scholarly journals Pregnancy-Related Aortic Aneurysm and Dissection in Patients With Marfan’s Syndrome: Medical and Surgical Management During Pregnancy and After Delivery

Medicina ◽  
2011 ◽  
Vol 47 (11) ◽  
pp. 87 ◽  
Author(s):  
Mile Vranes ◽  
Milos Velinovic ◽  
Natasa Kovacevic-Kostic ◽  
Dragutin Savic ◽  
Dejan Nikolic ◽  
...  
Keyword(s):  
Risk Factor ◽  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Aortic Root ◽  
Root Diameter ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Before And After ◽  
Aortic Aneurysm And Dissection ◽  
Current Article

In the current article, 3 cases of aortic aneurysm and dissection in pregnant patients with Marfan’s syndrome are reported. It is well known that pregnancy is a risk factor for the development of aortic aneurysm and dissection in women with Marfan’s syndrome since it is shown that dissection can develop both before and after labor. Marfan patients with an aortic root diameter greater than 4 cm should undergo preconceptual counseling for surgical aortic repair before pregnancy. Pregnant Marfan patients with an aortic aneurysm should be closely and continuously evaluated by multidisciplinary specialists in order to prevent possible aortic dissection that could be fatal for both the mother and the fetus.

scholarly journals Marfan’s syndrome and other aortopathies in pregnancy

2013 ◽  
Vol 6 (3) ◽  
pp. 112-119 ◽  
Author(s):  
Fiona M Stewart
Keyword(s):  
Aortic Valve ◽  
Aortic Aneurysm ◽  
Aortic Dissection ◽  
Bicuspid Aortic Valve ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Risk Of Death ◽  
Type Iv ◽  
In Pregnancy ◽  
Aortic Dimensions

Aortopathies, or disease affecting the aorta, are associated with a significant mortality risk for the mother and foetus during pregnancy because of an increased rate of aortic dissection. The hereditary aortopathies; Marfan’s syndrome, bicuspid aortic valve, Loeys–Dietz syndrome, Ehlers–Danlos (type IV) syndrome, Turner’s syndrome and nonsyndromic familial thoracic aortic aneurysm and dissection are all associated with an increased risk of aortic dissection particularly during the third trimester and early postpartum period. Maternal outcome in pregnancy depends on the underlying disorder and the aortic dimensions prior to pregnancy. The foetus has up to 50% chance of inheriting the underlying genetic defect. Vasculitis, particularly Takayasu’s arteritis may also be a problem in pregnancy and predispose to aortic dissection. Prepregnancy review, including careful assessment of the aorta and prophylactic aortic surgery for an aortic aneurysm may reduce the risk of aortic dissection in pregnancy for some of the aortopathies but for women with Marfan’s syndrome, Loeys–Dietz syndrome and Ehlers–Danlos (vascular type IV) who have had surgery, the risk of death remains high. A subgroup of women with Marfan’s syndrome or a bicuspid aortic valve and normal aortic dimensions prepregnancy should do well in a pregnancy. Multidisciplinary pregnancy care with agreement on pregnancy follow-up, delivery and postpartum care with a crisis plan for an aortic dissection can improve pregnancy outcome and ensure prompt management of an aortic dissection should it occur.

scholarly journals Pregnancy after Aortic Root Replacement in Marfan's Syndrome: A Case Series and Review of the Literature

2018 ◽  
Vol 08 (04) ◽  
pp. e234-e240 ◽  
Author(s):  
Dominique Williams ◽  
Kathryn Lindley ◽  
Melissa Russo ◽  
Jennifer Habashi ◽  
Harry Dietz ◽  
...  
Keyword(s):  
Literature Review ◽  
Aortic Dissection ◽  
Aortic Root ◽  
Aortic Surgery ◽  
Tertiary Care ◽  
Case Series ◽  
Aortic Root Replacement ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Case Series Study

Objectives We sought to characterize pregnancy-related aortic complications in women with Marfan's syndrome who had prior aortic root replacement. Study Design This is a retrospective case series study and literature review of women with Marfan's syndrome with pregnancy after aortic root replacement. We surveyed women with Marfan's syndrome who had successful pregnancy after aortic root replacement using the Marfan Foundation Website and from two large tertiary care Marfan's clinics. Clinical data, counseling information, and details of pregnancy-related aortic complications were compiled. A literature review was performed assessing aortic outcomes in women with Marfan's syndrome with pregnancy after aortic surgery. Results Fourteen women with 20 pregnancies were identified. Two women had three pregnancies following root replacement for aortic dissection. There were no aortic dissections during the 20 pregnancies. In contrast, aortic dissection was frequently reported in the literature. Conclusions Women with Marfan's syndrome who become pregnant following aortic root replacement remain at risk for distal aortic dissection related to pregnancy. The exact risk is difficult to quantify but is not zero and women should be counseled accordingly.

scholarly journals A patient with Marfan’s syndrome who developed an acute aortic dissection at 28 weeks of pregnancy treated with aortic root replacement

2021 ◽  
Author(s):  
Riki Sumiyoshi ◽  
Hideki Morita ◽  
Kento Fujii ◽  
Takehiro Shirasughi ◽  
Hiroyuki Kawaura ◽  
...  
Keyword(s):  
Aortic Dissection ◽  
Aortic Root ◽  
Acute Aortic Dissection ◽  
Mechanical Valve ◽  
Aortic Root Replacement ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Total Hysterectomy ◽  
First Child ◽  
Physical Findings

AbstractThe patient was a 31-year-old pregnant woman who gave birth to her first child by vaginal delivery 7 years ago. She was diagnosed with Marfan’s syndrome based on physical findings; however, the condition was not diagnosed before the onset. The patient developed acute aortic dissection at 28 weeks of pregnancy. A cesarean section was first performed to save the patient’s life; then, a total hysterectomy was performed to prevent the risk of postpartum hemorrhage. Furthermore, aortic root replacement was performed using a temporary mechanical valve. The patient and her child have survived without any complications.

Aortic root diameter and nasal intermittent positive airway pressure treatment in Marfan's syndrome

2003 ◽  
Vol 63 (2) ◽  
pp. 131-134 ◽  
Author(s):  
J Verbraecken ◽  
BP Paelinck ◽  
M Willemen ◽  
P Van de Heyning ◽  
W De Backer
Keyword(s):  
Aortic Root ◽  
Airway Pressure ◽  
Positive Airway Pressure ◽  
Root Diameter ◽  
Pressure Treatment ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome

Valve-Sparing Replacement of the Aortic Root for a 2-year-old Child with Loeys-Dietz Syndrome

2009 ◽  
Vol 20 (1) ◽  
pp. 94-96 ◽  
Author(s):  
Yoshio Ootaki ◽  
Minhaz Uddin ◽  
Ross M. Ungerleider
Keyword(s):  
Surgical Treatment ◽  
Aortic Aneurysm ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Marfan’S Syndrome ◽  
Marfan's Syndrome ◽  
Valve Sparing

AbstractLoeys-Dietz syndrome is a newly recognized constellation that presents with aortic aneurysm or dissection similar to Marfan’s syndrome. We describe successful surgical treatment in a 2-year-old with the syndrome in whom we performed a valve-sparing replacement of the aortic root because of significant dilation of the aortic root and the ascending aorta.

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