Pregnancy in Marfan Syndrome

Goland, Sorel; Barakat, Mohamad; Khatri, Nudrat; Elkayam, Uri

doi:10.1097/crd.0b013e3181bb83d3

Cited by 43 publications

(12 citation statements)

References 64 publications

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“…In our series, the more fully expressed the phenotype, the smaller the baby, although this was not a very strong trend. The increased rate of SGA in our study may result from the current universal recommendation that women with MFS should be prescribed beta-blockers for aortic root protection (in non-pregnant patients they have been shown to have a small impact on the rate of growth of the aortic root) 2,22–24. In our study beta-blockers were taken throughout 26 of 29 pregnancies: three women declined them because of their inability to tolerate the side effects.…”

Section: Discussionmentioning

confidence: 82%

“…The mean increase in aortic root diameter during pregnancy was 0.47 mm (median 0.40 mm, range 0.00–0.90 mm), although upon regression analysis this change was not statistically significant. Beta-blockers (thought to confer some protection against long-term dilatation of the aortic root) were taken throughout 26 pregnancies (two of the three pregnancies where a beta-blocker was not taken were in women with an aortic root >40 mm) 2,22–24…”

Section: Resultsmentioning

confidence: 99%

“…It is caused by a mutation in the fibrillin–1 gene ( FBN1 ) on chromosome 15q21, although heterozygous mutations in the tissue growth factor–B receptor 2 ( TGFBR2 ) gene on chromosome 3p24.2–25 have also been identified in other Marfan-like syndromes 2. Diagnosis is based on the 2010 revised Ghent nosology 1.…”

Section: Introductionmentioning

confidence: 99%

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Marfan syndrome and pregnancy: maternal and neonatal outcomes

Curry

Gelson

Swan

et al. 2014

BJOG

122

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ObjectiveTo report outcomes in a recent series of pregnancies in women with Marfan syndrome (MFS).DesignRetrospective case note review.SettingTertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals).SampleTwenty-nine pregnancies in 21 women with MFS between 1995 and 2010.MethodsMultidisciplinary review of case records.Main outcome measuresMaternal and neonatal mortality and morbidity of patients with MFS and healthy controls.ResultsThere were no maternal deaths. Significant cardiac complications occurred in five pregnancies (17%): one woman experienced a type–A aortic dissection; two women required cardiac surgery within 6 months of delivery; and a further two women developed impaired left ventricular function during the pregnancy. Women with MFS were also more likely to have obstetric complications (OR 3.29, 95% CI 1.30–8.34), the most frequent of which was postpartum haemorrhage (OR 8.46, 95% CI 2.52–28.38). There were no perinatal deaths, although babies born to mothers with MFS were delivered significantly earlier than those born to the control group (median 39 versus 40 weeks of gestation, Mann–Whitney U–test, P = 0.04). These babies were also significantly more likely to be small for gestational age (24% in the MFS group versus 6% in the controls; OR 4.95, 95% CI 1.58–15.55).ConclusionsPregnancy in women with MFS continues to be associated with significant rates of maternal, fetal, and neonatal complications. Effective pre-pregnancy counselling and meticulous surveillance during pregnancy, delivery, and the puerperium by an experienced multidisciplinary team are warranted for women with MFS.

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Section: Discussionmentioning

confidence: 82%

Section: Resultsmentioning

confidence: 99%

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Marfan syndrome and pregnancy: maternal and neonatal outcomes

Curry

Gelson

Swan

et al. 2014

BJOG

122

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“…Vaginal delivery can be safely performed in these women however in order to reduce the stress of labour, epidural anaesthesia and vacuum or forceps delivery to shorten the second stage are recommended. In high-risk women (aortic root diameter > 40 mm, progressive dilation during pregnancy or previous dissection) elective caesarean section is the preferred mode of delivery [4]. …”

Section: Discussionmentioning

confidence: 99%

“…However, there is still some debate regarding the aortic root diameter above which pregnancy should be discouraged in women with Marfan syndrome [4]. Nevertheless, appropriate counselling and close echocardiographic followup cannot be applied if women have not been diagnosed prior to pregnancy.…”

Section: Introductionmentioning

confidence: 99%

Acute Aortic Dissection in Pregnancy in a Woman with Undiagnosed Marfan Syndrome

Master

Day²

2012

Case Reports in Obstetrics and Gynecology

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We report a case of acute aortic dissection in a lady of 28 weeks of gestation with undiagnosed Marfan syndrome. The patient had been seen in our antenatal clinics. Her history documented in her pregnancy record was negative for genetic/congenital abnormalities. There was no family history documented. Subsequently, at 28 weeks of gestation, the patient presented with sudden onset chest, jaw, and back pain. Further history revealed that her father had died at the age of 27 of an aortic dissection. Echocardiography showed aortic root dissection with occlusion of aortic branches. She subsequently underwent an emergency lower segment caesarean section followed by surgical repair of type A dissection. A simultaneous type B dissection was managed conservatively. On later examination, our patient fulfilled the diagnostic criteria for phenotypic expression of Marfan syndrome. Genetic testing also confirmed that she has a mutation of the fibrillin (FBN 1) gene associated with the disease.

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Management of Acute Aortic Syndromes

Nienaber¹,

Clough²

2016

Interventional Cardiology

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Pregnancy in Marfan Syndrome

Cited by 43 publications

References 64 publications

Marfan syndrome and pregnancy: maternal and neonatal outcomes

Marfan syndrome and pregnancy: maternal and neonatal outcomes

Acute Aortic Dissection in Pregnancy in a Woman with Undiagnosed Marfan Syndrome

Management of Acute Aortic Syndromes

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