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Pregnancy and Marfan syndrome

Pregnancy and Marfan syndrome
Authors:
Michael J Wright, MBChB, MSc
Heidi M Connolly, MD, FACC, FASE
Section Editors:
Charles J Lockwood, MD, MHCM
Harry C Dietz, MD
Deputy Editor:
Susan B Yeon, MD, JD, FACC
Literature review current through: Nov 2022. | This topic last updated: Jan 27, 2021.

INTRODUCTION — The Marfan syndrome (MFS, MIM #154700) is an autosomal dominant condition with a reported incidence of 1 in 3000 to 5000 individuals. There is a wide range of clinical severity associated with MFS. Although many clinicians view the disorder in terms of classic ocular, cardiovascular, and musculoskeletal abnormalities, these patients also demonstrate significant involvement of the lungs, skin, and central nervous system.

Pregnancy and the postpartum period is a high-risk time for aortic dissection and rupture in patients with MFS. The increased risk may be due to increased arterial wall stress associated with the hypervolemic and hyperdynamic circulatory state and/or hormonal effects on aortic wall composition [1,2]. Aortic root dilatation can also lead to worsening aortic regurgitation.

The management of pregnancy in patients with MFS and related disorders will be reviewed here. The genetics, pathogenesis, clinical manifestations, diagnosis, and management of MFS and related disorders are discussed separately. The management of pregnancy in patients with bicuspid aortic valve is also discussed separately. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders" and "Management of Marfan syndrome and related disorders" and "Bicuspid aortic valve: Management during pregnancy".)

ROLE OF PREGNANCY HEART TEAM — Preconception care for patients with MFS includes assessment of maternal, fetal, and neonatal risk and counseling by a multidisciplinary pregnancy heart team, which includes a cardiologist, maternal-fetal medicine specialist/obstetrician, anesthesiologist, and geneticist [3]. The assessment includes aortic and cardiac imaging and may also include prenatal genetic testing, after discussion of the potential benefits and limitations of such testing. (See 'Evaluation of risk' below and 'Preconception counseling and management' below.)

If a pregnant patient with MFS has not received preconception evaluation and counseling, an assessment of risks and counseling should be undertaken by the pregnancy heart team as soon as the patient presents for care. Lack of knowledge of the diagnosis of MFS prior to pregnancy is a risk factor for adverse pregnancy outcome [4].

EVALUATION OF RISK

Testing — Assessment of risks which may be encountered during pregnancy includes preconception (or earliest possible) cardiovascular imaging and discussion of and possible performance of prenatal genetic testing.

Role of cardiovascular imaging — Any patient with Marfan syndrome (MFS) who is contemplating pregnancy should undergo the following imaging:

A screening transthoracic echocardiogram for assessment of aortic root, ascending aorta dimensions, and assessment of possible associated valve or myocardial disease.

Preconception imaging of the entire aorta by computed tomography (CT) or magnetic resonance (MR) imaging. For pregnant patients with MFS who have not undergone preconception evaluation, MR without gadolinium contrast is generally the preferred modality for imaging of the entire aorta during pregnancy. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Imaging diagnosis' and "Echocardiographic evaluation of the thoracic and proximal abdominal aorta", section on 'Two-dimensional echocardiography' and "Diagnostic imaging in pregnant and nursing patients".)

Follow-up cardiovascular imaging during pregnancy is discussed below. (See 'Monitoring' below.)

Prenatal genetic testing — The availability and limitations of prenatal diagnostic testing should be discussed, ideally prior to conception. Prenatal diagnostic testing is feasible if a disease-causing mutation has been identified in the family. However, the severity of disease in a child who inherits a mutation in the FBN1 gene is unpredictable. Analysis of the FBN1 gene from cells or tissue obtained during preimplantation genetic diagnosis (PGD) using in vitro fertilization techniques, amniocentesis, or chorionic villus sampling can also be informative. However, such analyses are time consuming, costly (generally not covered by insurance carriers), and carry a risk to the fetus. Planning for them should ideally be done prior to conception. Resources for genetic testing are discussed in more detail elsewhere. (See "Genetic testing".)

