特纳综合征女性的妊娠：一项回顾性英国多中心研究。
Pregnancies in women with Turner syndrome: a retrospective multicentre UK study.
机构信息
Department of Obstetrics, Maternal Medicine Service, St George's Hospital, London, UK.
Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, London, UK.
出版信息
BJOG. 2022 Apr;129(5):796-803. doi: 10.1111/1471-0528.17025. Epub 2022 Jan 4.
OBJECTIVE
To determine the characteristics and outcomes of pregnancy in women with Turner syndrome.
DESIGN
Retrospective 20-year cohort study (2000-20).
SETTING
Sixteen tertiary referral maternity units in the UK.
POPULATION OR SAMPLE
A total of 81 women with Turner syndrome who became pregnant.
METHODS
Retrospective chart analysis.
MAIN OUTCOME MEASURES
Mode of conception, pregnancy outcomes.
RESULTS
We obtained data on 127 pregnancies in 81 women with a Turner phenotype. All non-spontaneous pregnancies (54/127; 42.5%) were by egg donation. Only 9/31 (29%) pregnancies in women with karyotype 45,X were spontaneous, compared with 53/66 (80.3%) pregnancies in women with mosaic karyotype 45,X/46,XX (P < 0.0001). Women with mosaic karyotype 45,X/46,XX were younger at first pregnancy by 5.5-8.5 years compared with other Turner syndrome karyotype groups (P < 0.001), and more likely to have a spontaneous menarche (75.8% versus 50% or less, P = 0.008). There were 17 miscarriages, three terminations of pregnancy, two stillbirths and 105 live births. Two women had aortic dissection (2.5%); both were 45,X karyotype with bicuspid aortic valves and ovum donation pregnancies, one died. Another woman had an aortic root replacement within 6 months of delivery. Ten of 106 (9.4%) births with gestational age data were preterm and 22/96 (22.9%) singleton infants with birthweight/gestational age data weighed less than the tenth centile. The caesarean section rate was 72/107 (67.3%). In only 73/127 (57.4%) pregnancies was there documentation of cardiovascular imaging within the 24 months before conceiving.
CONCLUSIONS
Pregnancy in women with Turner syndrome is associated with major maternal cardiovascular risks; these women deserve thorough cardiovascular assessment and counselling before assisted or spontaneous pregnancy managed by a specialist team.
TWEETABLE ABSTRACT
Pregnancy in women with Turner syndrome is associated with an increased risk of aortic dissection.
目的
确定特纳综合征女性妊娠的特征和结局。
设计
回顾性 20 年队列研究（2000-20 年）。
地点
英国 16 家三级转诊产科单位。
人群或样本
特纳表型共 81 名妊娠妇女。
方法
回顾性图表分析。
主要观察指标
受孕方式，妊娠结局。
结果
我们获得了 81 名特纳表型女性 127 例妊娠的数据。所有非自然受孕（54/127；42.5%）均通过捐卵。核型 45,X 的自发性妊娠仅占 9/31（29%），而嵌合体核型 45,X/46,XX 的妊娠占 53/66（80.3%）（P < 0.0001）。与其他特纳综合征核型组相比，嵌合体核型 45,X/46,XX 的女性首次妊娠年龄小 5.5-8.5 岁（P < 0.001），且更有可能出现自然初潮（75.8%比 50%或更低，P = 0.008）。共有 17 例流产、3 例终止妊娠、2 例死产和 105 例活产。有 2 例发生主动脉夹层（2.5%）；均为核型 45,X 伴二叶主动脉瓣和捐卵妊娠，1 例死亡。另一名女性在分娩后 6 个月内进行了主动脉根部置换术。106 例有胎龄数据的分娩中有 10 例（9.4%）早产，96 例有出生体重/胎龄数据的单胎婴儿中有 22 例（22.9%）体重低于第 10 百分位数。剖宫产率为 72/107（67.3%）。在 127 例妊娠中，只有 73 例（57.4%）在受孕前 24 个月内有心血管影像学检查记录。
结论
特纳综合征女性妊娠与母体心血管风险增加相关；这些女性应在专家团队管理的辅助或自然妊娠前接受全面的心血管评估和咨询。
推文摘要
特纳综合征女性妊娠与主动脉夹层风险增加相关。
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