The Marfan syndrome and pregnancy

Donaldson, L. Bruce; Alvarez, Russell R. de

doi:10.1016/0002-9378(65)90432-1

Cited by 15 publications

(4 citation statements)

References 21 publications

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“…Although cardiac output is increased during pregnancy because of increases in stroke volume and heart rate, substantial further increases occur in association with labor and delivery, potentially further increasing pulsatile stress on the aorta. Type B dissections have been described in previous reports; of these, 5 of 8 were postpartum. Reports of postpartum type A dissection are less common, including 1 of 3 in our series.…”

Section: Discussionmentioning

confidence: 89%

“…The early literature on pregnancy risk in women with MFS is limited to publication of single case reports or very small series . In addition, the diagnosis of MFS was not always firmly established, but presumptive, based on nonspecific skeletal features or in the setting of underlying bicuspid aortic valve, a known risk factor for aortic aneurysm and dissection. Furthermore, some women with a firm diagnosis of MFS at the time might have actually had Loeys‐Dietz syndrome, which had not yet been described and may carry a higher risk of dissection than MFS.…”

Section: Discussionmentioning

confidence: 99%

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Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)

Roman

Pugh

Hendershot

et al. 2016

JAHA

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)

Roman

Pugh

Hendershot

et al. 2016

JAHA

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“…Aortic dilatation usually precedes dissection and the advent of surgical techniques to replace the ascending aorta electively with a composite graft once the aortic root dimension reaches 5.5 cm has improved life expectancy 5–8 . Medical literature suggests that pregnancy increases the risk of aortic dissection 9–12 . Pyeritz 13 reviewed 32 case reports of women with Marfan's syndrome who had had at least one pregnancy, describing the occurrence of aortic dissection relating to pregnancy or the puerperium in 20 of these women, most of whom had pre‐existing cardiovascular abnormalities.…”

Section: Introductionmentioning

confidence: 99%

Outcome of pregnancy in women with Marfan's syndrome

Lipscomb

Smith²,

Clarke

et al. 1997

BJOG

183

114

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Objective To improve life expectancy and prevent premature mortality in women with Marfan's syndrome. Methods During the development of a regional genetic register for Marfan's Syndrome the outcome of 91 pregnancies in 36 women with this condition was established retrospectively and the cardiovascular and obstetric complications documented. Results No patient had a significant cardiovascular abnormality limiting function before her pregnancy. Of 36 women, four had an aortic dissection relating to pregnancy and two others required aortic surgery following delivery. Thirty women had uncomplicated gestational histories. The incidence of obstetric complications did not exceed expectation. Conclusions Women with Marfan's syndrome are at significant risk of aortic dissection in pregnancy even in the absence of preconceptional cardiovascular abnormality. Aortic root dilatation may be a predictor of risk but dissection may occur without significant dilatation. Guidelines for obstetric care are suggested and preconceptional assessment recommended.

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“…A similar etiology has been postulated for the high incidence of aortic dissection seen during pregnancy in patients with Marfan's syndrome. 13 This is the first case of maternal coronary artery dissection leading to myocardial infarction and death. One previous case of cardiac rupture of the anterior left ventricular wall leading to death in a non-pregnant female with EDS has been reported.…”

Section: Discussionmentioning

confidence: 95%

Myocardial Infarction and Coronary Artery Dissection During Pregnancy Associated with Type IV Ehlers-Danlos Syndrome

Athanassiou¹,

Turrentine²

1996

Amer J Perinatol

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Ehlers-Danlos syndrome (EDS) is a rare, genetically transmitted connective tissue disorder which occurs with an incidence of 1 in 5000. Nine subtypes of EDS have been identified, with type IV being associated with sudden death due to rupture of the bowel, uterus, or major blood vessels. We describe a case of a patient with type IV EDS who presented at 30 weeks' gestation with preterm labor which was treated with subcutaneous terbutaline followed by oral maintenance therapy. Approximately 72 hours later she suffered a myocardial infarction, which led to coronary artery dissection and death. This is the first case of type IV EDS in pregnancy that resulted in maternal death due to coronary artery dissection. We suggest that betamimetic tocolytics should be avoided in patients with EDS and preterm labor.

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The Marfan syndrome and pregnancy

Cited by 15 publications

References 21 publications

Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)

Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)

Outcome of pregnancy in women with Marfan's syndrome

Myocardial Infarction and Coronary Artery Dissection During Pregnancy Associated with Type IV Ehlers-Danlos Syndrome

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