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女性法洛四联症患者妊娠:短期和长期结局。

Pregnancy in women with a Fontan circulation: Short and long-term outcomes.

机构信息

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom.

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College London, London, United Kingdom.

出版信息

Int J Cardiol. 2024 Nov 15;415:132445. doi: 10.1016/j.ijcard.2024.132445. Epub 2024 Aug 10.

DOI:10.1016/j.ijcard.2024.132445
PMID:39134124
Abstract

BACKGROUND

The Fontan circulation procedure is the palliative surgery of choice for patients with single ventricle physiology, many of whom are now reaching childbearing age due to advances in care. Our study examines the impact of pregnancy on women with Fontan circulation, assessing both short and long-term outcomes.

METHODS

We retrospectively analysed pregnancies in women with Fontan circulation at our centre from 2005 to 2023, including a matched non-pregnant, nulliparous cohort for comparison. Pregnancies lost before 18 weeks were analysed separately.

RESULTS

Among 26 pregnancies in 18 women, preterm births were common (73.1%), with 3 fetal losses and no maternal deaths. Neonatal complications included a 50% incidence of babies small for gestational age (SGA). Cardiac events occurred in 19.2% of pregnancies, primarily supraventricular arrhythmias, and 23% experienced postpartum haemorrhage. Over a median 6.1 [5.2-10.6]-year follow-up, no deaths, heart failure (HF), or Fontan-associated liver disease (FALD) developed, and functional status remained stable.

CONCLUSIONS

Women with a Fontan-type circulation undergoing pregnancy had a few serious maternal cardiac events, though there was a high rate of post-partum haemorrhage. The rates of neonatal complications, particularly related to restricted growth and pre-term birth, were also high. Our findings indicate a generally positive medium-term outlook for these patients, though the impact of pregnancy on long-term survival remains unclear. Careful selection of patients with Fontan circulation during pre-pregnancy counselling may help to reduce complications during and after pregnancy.

摘要

背景

Fontan 循环手术是单心室生理患者的姑息性手术选择,由于治疗的进步,许多患者现在已达到生育年龄。我们的研究考察了妊娠对 Fontan 循环患者的影响,评估了短期和长期结局。

方法

我们回顾性分析了 2005 年至 2023 年在我们中心接受 Fontan 循环的女性的妊娠情况,包括为比较而设立的非妊娠、未生育的匹配队列。对 18 周前流产的妊娠分别进行分析。

结果

在 18 名女性的 26 次妊娠中,早产很常见(73.1%),有 3 例胎儿丢失,无孕产妇死亡。新生儿并发症包括 50%的婴儿小于胎龄(SGA)。心脏事件发生在 19.2%的妊娠中,主要是室上性心律失常,23%的患者出现产后出血。在中位随访 6.1[5.2-10.6]年期间,无死亡、心力衰竭(HF)或 Fontan 相关肝疾病(FALD)发生,功能状态保持稳定。

结论

Fontan 型循环的女性在妊娠期间发生了一些严重的母体心脏事件,但产后出血的发生率很高。新生儿并发症的发生率也很高,特别是与生长受限和早产相关的并发症。我们的研究结果表明,这些患者的中期预后总体良好,但妊娠对长期生存的影响仍不清楚。在妊娠前咨询期间仔细选择 Fontan 循环患者,可能有助于减少妊娠期间和之后的并发症。

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