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Brugada Syndrome and Pregnancy: Highlights on Antenatal and Prenatal Management

机译:Brugada综合征和妊娠:产前和产前管理重点

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Introduction. Brugada syndrome is characterized by a disruption of heart’s normal rhythm. It is an autosomal dominant disease due to a mutation of SNC5A gene. Its prevalence is low all over the world, but it is a lethal disease. Sudden cardiac death is the result of phenotypic manifestation of Brugada syndrome. Among asymptomatic Brugada patients, arrhythmia could be provoked by physical activity, fever, or pregnancy. About obstetrical management, very few data or reports have been published since this syndrome has been diagnosed in late 1992.Case Presentation. A 20-year-old pregnant woman at 13 weeks of gestation was referred to our department because of her familial history of sudden cardiac deaths. Brothers and sisters of her mother died of Brugada syndrome in childhood or older and live components of this family were carrier of mutation in Brugada gene. The pregnancy was uneventful. The patient gave birth vaginally without any arrhythmia. Strictly cardiological monitoring was performed during labour, delivery, and 12 hours of the postpartum.Conclusion. Even though patient at low risk may never have arrhythmia, some conditions could represent a Brugada trigger. The management could be very easy and uneventful. Otherwise it could be very difficult with need of ECMO or antiarrhythmics drugs or intracardiac device. Obstetrical management of Brugada pregnant women should be very strict and multidisciplinary in cooperation with cardiologist and anaesthesiologist and should provide an informed consent to the couple.
机译:介绍。 Brugada综合征的特征是心脏的正常节律紊乱。由于SNC5A基因的突变,它是一种常染色体显性遗传疾病。它的流行率在全世界都很低,但这是一种致命的疾病。猝死是Brugada综合征表型表现的结果。在无症状的Brugada患者中,体育活动,发烧或怀孕可能引起心律不齐。关于产科管理,自1992年末诊断出该综合征以来,几乎没有数据或报告发表过。一名怀孕13周的20岁孕妇因其家族性心脏性猝死病史而被转诊至我们部门。她母亲的兄弟姐妹在童年或更高年龄时死于Brugada综合征,该家庭的生活组成部分是Brugada基因突变的携带者。怀孕顺利。病人经阴道分娩没有任何心律不齐。在分娩,分娩和产后12小时进行严格的心脏监测。即使处于低风险的患者可能永远不会出现心律不齐,但某些情况可能代表了Brugada触发因素。管理可能非常容易且平稳。否则,可能需要ECMO或抗心律不齐药物或使用心内装置非常困难。 Brugada孕妇的产科管理应与心脏病专家和麻醉专家合作非常严格和多学科的,并应向这对夫妇提供知情同意。

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