Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome

Cauldwell, Matthew; Steer, Philip; Curtis, Stephanie; Mohan, Aarthi R.; Dockree, Samuel; Mackillop, Lucy; Parry, Helen; Oliver, James; Sterrenberg, Monique; Wallace, Suzanne; Malin, GL; Partridge, Gemma; Freeman, Leisa J; Bolger, Aidan P.; Siddiqui, Farah; Wilson, Dirk G.; Simpson, Margaret; Walker, Niki L.; Hodson, Kenneth; Thomas, K.; Bredaki, Foteini E.; Mercaldi, Rebecca; Walker, Fiona; Johnson, Mark R.

doi:10.1136/heartjnl-2019-314817

Cited by 34 publications

(46 citation statements)

References 26 publications

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“…Similar to the findings of Cauldwell et al (2019), we reported no maternal deaths. Other studies on MFS and pregnancy have found higher risk of adverse cardiovascular events, particularly aortic dissection (Cauldwell et al, 2019; Curry et al, 2014), accelerated aortic dilation, and progressive aortic and mitral regurgitation (Donnelly, Pinto, Kocolas, & Yetman, 2012; Meijboom et al, 2005; Rossiter, Repke, Morales, Murphy, & Pyeritz, 1995). However, in our study, no adverse cardiovascular events occurred in women with MFS.…”

Section: Discussionsupporting

confidence: 92%

Maternal, pregnancy, and neonatal outcomes for women with Marfan syndrome

Ramage

Grabowska

Silversides

et al. 2020

Birth Defects Research

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Background: Marfan syndrome (MFS) is an autosomal dominant hereditary disorder which affects cardiovascular structure and function. With medical advances, more women with MFS experience pregnancy, which may increase maternal and neonatal risk. Existing research has been limited by small or clinical samples. This study examines the association of MFS and adverse maternal, neonatal, and obstetric outcomes. Methods: We conducted a cross-sectional study using the discharge abstract database, containing all labor and delivery hospitalizations in Canada (excluding Quebec) from fiscal years 2004-2015 where women delivered a live-or stillbirth. We measured maternal and neonatal morbidity, preterm births (<37 weeks), small-for-gestational-age births, perinatal mortality, and adverse maternal cardiovascular events. For each outcome, we calculated the absolute risk for women with and without MFS and used generalized estimating equations with a logit function to calculate odds. Results: Overall, 2,682,461 women delivered a live or stillborn infant in Canada during the study period, with 135 birth events to women with MFS. Women with MFS did not have significantly higher odds of severe maternal morbidity during their delivery (aOR:1.3; 95%CI: 0.4-4.0). Similarly, their infants did not have significantly higher odds of neonatal morbidity. However, infants born to women with MFS were significantly more likely to be born preterm (aOR:2.6; 95%CI: 1.6-4.3) and to be small-for-gestational-age (aOR:1.8; 95%CI:1.0-3.1). Conclusions: This population-based study indicates that, although some women with MFS may experience higher odds of maternal and/or neonatal morbidity during labor and delivery, the majority of women with MFS can have healthy births with proper clinical management.

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Section: Discussionsupporting

confidence: 92%

Maternal, pregnancy, and neonatal outcomes for women with Marfan syndrome

Ramage

Grabowska

Silversides

et al. 2020

Birth Defects Research

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“…One patient in early gestation (12 weeks) was recommended termination of pregnancy; she had MFS and a prior AVR. No information was available on this case, but she went on to have medical treatment only 59 . This supports the consensus of delivering where possible and subject to fetal viability.…”

Section: Reviewsupporting

confidence: 53%

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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“…A recent study comprising of 12 UK centers, over the past 20 years shows that the rate of aortic dissection in pregnant women with MFS was 1.9% (one type A and four type B) and there were no deaths. It also reports that preconception counseling rates were low [4]. The patient in our case did not undergo pre-conception counseling.…”

Section: Discussionmentioning

confidence: 53%

Application of cook balloon during aorta replacement in a pregnant Marfan-syndrome patient: a case report

Zhang

Yan

Xue

et al. 2020

BMC Pregnancy Childbirth

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Background: Aortic dissection is a rare and emergent condition. Aortic dissection during pregnancy is not much known but it is quite lethal to both mother and infant. Earlier reports published show that clinicians conducted hysterectomies during cesarean section to avoid anticoagulant-induced uterine bleeding during the following aortic surgery. Case presentation: A woman (38, gravida 1, para 0) in the 37th gestational week suffered an acute, severe, sharp pain in the chest and back. She was diagnosed with Standford type A aortic dissection and suspected with Marfan syndrome. An emergency cesarean section was performed immediately to deliver the baby. Since the patient was on anticoagulants during aortic replacement, so Cook balloon was inserted into the uterus to prevent postpartum hemorrhage. This helped to maintain the uterus intact. Family genetic testing showed that the patient was a carrier of FBN1 mutation which was inherited from the patient's mother, and the newborn also carried the mutation. Hence the patient was concluded to be positive for Marfan syndrome. Conclusion: It is important that clinicians should pay attention to the possibility of aortic dissection in a pregnant woman with chest, abdominal or back pain. In this case study, we employed Cook balloon during cesarean section to avoid anticoagulant-induced uterine bleeding during the following aortic surgery.

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Maternal and fetal outcomes in pregnancies complicated by Marfan syndrome

Cited by 34 publications

References 26 publications

Maternal, pregnancy, and neonatal outcomes for women with Marfan syndrome

Maternal, pregnancy, and neonatal outcomes for women with Marfan syndrome

Pernicious pregnancy: Type B aortic dissection in pregnant women

Application of cook balloon during aorta replacement in a pregnant Marfan-syndrome patient: a case report

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