Risk of Development of Abdominal Aortic Aneurysm and Dissection of Thoracic Aorta in a Postpartum Womanwith Marfans Syndrome

Tomihara, Aki; Ashizawa, Naoto; Abe, Kuniko; Kinoshita, N.; Chihaya, Keisuke; Yonekura, Tsuyoshi; Fukae, Satoki; Ashizawa, Kazuto; Eishi, Kiyoyuki; Hayashi, Tomayoshi; Yano, Katsusuke

doi:10.2169/internalmedicine.45.1880

Cited by 16 publications

(8 citation statements)

References 19 publications

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“…The mean ± SD days after delivery for postpartum dissections in the MFS group ( n = 8) was 44 ± 78.50; in the non‐MFS group ( n = 13), these values were 11.23 ± 14.78, P = .280). The large standard deviation in the MFS group for postpartum dissections can be attributed to 2 outliers of dissections at 7‐months postpartum and 4‐months postpartum . The median values for days after delivery for presentation with a postpartum dissection were 5.5 days (range 1 day‐7 months) in the MFS group and 7 days (range 1 day‐2 months) in the non‐MFS group.…”

Section: Resultsmentioning

confidence: 96%

“…Only 6 of the MFS cases reported beta‐blocker therapy during the pregnancy . None of the cases with non‐MFS patients reported beta‐blocker therapy.…”

Section: Resultsmentioning

confidence: 99%

“…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%

“…In the literature identified in this study, only 6 of the MFS cases reported beta-blocker therapy during the pregnancy. 35,44,47,52,54,79 It is unclear if the other MFS patients were not receiving beta-blocker therapy during their pregnancy, if they were treated with beta blockade prior to pregnancy and discontinued therapy, or if the patients were receiving beta blockers but it was not reported in the literature.…”

Section: Beta Blockersmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 96%

“…Only 6 of the MFS cases reported beta‐blocker therapy during the pregnancy . None of the cases with non‐MFS patients reported beta‐blocker therapy.…”

Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Beta Blockersmentioning

confidence: 99%

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

Smith

Gros

2017

Congenital Heart Disease

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“…M arfan syndrome is an autosomal dominant, multisystem disease characterized by long bone overgrowth and other skeletal abnormalities, such as dislocation of the ocular lens, pneumothorax, decreased skeletal muscle mass, mitral valve prolapse, and aortic arch dilatation. 1,2 The combination of structural microfibril matrix abnormalities, dysregulation of matrix homeostasis mediated by excess transforming growth factor, and abnormal cell-matrix interactions is responsible for the phenotypic features of Marfan syndrome. 1,3,4 Aortic dilatation in Marfan syndrome is related to degeneration of the medial layer, with fragmentation, disarray, and loss of elastic lamina with replacement by basophilic-staining proteoglycan.…”

mentioning

confidence: 99%

Retrograde Endovascular Treatment of Internal Iliac Aneurysm in a Patient with Marfan Syndrome

Yoshida

Kolvenbach

et al. 2010

Vascular

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To report a technique to maintain pelvic flow to an internal iliac artery (IIA) with aneurysm in a patient with Marfan syndrome, and previously treated by infrarenal abdominal aortic aneurysm open procedure. Retrograde endovascular hypogastric artery preservation (REHAP) through flexible endograft implantation from external iliac artery (EIA) to internal iliac artery (IIA) was used. REHAP was a reasonable, minimally invasive and elegant alternative (new) to maintain pelvic arterial flow in Marfan syndrome. However, the long-term durability is unknown, and so, it should be used in selected patients.

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Marfan Syndrome and Pregnancy

Goland

Elkayam

2019

Cardiac Problems in Pregnancy, 4th Edition

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Risk of Development of Abdominal Aortic Aneurysm and Dissection of Thoracic Aorta in a Postpartum Womanwith Marfans Syndrome

Abstract: Abstract

Cited by 16 publications

References 19 publications

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

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

Retrograde Endovascular Treatment of Internal Iliac Aneurysm in a Patient with Marfan Syndrome

Marfan Syndrome and Pregnancy

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