Fatal Aortic Dissection in a Patient With a Family History of Marfan Syndrome

Birsner, Meredith L.; Farber, John L.; Berghella, Vincenzo

doi:10.1097/aog.0b013e3181743312

Cited by 8 publications

(4 citation statements)

References 9 publications

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“…The systematic literature search yielded 465 potentially eligible studies. After exclusion, cross‐referencing, and reaching an agreement on three authors, 80 studies were included in this systematic literature review 5–84 (Figure 1). This resulted in a total study of 103 patients with aortic dissection during 6 months before delivery to 12 weeks postpartum.…”

Section: Resultsmentioning

confidence: 99%

Aortic dissection during pregnancy and postpartum

Meng

Han

Wang

2021

Journal of Cardiac Surgery

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Background: Patients with aortic dissection during pregnancy and postpartum period exhibit a high mortality. At present, a complete overview of aortic dissection during pregnancy and postpartum period is lacking.Methods: This systematic review included 80 reports published from 2000 to 2020, comprising a total study population of 103 patients with aortic dissection.Results: We found that Stanford Type A aortic dissection was more common in prepartum cases, especially in the third trimester, while postpartum cases of aortic dissection were more common in Stanford Type B. The most common risk factor was connective tissue disease, with no other known risk factors. The mode of delivery had no significant effect on the type of postpartum aortic dissection. Reduced maternal and fetal mortality was observed when patients with Stanford Type A aortic dissection occurring after 28 gestational weeks underwent cesarean section followed by aortic replacement. Patients with Stanford Type B aortic dissection were treated mainly with medication and/or endovascular repair. Conclusion:Contemporary management of patients during pregnancy and within 12 weeks postpartum requires multidisciplinary cooperation and includes serial, noninvasive imaging, biomarker testing, and genetic risk profiling for aortopathy.Early diagnosis and accurate treatment are essential to reduce maternal and fetal mortality.

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Section: Resultsmentioning

confidence: 99%

Aortic dissection during pregnancy and postpartum

Meng

Han

Wang

2021

Journal of Cardiac Surgery

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“…However, we do not have detailed information concerning the clinical status of the women with diagnosed MFS who chose not to become pregnant, and it may therefore be possible that some of these individuals that decided not to become pregnant, indeed were very high risk patients (Sayama et al, 2018). We anticipate that no cardiac surveillance program was instituted for the previously undiagnosed women with MFS and the relatively high number of dissections among these women emphasizes the need of an early MFS diagnosis (Birsner et al, 2008). It is important to realize that the median age at diagnosis for a woman with MFS is 19 years, which means that more than half of the women with MFS and childbearing potential are undiagnosed (Groth et al, 2015; Vanem et al, 2020).…”

Section: Discussionmentioning

confidence: 99%

Maternal health and pregnancy outcome in diagnosed and undiagnosed Marfan syndrome: A registry‐based study

Groth

Nielsen

Sheyanth

et al. 2021

American J of Med Genetics Pt A

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In Marfan syndrome (MFS), pregnancy is considered as high risk due to the deficiency of fibrillin in the connective tissue and increased risk of aortic dissection. The objective was to demonstrate the consequences on maternal health, in women with diagnosed and undiagnosed MFS at the time of pregnancy and childbirth. By using national health care registries, we identified all pregnancy related outcomes, from women with MFS (n = 183) and an age‐matched background population (n = 18,300). We found 91 pregnancies during follow‐up. Significantly fewer women with MFS gave birth, compared to the background population. No women with known MFS had a pregnancy related aortic dissection but complications related to the cervix were increased (HR:19.8 [95% CI:2.2–177.5]). Fifty women with MFS were undiagnosed at the time of their first pregnancy and/or childbirth. Among these, there were more birth canal related complications HR:27.2 (95% CI: 2.3–315.0), preeclampsia (HR:2.25 [95% CI: 1.11–4.60]), fetal deaths (HR:12.3 [95% CI: 1.51–99.8]), and all delivery‐related dissections came from this subgroup. In conclusion, undiagnosed women with MFS experienced more pregnancy and childbirth related complications including fetal death, birth canal issues, preeclampsia, and aortic disease, which emphasizes the need for an early MFS diagnosis and special care during pregnancy and childbirth.

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“…We identified 50 articles that fit our inclusion criteria (41 solitary cases and 9 case series) . The 50 articles yielded a total of 72 patients who met the inclusion criteria (AAD during pregnancy or within 1 year postpartum).…”

Section: Methodsmentioning

confidence: 99%

“…18,50.0%), and an equal number of patients presented with second trimester and postpartum dissections (n 5 8, 22.2%). Two patients in the MFS group presented with a dissection in the first trimester (n 5 2, 5.6%).…”

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confidence: 99%

Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature

Smith

Gros

2017

Congenital Heart Disease

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A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of women with Marfan syndrome (n = 36) and without Marfan syndrome (n = 36) were conducted. There were no significant differences in demographics (age, gravidity, parity) between the Marfan and non-Marfan cases. Marfan patients presented with antepartum dissections significantly earlier in pregnancy than those without Marfan syndrome (P = .002). However, there were no significant difference between the 2 groups in maternal mortality, fetal mortality, or obstetric outcomes (mode of delivery and gestational age at delivery). Eight cases described events in Marfan women with an aortic root diameter ≤40 mm. Six events occurred in Marfan women who were managed with beta blockers. Current guidelines rely on aortic root diameter for stratification of Marfan women into risk categories, but we identified several cases that would be missed by these guidelines. Specifically, the existing literature suggest that women with Marfan syndrome should take precautions throughout pregnancy, rather than the third trimester.

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Fatal Aortic Dissection in a Patient With a Family History of Marfan Syndrome

Cited by 8 publications

References 9 publications

Aortic dissection during pregnancy and postpartum

Aortic dissection during pregnancy and postpartum

Maternal health and pregnancy outcome in diagnosed and undiagnosed Marfan syndrome: A registry‐based study

Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature

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