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免疫介导的胎儿完全性房室传导阻滞:地塞米松治疗能否逆转这一过程?

Immune-Mediated Fetal Complete Atrioventricular Block: Can Dexamethasone Therapy Revert the Process?

作者信息

Perles Zeev, Ishay Yuval, Nir Amiram, Gavri Sagui, Golender Julius, Ta-Shma Asaf, Abu-Zahira Ibrahim, Natsheh Juma, Elchalal Uriel, Mevorach Dror, Rein Azaria Jjt

机构信息

Department of Pediatric Cardiology, Hadassah Hebrew University Hospital, Jerusalem, Israel.

Department of Internal Medicine B, Hadassah Hebrew University Hospital, Jerusalem, Israel.

出版信息

Isr Med Assoc J. 2020 Nov;11(22):711-716.

Abstract

Fetal complete atrioventricular block (CAVB) is usually autoimmune mediated. The risk of developing CAVB is 2% to 3% in anti-Ro/SS-A seropositive pregnancies and it increases 10 times after previous CAVB in siblings. Despite being a rare complication, CAVB carries a 20% mortality rate and substantial morbidity, as about 65% of newborns will eventually need life-long pacing. Once found, fetal CAVB is almost always irreversible, despite aggressive immunotherapy. This poor outcome prompted some research groups to address this situation. All groups followed anti-Ro/SS-A seropositive pregnancies on a weekly basis during the second trimester of pregnancy and tried to detect first degree atrioventricular block (AVB) using accurate echocardiographic tools, assuming they may characterize the initiation of the immune damage to the A-V conduction system, at which point the process might still be reversible. Some of the groups treated fetuses with first degree AVB with maternal oral fluorinated steroids. We summarized the results of all groups, including our group. We describe a case of a fetus that developed CAVB 6 days after normal sinus rhythm (NSR), who under aggressive dexamethasone therapy gradually reverted to NSR. This fetus had a previous sibling with CAVB. We assumed the immune damage to the conduction system in this small group of fetuses with a previous CAVB sibling may have occurred more quickly than usual. We therefore recommend a twice-weekly follow-up with these fetuses.

摘要

胎儿完全性房室传导阻滞(CAVB)通常由自身免疫介导。抗Ro/SS-A血清学阳性的妊娠中发生CAVB的风险为2%至3%,而同胞中有既往CAVB病史时,该风险会增加10倍。尽管CAVB是一种罕见的并发症,但它的死亡率为20%,且发病率很高,因为约65%的新生儿最终需要终身起搏治疗。一旦发现胎儿CAVB,尽管进行积极的免疫治疗,几乎总是不可逆的。这种不良结局促使一些研究团队着手应对这种情况。所有团队在妊娠中期每周对抗Ro/SS-A血清学阳性的孕妇进行随访,并尝试使用精确的超声心动图工具检测一度房室传导阻滞(AVB),假定一度AVB可能是对房室传导系统免疫损伤的起始阶段,此时该过程可能仍可逆。一些团队用母体口服氟化类固醇治疗一度AVB的胎儿。我们总结了所有团队(包括我们团队)的结果。我们描述了一例胎儿在正常窦性心律(NSR)6天后发生CAVB的病例,该胎儿在积极的地塞米松治疗下逐渐恢复为NSR。这个胎儿有一个患CAVB的同胞。我们推测,在这一小群有CAVB同胞的胎儿中,对传导系统的免疫损伤可能比平常发生得更快。因此,我们建议对这些胎儿每周随访两次。

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