Careful cardiovascular screening and follow-up of women with Turner syndrome before and during pregnancy is necessary to prevent maternal mortality

Boissonnas, Céline Chalas; Davy, Céline; Bornes, Marie; Arnaout, Leila; Meune, Christophe; Tsatsatris, Vassilis; Mignon, Alexandre; Jouannet, Pierre

doi:10.1016/j.fertnstert.2008.09.037

Cited by 58 publications

(40 citation statements)

References 17 publications

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“…Boissonnas et al re-evaluated 18 women with TS requesting OD, who had undergone pre-pregnancy cardiac evaluations before treatment (Boissonnas et al, 2009). All women were considered to be cardiovascular healthy.…”

Section: Discussionmentioning

confidence: 99%

Obstetric and neonatal outcome after oocyte donation in 106 women with Turner syndrome: a Nordic cohort study

et al. 2013

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summary answer: Pregnancies among women with TS carry a substantial risk, particularly for hypertensive disorders. Potentially lifethreatening complications occurred in 3.3% of pregnancies. The neonatal outcomes were generally reassuring, with similar rates of preterm birth and low birthweight (LBW) as after conventional IVF and better than previously reported in deliveries after OD in women with TS.what is known already: OD pregnancies in women with TS are known to be high-risk pregnancies. study design, size, duration: This retrospective cohort study included 106 women with TS who delivered after OD (n ¼ 122 deliveries, n ¼ 131 newborns) in three Nordic countries (Finland, Denmark, Sweden) between 1992 and participants, setting and methods: Women with TS who delivered after OD in three Nordic countries were identified (n ¼ 110). Four women declined to participate or were lost to follow-up, thus 106 women were included in the study. The medical data from fertility clinics, antenatal clinics and the hospitals where the women had been treated and/or delivered were scrutinized.main results and the role of chance: In this cohort, the karyotype was 45,X in 44% of the women with TS. Ten women (9.4%) had a known cardiac defect before pregnancy. Single embryo transfer was performed in 70.3% of the cases and the multiple birth rate was 7.4%. In total, 35.0% of the pregnancies were associated with a hypertensive disorder including pre-eclampsia in 20.5%. Potentially lifethreatening complications occurred in four pregnancies (3.3%), including one woman with aortic dissection, one with mild regurgitation of the tricuspid and mitral valve, one with a mechanical heart valve who developed HELLP syndrome (haemolysis, elevated liver enzymes, low platelets) and one who underwent a post-partum hysterectomy due to severe haemorrhaging. Neonatal outcomes were reassuring, with a preterm birth rate of 8.0% and LBW rate of 8.8% in singletons. Major birth defects were found in 3.8% of the children. The perinatal mortality was 2.3% (3/131), including a set of extremely preterm twins.limitations, reasons for caution: Although this study was performed over a period of almost 20 years in three different countries, with a low drop-out rate and little missing data, much larger series are needed to assess rare events. This study also lacks an appropriate control group. Reproduction, Vol.28, No.6 pp. 1598-1609, 2013 Advanced Access publication on March 27, 2013 doi:10.1093/humrep/det082 Downloaded from https://academic.oup.com/humrep/article-abstract/28/6/1598/605487 by guest on 07 June 2019 wider implications of the findings: This study suggests that cardiovascular evaluation before and during pregnancy may contribute to favourable obstetric outcomes in many cases. Maternal outcomes were in agreement with the literature while neonatal outcomes were generally better than previously reported. The outcomes were consistent across the three countries, supporting generalizability to similar populations.

