The Marfan syndrome and pregnancy: a retrospective study in a Dutch population

Lind, Jan; Wallenburg, Henk C.S.

doi:10.1016/s0301-2115(01)00314-1

Cited by 149 publications

(99 citation statements)

References 15 publications

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“…69 Aortic dissection occurs in around 4.5% of pregnancies in women with Marfan syndrome 69 and the risk is greater if the aortic root exceeds 4 cm at the start of pregnancy, or if it dilates rapidly. 70 More frequent monitoring of aortic diameter in pregnancy is advisable. The rate of aortic dilatation is greater in women who have been pregnant with an aortic root diameter of more than 4 cm, than in women who have remained childless, or women with children whose aortic root is less than 4 cm.…”

Section: Pregnancy In Marfan Syndromementioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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In association withMarfan syndrome: clinical diagnosis and management Marfan syndrome is a multisystem connective tissue disorder usually associated with mutation in fibrillin, and occasionally with mutation in TGFBR1 or 2. The clinical diagnosis is made using the Ghent nosology, which will unequivocally diagnose or exclude Marfan syndrome in 86% of cases. Use of a care pathway can help implementation of the nosology in the clinic. The penetrance of some features is age dependent, so the nosology must be used with caution in children. Molecular testing may be helpful in this context. The nosology cannot be used in families with isolated aortic dissection, or with related conditions such as Loeys -Dietz syndrome, although it may help identify families for further diagnostic evaluation because they do not fulfill the nosology, despite a history of aneurysm. Prophylactic medical (eg b-blockade) and surgical intervention is important in reducing the cardiovascular complications of Marfan syndrome. Musculoskeletal symptoms are common, although the pathophysiology is less clear -for example, the correlation between dural ectasia and back pain is uncertain. Symptoms in other systems require specialist review such as ophthalmology assessment of refractive errors and ectopia lentis. Pregnancy is a time of increased cardiovascular risk for women with Marfan syndrome, particularly if the aortic root exceeds 4 cm at the start of pregnancy. High-intensity static exercise should be discouraged although low-moderate intensity dynamic exercise may be beneficial. The diagnosis and management of Marfan syndrome requires a multidisciplinary team approach, in view of its multisystem effects and phenotypic variability.

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Section: Pregnancy In Marfan Syndromementioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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“…In summary, the rates of aortic complications reported in prospective studies appear to reach on average 4.0% (3,19,41,53) and is 2-6% in large retrospective reports including 1,142 pregnancies (20,22,(53)(54)(55)(56)(57)(58). The expected rate of AoD is ranging from 1% in women with aortic diameter <40 mm to as much as 10% in high-risk patients (those with aortic root diameter >40 mm, rapid dilatation or previous dissection of the ascending aorta) (3,9,53,59).…”

Section: Risk For the Mothermentioning

confidence: 89%

“…Immer et al (18) described 16 cases of pregnant women with MFS with type A dissection (mean aortic root diameter of 4.8±0.8 cm) with no maternal death, however, fetal deaths were reported in three cases. Goland et al (9) in a review of 39 cases of pregnancy related complications described AoD in 29, involving the ascending aorta (19 cases), descending aorta (8 cases) or both (2 cases), 8 of these women were undiagnosed with MFS prior to the occurrence of aortic complications (9,(19)(20)(21)(22). Five patients developed an AoD before week 20 of gestation (13-20 weeks), 18 patients at 24-40 weeks, and 6 after delivery.…”

Section: Risk For the Mothermentioning

confidence: 99%

“…Such an assumption is supported by several reports of significantly lower rate of complications. Pyeritz et al (17) Several studies focused on the potential growth of the aorta in pregnant women with MFS and have reported contradictory results (19,22,51,53,55). Rossiter et al (19) prospectively evaluated 45 pregnancies in 21 women with MFS and found AoD in only 2 while the remaining women with an aortic diameter <40 mm tolerated pregnancy well without an increase in aortic diameter.…”

Section: Risk For the Mothermentioning

confidence: 99%

“…Pregnancy in women with MFS has been shown to be associated with a high rate (up to 40%) of obstetric complications, such as premature delivery, mainly due to premature rupture of membranes, intrauterine growth restriction, small for gestational age and neonatal mortality (8,9,22,41,51,56,65,66).…”

Section: Risk For the Fetus And Obstetric Complicationsmentioning

confidence: 99%

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Pregnancy and Marfan syndrome

Goland¹,

Elkayam²

2017

Ann. Cardiothorac. Surg.

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Pregnancy in women with Marfan syndrome (MFS) presents challenges to the clinician and the patient due to the increased incidence of maternal complications and involvement of the fetus, and deserves special consideration. The leading cause of morbidity and mortality in MFS is aortic dissection. This article presents an extensive review of available clinical information and provides recommendations for the management of patients with MFS during pregnancy.

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