Maternal risks — Pregnant and postpartum patients with MFS with aortic root dilatation are at increased risk for aortic dissection and/or rupture, although a normal dimension does not exclude the possibility of dissection [2,5,6]. The risk of aortic rupture or dissection during pregnancy and postpartum is difficult to quantify, since limited data are available. Complications can occur at any time during pregnancy, but are most often seen after the second trimester or during the postpartum period [2].

Cardiovascular complications

Aortic complications — In a patient with MFS, the risk of aortic dissection associated with pregnancy has been estimated as approximately 3 percent; the risk is higher with greater aortic root size but there is some risk even with an aortic root diameter <40 mm. The risk of dissection or other serious complications such as endocarditis or heart failure has been estimated to be approximately 1 percent in patients with MFS with an aortic root diameter ≤40 mm [5,6]. In a review of 36 cases of aortic dissection associated with pregnancy, 8 cases occurred with an aortic root diameter ≤40 mm [7]. Patients with MFS with an aortic root diameter >40 mm and/or rapidly increasing aortic root size (>5 mm per year) are at greater risk of dissection and adverse cardiovascular outcomes [4,8]. Patients with a previous aortic dissection are considered at high risk for complications during pregnancy, but there are insufficient data to quantify the risk. One series included two patients with a previous aortic dissection, one of whom developed another dissection during pregnancy despite having undergone aortic root replacement after the first event [9].

In addition to the risk of complications during pregnancy, limited data suggest that pregnancy may increase the long-term rate of aortic dilatation in patients with MFS. One small study of women with MFS found a higher rate of aortic root growth in those who experienced pregnancy compared with controls that had not been pregnant (0.36 versus 0.14 mm per year) only among those with baseline aortic diameter ≥40 mm [9]. In a larger study of women with MFS, aortic root size was significantly larger among women who had experienced pregnancy compared with controls who had not been pregnant, though baseline aortic root sizes were similar [8]. In addition, in a subset of women with echocardiograms prior to, during, and after pregnancy, the rate of aortic root growth increased significantly during pregnancy and declined following delivery though remaining above the baseline rate.

Other cardiovascular complications — Patients with MFS are at risk for additional cardiovascular disorders which may pose risks during pregnancy, including mitral regurgitation due to mitral valve prolapse, new or worsening arrhythmia, and heart failure due to ventricular dysfunction (such as may be associated with valve disease). Assessment and management of these conditions during pregnancy are discussed separately. (See "Pregnancy and valve disease" and "Supraventricular arrhythmias during pregnancy" and "Ventricular arrhythmias during pregnancy".)

Obstetric complications — Patients with MFS are at risk for obstetric complications. As an example, in a series comparing 29 pregnancies in 21 patients with MFS with pregnancies in healthy controls between 1995 and 2010, the risk of obstetric complications was high (OR 3.29, 95% CI 1.30 to 8.34), with a particularly high risk of postpartum hemorrhage (OR 8.46, 95% CI 2.52 to 28.38) [10].

Offspring risks — The risk of pregnancy in a patient with MFS includes fetal and neonatal risks. There is also a risk of transmission from an affected parent to her or his child. (See 'General considerations' below and "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)

Limited data are available on fetal and neonatal complications associated with pregnancy in patients with MFS. In the above referenced series of 29 pregnancies in 21 patients with MFS, babies born to mothers with MFS were delivered significantly earlier than those born to the control group (median 39 versus 40 weeks of gestation) and were significantly more likely to be small for gestational age (24% in the MFS group versus 6% in the controls) [10].

PRECONCEPTION COUNSELING AND MANAGEMENT

General considerations — Pregnancy-related risks of MFS include maternal risks and offspring risks:

Patients with MFS who are considering pregnancy should receive counseling regarding maternal and offspring risks (as described above) by the pregnancy heart team [3]. These patients require specialized management prior to, during, and following pregnancy, including delivery and the postpartum period.

In addition, patients with MFS should receive counseling regarding the risk of transmission (generally autosomal dominant). The 50 percent chance of transmission from an affected parent to their child, phenotypic variability, and the availability of prenatal diagnostic testing should be discussed. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)

Discussion of options should include potential alternatives to pregnancy (childlessness, adoption, and use of a gestational carrier [who carries a pregnancy with an embryo conceived using egg and sperm of the prospective parents] or gestational surrogate [who carries a pregnancy with an embryo conceived using the surrogate's egg]).

Management of high risk features — Additional considerations apply for patients with high risk features such as dilated ascending aorta, aortic dissection, or severe valve disease.