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Section: Discussionmentioning

confidence: 99%

Obstetric and neonatal outcome after oocyte donation in 106 women with Turner syndrome: a Nordic cohort study

et al. 2013

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“…Intensive cardiac screening is recommended before pregnancy, but normal results do not preclude the possibility of maternal death from AoD or aortic rupture (228, 231,251,252,253). More challenging is the patient who has identifiable risk factors for AoD, but who wishes to proceed with fertility treatment.…”

Section: Counseling and Ethical Considerations About Fertility Presermentioning

confidence: 99%

“…We suggest that during pregnancy prophylactic surgery is reasonable in case of a dilated aorta with rapid increase in diameter (⨁◯◯◯). In 1997, the first reports of serious cardiac complications and deaths in pregnant women with TS were published (254), followed by additional reports of deaths after AoD related to pregnancy (228, 231,251,252,253). The risk of maternal death from aortic dissection/ rupture during pregnancy in women with TS has been estimated to be 2% (231,255).…”

Section: Cardiovascular Risks During Pregnancymentioning

confidence: 99%

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Gravholt¹,

Andersen

Conway

et al. 2017

European Journal of Endocrinology

908

1,259

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Turner syndrome affects 25-50 per 100,000 females and can involve multiple organs through all stages of life, necessitating multidisciplinary approach to care. Previous guidelines have highlighted this, but numerous important advances have been noted recently. These advances cover all specialty fields involved in the care of girls and women with TS. This paper is based on an international effort that started with exploratory meetings in 2014 in both Europe and the USA, and culminated with a Consensus Meeting held in Cincinnati, Ohio, USA in July 2016. Prior to this meeting, five groups each addressed important areas in TS care: 1) diagnostic and genetic issues, 2) growth and development during childhood and adolescence, 3) congenital and acquired cardiovascular disease, 4) transition and adult care, and 5) other comorbidities and neurocognitive issues. These groups produced proposals for the present guidelines. Additionally, four pertinent questions were submitted for formal GRADE (Grading of Recommendations, Assessment, Development and Evaluation) evaluation with a separate systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association, the Society for Endocrinology, and the European Society of Cardiology. The guideline has been formally endorsed by the European Society of Endocrinology, the Pediatric Endocrine Society, the European Society for Paediatric Endocrinology, the European Society of Human Reproduction and Embryology and Summary of recommendationsThe recommendations (R) are worded as recommend (strong recommendation) and suggest (weak recommendation). We formally graded only the evidence underlying recommendations for therapeutic choices. The quality of evidence behind the recommendations is classified as very low (⨁◯◯◯), low (⨁⨁◯◯), moderate (⨁⨁⨁◯) and strong (⨁⨁⨁⨁). See further section 'Summary of methods used for guideline development'. Diagnosis and geneticsR 1.1. We recommend considering a diagnosis of TS in phenotypic females with a karyotype containing one X chromosome and complete or partial absence of the second sex chromosome, associated with one or more typical clinical manifestations of TS (⨁⨁⨁⨁). R 1.2. We recommend against considering a diagnosis of TS in females with one X chromosome and a deletion distal to Xq24 on the other X chromosome, and in women over the age of 50 years with less than 5% 45,X mosaicism (⨁⨁◯◯). R 1.3. We suggest that the new general surveillance management guideline applies to TS patients with any karyotype (⨁⨁◯◯). R 1.4. We recommend to consider testing for Turner syndrome (TS) in a female with typical signs (Table 2) (⨁⨁⨁⨁). R 1.5. We ...

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“…Recommendations regarding pregnancy in TS have been stated by the American Society for Reproductive Medicine (5). Recently, a case report described a woman with TS with a Y-fragment who passed an oocyte donation but died because of acute aortic dissection in the 38th week of the pregnancy (21). In retrospect, she had a bicuspid aortic valve, which might have predisposed her dissection (21)(22)(23).…”

mentioning

confidence: 99%

Pregnancy rate and outcome in Swedish women with Turner syndrome

Bryman

Sylvén

Berntorp

et al. 2011

Fertility and Sterility

138

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Careful cardiovascular screening and follow-up of women with Turner syndrome before and during pregnancy is necessary to prevent maternal mortality

Cited by 58 publications

References 17 publications

Obstetric and neonatal outcome after oocyte donation in 106 women with Turner syndrome: a Nordic cohort study

Obstetric and neonatal outcome after oocyte donation in 106 women with Turner syndrome: a Nordic cohort study

Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting

Pregnancy rate and outcome in Swedish women with Turner syndrome

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