beginning in 2010, information on menarche onset, OC use, pregnancy, and menopause were obtained from women enrolled in the Rochester LQTS Registry. Type of OC was categorized as progestin-only, estrogen-only, or combined (estrogen/progestin). Andersen-Gill multivariate modeling was used to evaluate the association of time-dependent OC use with the burden of CE (total number of syncope, aborted cardiac arrest, and LQTS-related sudden cardiac death) from menarche onset through 40 years. Findings were adjusted for genotype, corrected QT duration, and time-dependent β-blocker therapy.

Results

A total of 1659 women with LQTS followed through March 2021, of whom 370 (22%) were treated with an OC. During a cumulative follow-up of 35,797 years, there were a total of 2027 CE. Multivariate analysis showed that progestin-only OC was associated with a pronounced 2.8-fold (P = .01) increased risk of CEs in women who did not receive β-blocker therapy, while β-blockers were highly protective during progestin-only OC treatment (hazard ratio 0.22; P = .01; P = .006 for β-blocker-by-OC interaction). The risk associated with OC use without concomitant β-blocker treatment was pronounced in women with LQTS type 2.

Conclusion

Our findings suggest that progestin-only OC should not be administered in women with LQTS without concomitant β-blocker therapy. OCs should be used with caution in women with LQTS type 2.

中文翻译：

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先天性长 QT 综合征女性使用口服避孕药

背景

口服避孕药 (OCs) 的使用可能会调节先天性长 QT 综合征 (LQTS) 女性的临床病程。尚未在 LQTS 女性中评估使用性激素含量的 OC 的安全性。

客观的

我们旨在评估 OCs 与 LQTS 女性心脏事件 (CEs) 风险的关系。

方法

从 2010 年开始，从罗彻斯特 LQTS 登记处登记的女性那里获得有关初潮开始、OC 使用、怀孕和更年期的信息。OC 的类型分为纯孕激素、纯雌激素或组合（雌激素/孕激素）。Andersen-Gill 多变量模型用于评估从初潮开始到 40 年的时间依赖性 OC 使用与 CE 负担（晕厥、流产心脏骤停和 LQTS 相关心源性猝死的总数）之间的关联。结果根据基因型、校正的 QT 持续时间和时间依赖性 β 受体阻滞剂治疗进行了调整。

结果

到 2021 年 3 月，共有 1659 名患有 LQTS 的女性，其中 370 名（22%）接受了 OC 治疗。在 35,797 年的累积随访中，共有 2027 个 CE。多变量分析表明，仅使用孕激素的 OC 与未接受 β 受体阻滞剂治疗的女性的 CE 风险显着增加2.8 倍（ P = .01）相关，而 β 受体阻滞剂在仅使用孕激素的 OC 治疗期间具有高度保护性（对于 β-受体阻滞剂与 OC 的相互作用，风险比为 0.22；P = .01；P = .006）。在 LQTS 2 型女性中，与 OC 使用不伴随 β 受体阻滞剂治疗相关的风险更为明显。

结论

我们的研究结果表明，在没有伴随 β 受体阻滞剂治疗的情况下，不应在患有 LQTS 的女性中使用仅含孕激素的 OC。LQTS 2 型女性应谨慎使用 OC。