Dilated ascending aorta — As noted above, patients with MFS and a dilated aorta are at increased risk for complications during or following pregnancy; options include preconception elective aorta repair or forgoing pregnancy.

For patients with MFS with a maximal aortic root/ascending aorta diameter ≥45 mm (or >27 mm/m2), we suggest elective repair prior to conception. This recommendation is consistent with the 2010 Canadian guidelines [11], although limited supporting data are available. The 2010 American College of Cardiology/American Heart Association/American Association of Thoracic Surgeons (ACC/AHA/AATS) guidelines use a lower threshold for elective repair, suggesting that elective repair is reasonable if the aortic diameter exceeds 40 mm in patients with MFS who are contemplating pregnancy [12].

Although the risk of ascending aortic dissection is reduced with successful surgical correction, there is a risk of aortic dissection in the remaining aorta during subsequent pregnancies [5,9,13]. The available data are insufficient to estimate this risk.

Some experts advise against pregnancy in patients with MFS with a maximal aortic root/ascending aorta diameter >45 mm, and do not recommend preconception aorta repair. This is the approach taken in the 2018 European Society of Cardiology (ESC) guidelines for the management of cardiovascular diseases during pregnancy which do not include discussion of pre-pregnancy aorta surgery [3] (which had been included in the 2011 ESC guidelines).

Aortic dissection — For patients with aortic dissection, or history of aortic dissection, we advise against pregnancy, consistent with the 2018 ESC guidelines [3]. Management of aortic dissection during pregnancy is discussed below. (See 'Aortic dissection' below.)

Valve disease — Patients with MFS may have valve disease, primarily aortic regurgitation (generally associated with aortic dilation) or mitral valve prolapse, often accompanied by mitral regurgitation. The preconception counseling and management of these valve lesions prior to and during pregnancy is discussed separately. (See "Pregnancy and valve disease".)

MANAGEMENT DURING PREGNANCY — Among patients with Marfan syndrome (MFS) who become pregnant, a multidisciplinary approach to prenatal care is recommended, preferably at a center with experience in the management of patients with MFS [14].

Monitoring — Serial clinical assessment should include echocardiographic monitoring in all pregnant patients with MFS, even among patients with baseline aortic root diameter ≤40 mm, assuming the ascending aorta can be adequately visualized using echocardiography [2,5,6]. We suggest clinical and echocardiographic follow-up every 8 to 12 weeks if the aortic root diameter remains ≤40 mm, and we suggest follow-up every 4 to 8 weeks if the aortic root diameter is >40 mm. The frequency of clinical and imaging follow-up should be individualized depending on patient characteristics. The 2018 European Society of Cardiology (ESC) Guidelines suggest repeat echocardiographic imaging every 4 to 12 weeks during pregnancy and 6 months postpartum in patients with ascending aorta dilatation [15]. Similarly, the 2010 American College of Cardiology/American Heart Association/American Association of Thoracic Surgeons (ACC/AHA/AATS) guidelines recommend monthly or bimonthly echocardiographic measurement of the aortic root and ascending aortic dimensions in this setting [12].

When transthoracic echocardiography is not sufficient to image the ascending aorta, magnetic resonance (MR) imaging is recommended over computed tomography (CT) for pregnant patients with aortic dilatation, to avoid exposing the mother and fetus to ionizing radiation, as noted in the 2018 ESC and 2010 ACC/AHA/AATS guidelines. Also, in patients with MFS with aortic arch or descending aorta dilation not well seen by TTE, we suggest serial MR imaging without gadolinium during pregnancy. MR imaging without gadolinium contrast is preferred and is generally sufficient for monitoring aortic size [3]. Gadolinium should generally be avoided in the pregnant patient, unless the potential benefit is deemed to outweigh the potential risk to the fetus. (see "Diagnostic imaging in pregnant and nursing patients", section on 'Fetal risks from magnetic resonance imaging').

Transesophageal echocardiography is an alternative for imaging the thoracic aorta, but the distal ascending aorta and proximal aortic arch are generally not well visualized by this modality.

Aortic measurement methods are discussed separately. (See "Management of Marfan syndrome and related disorders", section on 'How to measure'.)

