Obstetric and cardiac outcomes in women with Marfan syndrome and an aortic root diameter ≤ 45mm

Minsart, Anne-Frédérique; Mongeon, François‐Pierre; Laberge, Anne‐Marie; Morin, Francine; Dore, Annie; Leduc, Line

doi:10.1016/j.ejogrb.2018.09.012

Cited by 16 publications

(20 citation statements)

References 35 publications

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“…8 Consensus guidelines on management of thoracic aortic disease in MFS do not specify how or where the aortic root should be measured. 12 17 Furthermore, studies that have reported echocardiographic measurements of aortic root at the Sinus of Valsalva for MFS in pregnancy also differ in convention of timing of measurement, with some taken during end-diastole 16 and others during mid-systole. 11 Whether to measure leading-edge to leading-edge, outer-edge to outer-edge, versus inner-edge to inner-edge differs between the pediatric and adult literature and varies depending on mode of imaging (CT vs. echo) and has changed over time.…”

Section: Discussionmentioning

confidence: 99%

“…11 Whether to measure leading-edge to leading-edge, outer-edge to outer-edge, versus inner-edge to inner-edge differs between the pediatric and adult literature and varies depending on mode of imaging (CT vs. echo) and has changed over time. 16 17 21 These discrepancies are important as they may cause variations of up to 4 mm, which could significantly change counseling. In our case, echocardiographic measurements were obtained during diastole measuring the widest portion of the right coronary to left coronary sinus from leading-edge to leading-edge in the parasternal long-axis view in accordance with 2015 American Society of Echocardiography guidelines.…”

Section: Discussionmentioning

confidence: 99%

“…10 Minsart et al prospectively followed 21 pregnancies in women with MFS and reported two complications, both of which were Type B dissections in women with an aortic root <40 mm. 16 Providers should be alerted to the fact that a substantial minority of MFS pregnancy related complications will occur more distally along the aorta and, therefore, the entire aorta should be imaged prior to pregnancy or as early on as possible. Serial measurements of the descending and abdominal aorta are costly and there is no established cutoff at which a patient is at increased risk; however, this may be considered in patients who have aneurysmal dilation of distal portions of the aorta at baseline.…”

Section: Discussionmentioning

confidence: 99%

“…Based on prospective studies in which women were followed with serial aortic root measurements and were under the care of a cardiologist/multidisciplinary team during pregnancy, the risk of aortic complications appears to be low, including when the aortic root is >40 mm but less than 45 mm. 7 11 13 14 15 16 Consistent with these findings, the 2014 European Society of Cardiology (ESC) guidelines on diagnosis and treatment of aortic disease 17 and the 2014 Canadian Cardiovascular Society position statement on the management of thoracic aortic disease, 18 as well as the ESC guidelines for the management of cardiovascular diseases during pregnancy, 19 recommend against pregnancy when the aortic root is >45 mm, or 41 to 45 mm in cases a rapidly enlarging aortic root or family history of dissection/sudden death.…”

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confidence: 99%

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Rupture of a Type B Aortic Dissection in a Postpartum Patient with Marfan Syndrome

Patberg

Duffy

Hameed

2019

AJP Rep

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Background Pregnant women with Marfan syndrome (MFS) are known to be at increased risk of aortic dissection; however, cases of aortic rupture are extremely rare. There is lack of consensus on the exact site and size of aortic diameter measurement that increases this risk, and whether this applies to both Type A and Type B dissections. Case A 23-year-old G2P1001 with known Marfan syndrome who underwent an uncomplicated antepartum and intrapartum course. She experienced persistent backache 10 days postpartum that led to the diagnosis of Stanford Type B dissection. The patient was hospitalized for close observation. Dissection progressed to aortic rupture within 24 hours that required emergent thoracic endovascular aortic repair. She had an uncomplicated postoperative course. Conclusion Our report demonstrates rupture of a known aortic dissection within a very short time in the postpartum period. The case highlights the importance of patient education and close surveillance especially in the postpartum period. It also brings home the value of imaging of the whole aorta rather than focusing on the ascending alone. Multidisciplinary care and timely diagnosis and intervention likely led to the favorable outcome in our case.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Rupture of a Type B Aortic Dissection in a Postpartum Patient with Marfan Syndrome

Patberg

Duffy

Hameed

2019

AJP Rep

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“…They reported a 1‐year follow‐up without any complications apparent from this 53 . In some cases, CS was enough to then manage the mother's TBAD medically 56,62 . This decision is not without some risk; as stated by Yuan et al, 50 medical treatments can progress with further expansion of the distal segments in up to 40% of patients.…”

Section: Reviewmentioning

confidence: 99%

Pernicious pregnancy: Type B aortic dissection in pregnant women

et al. 2021

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Background Type B aortic dissection (TBAD) occurs seldomly, particularly in pregnancy, but has disastrous consequences for both mother and fetus. The focus of immediate surgical repair of type A aortic dissection due to higher mortality of patients is less clear in its counterpart, TBAD, in which management is controversial and debated. This article collates knowledge so far on this rare event during pregnancy. Methods A comprehensive literature search was performed in PubMed, Scopus, Google Scholar, Embase, and Medline. Key search terms included “type B aortic dissection,” “pregnancy,” and corresponding synonyms. Non‐English papers were excluded. Results Risk factors for TBAD include aortic wall stress due to hypertension, previous cardiac surgery, structural abnormalities (bicuspid aortic valve, aortic coarctation), and connective tissue disorders. In pregnancy, pre‐eclampsia is a cause of increased aortic wall stress. Management of this condition is often conservative, but this is dependent on a number of factors, including gestation, cardiovascular stability of the patient, and symptomology. In most cases, a cesarean section before intervention is carried out unless certain indications are present. Conclusions Due to a scarce number of cases across the decades, it is difficult to determine which management is optimal. The gold‐standard management of TBAD has traditionally been the medical treatment for uncomplicated cases and open surgery for those needing urgent intervention, but with the advent of techniques, such as thoracic endovascular aortic repair, the management of these group of patients continues to develop.

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