Giambanco Laura, Incandela Domenico, Maiorana Antonio, Alio Walter, Alio Luigi
Obstetric and Gynecology Department, Civico Hospital, Piazza N. Leotta 4, 90100 Palermo, Italy.
Case Rep Obstet Gynecol. 2014;2014:531648. doi: 10.1155/2014/531648. Epub 2014 May 22.
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.
引言。 Brugada综合征的特征是心脏正常节律紊乱。它是一种由于SNC5A基因突变导致的常染色体显性疾病。其在全球的患病率较低,但却是一种致命疾病。心脏性猝死是Brugada综合征表型表现的结果。在无症状的Brugada患者中,心律失常可能由体力活动、发热或妊娠诱发。关于产科管理,自1992年末该综合征被诊断以来,很少有数据或报告发表。病例报告。一名妊娠13周的20岁孕妇因家族性心脏性猝死病史被转诊至我院。她母亲的兄弟姐妹在儿童期或成年期死于Brugada综合征,该家族的在世成员是Brugada基因突变携带者。此次妊娠过程顺利。患者经阴道分娩,未出现任何心律失常。在分娩期间、分娩时及产后12小时进行了严格的心脏监测。结论。尽管低风险患者可能从未出现心律失常,但某些情况可能引发Brugada综合征。管理可能非常简单且顺利。否则可能会非常困难,需要体外膜肺氧合(ECMO)、抗心律失常药物或心内装置。Brugada综合征孕妇的产科管理应非常严格,与心脏病专家和麻醉师多学科合作,并应向夫妇提供知情同意书。