Medical therapy — For pregnant patients with MFS, we suggest beta blocker therapy. Labetalol or extended-release metoprolol are the preferred beta blockers in pregnant patients since atenolol may impair fetal growth (see "Treatment of hypertension in pregnant and postpartum patients", section on 'Beta blockers'). The dose of beta blocker is adjusted to maintain the heart rate after submaximal exercise (eg, running up and down two flights of stairs) <100 beats/minute.

The use of beta blocker therapy in pregnant patients with MFS is an extension of the indication for beta blocker therapy in patients with MFS with aortic dilation. Since pregnancy is associated with increased risk of aortic complications, we suggest beta blocker therapy during pregnancy in patients with or without aortic dilation in an attempt to minimize progressive aortic dilation and risk of aortic dissection. This approach is based largely on indirect evidence in nonpregnant patients with MFS. Limited evidence is available on the effects of beta blocker therapy in pregnant patients with MFS. Observational studies suggest that beta blocker therapy does not abolish the risk of progressive aortic dilation and aortic dissection during pregnancy [7]. (See "Management of Marfan syndrome and related disorders", section on 'Beta blocker'.)

Strict blood pressure control is recommended for all pregnant patients with MFS [12,15]. Dose adjustment of therapy for aortic preservation is suggested when the systolic blood pressure exceeds 130 mmHg. Angiotensin converting enzyme inhibitors and angiotensin II receptor blockers are teratogens and thus contraindicated during pregnancy. (See "Treatment of hypertension in pregnant and postpartum patients" and "Adverse effects of angiotensin converting enzyme inhibitors and receptor blockers in pregnancy".)

Antibiotic prophylaxis is optional at delivery (unless bacteremia is suspected) for patients with prior valve replacement surgery or prior endocarditis. (See "Prevention of endocarditis: Antibiotic prophylaxis and other measures".)

Prophylactic aortic intervention — Prophylactic intervention during pregnancy is reserved for selected patients with ascending aortic diameter that is >45 mm and increasing rapidly, as suggested in the 2018 ESC guidelines [3]. Decisions are highly complex and should be individualized based upon factors including the gestational age and rapidity of aortic size increase and involve weighing the maternal and fetal risks and benefits of prophylactic aortic surgery during pregnancy. Considerations include the potential maternal and fetal risks of aortic dissection and the risks and benefits of surgery. (See "Anesthesia for nonobstetric surgery during pregnancy".)

Aortic dissection — The diagnosis and management of aortic dissection that occurs during pregnancy or the postpartum period are similar to that for aortic dissection in general.

Given the lethal nature of aortic dissection, prompt diagnosis and intervention are critical. A review including papers describing 36 cases of women with MFS with aortic dissection antepartum or postpartum, reported a 21 percent maternal mortality rate and 12 percent fetal mortality rate [7]. Confirming the diagnosis is the first objective and the availability, reliability, and speed of CT often make this the test of choice in pregnant patients presenting with a high clinical suspicion for aortic dissection. Patients presenting with an intermediate or low clinical suspicion of aortic dissection can be evaluated by other methods. Due to concerns about ionizing radiation (CT) during pregnancy, transesophageal echocardiogram or MR aortic imaging may be the initial diagnostic modalities, assuming immediate availability [16,17]. MR imaging without gadolinium contrast is preferred, although gadolinium may be appropriate if it enhances the clarity of the diagnosis. Limited data suggest that the risk to the fetus of gadolinium may be minimal [18]. (See "Diagnostic imaging in pregnant and nursing patients" and "Management of acute type B aortic dissection".)

Optimal treatment of aortic dissection during pregnancy includes a high-risk maternal-fetal medicine team and an aortic specialty team with medical, percutaneous, and surgical aortic treatment capabilities.

We agree with the following recommendations included in the 2010 ACC/AHA/AATS guidelines [12]:

For Type A (ascending aorta) dissection during the first or second trimester, urgent surgical repair with fetal monitoring when feasible is preferred. Fetal loss is common during hypothermia and prolonged cardiopulmonary bypass and there is an increased rate of teratogenesis [19]. (See "Anesthesia for nonobstetric surgery during pregnancy".)

For Type A dissection during the third trimester, urgent cesarean delivery followed by aortic repair is suggested.

For acute arch or Type B aortic dissection, medical therapy is preferred unless intervention (percutaneous stent or surgery) is required to treat malperfusion, aortic rupture, or subacute aortic leaking. Nonsurgical methods are generally preferred when Type B aortic dissection requires intervention during pregnancy, as they may be the safest approach for mother and fetus [20], but the approach should be individualized.

In patients with aortic dissection, pharmacologic interventions to reduce shear stress and blood pressure are needed but the administration of nitroprusside during pregnancy should be avoided when feasible in order to avoid possible fetal thiocyanate toxicity.

Valve intervention — Indications for and management of valve intervention during pregnancy are discussed separately. (See "Pregnancy and valve disease".)

DELIVERY — Given the risk of aortic dissection and other cardiovascular complications, pregnant patients with MFS with aortic root aneurysm (aortic root ≥40 mm) or history of aortic dissection should be delivered at an institution where cardiothoracic surgery is available, although it could be argued that this is a reasonable practice for all pregnant patients with MFS [12,15].

There is no strong consensus regarding the preferred mode of delivery for patients with MFS since scant evidence is available. The European Society of Cardiology guidelines suggest vaginal delivery in patients with MFS who have an ascending aorta diameter <40 mm and no other high-risk features, vaginal delivery utilizing epidural anesthesia with either an expedited second stage or delayed pushing to minimize Valsalva in patients with MFS who have an ascending aorta diameter ≥40 mm and ≤45 mm (though some prefer cesarean section in this setting), and cesarean delivery in patients with MFS who have an ascending aorta diameter >45 mm [15]. We do not believe that there is sufficient information to endorse any general recommendations and that the method of delivery should be carefully considered and individualized. More specifically, general obstetric principles should be applied to select the least hemodynamically stressful mode of delivery in the context of the particular pregnancy at hand unless there is an overriding specific obstetric indication for cesarean delivery. This approach is in keeping with the position of the Professional Advisory Board of the Marfan Foundation [21].

With vaginal delivery, epidural anesthesia to minimize pain, outlet forceps or vacuum delivery without maternal pushing, and continued use of a beta blocker during labor are appropriate ancillary measures to minimize surges in blood pressure and cardiac output. In patients assessed to be at high risk for labor complications that may induce hemodynamic stress, a planned cesarean delivery may be preferred. (See "Anesthesia for labor and delivery in high-risk heart disease: General considerations".)

As noted below, Type A aortic dissection is an indication for urgent cesarean delivery if the fetus is viable, followed by or with simultaneous aortic repair. (See 'Aortic dissection' above.)

Prior to attempting epidural anesthesia, the presence of dural ectasia should be considered [22]. Dural sac dilation can be present in up to 90 percent of patients with MFS and may be asymptomatic or associated with low back pain, headache, or proximal leg pain, weakness, or numbness. Dural ectasia can be visualized by lumbosacral MR imaging or CT studies (image 1), generally performed prior to conception as part of comprehensive aortic imaging. Although dural ectasia is not an absolute contraindication for epidural anesthesia, the increased risk of dural puncture or inadequate anesthesia should be discussed with the patient. It has been recommended that the epidural technique be performed in the lateral decubitus position to decrease the possibility of extension of the ectatic dural sac into the epidural space [23]. It is important to recognize that an increased amount of cerebrospinal fluid volume is generally present with dural ectasia, which may lead to spinal anesthesia failure [24]. Consultation with an experienced obstetrical anesthesiologist is recommended prior to delivery to determine the type of anesthetic and management strategy. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders", section on 'Dural ectasia'.)

Obstetric complications appear to be increased in patients with MFS. This was illustrated in a retrospective multicenter study of 111 completed pregnancies among 63 patients with MFS [25]. There was a high rate of premature deliveries (15 percent), mainly due to preterm premature rupture of membranes and cervical insufficiency, which resulted in a markedly increased combined rate of fetal and neonatal mortality (7 percent; 5 percent accounted for by preterm premature rupture of membranes). For comparison, in the United States general population the preterm birth rate is approximately 12 percent, neonatal mortality is approximately 0.4 percent and fetal mortality is approximately 0.7 percent [26]. Postpartum hemorrhage and uterine inversion have also been reported [27].

POSTPARTUM — Due to the increased risk of aortic dissection postpartum, we suggest monitoring patients with MFS for complications during the first four to six weeks postpartum. Postpartum follow-up should be individualized and may range from one visit for low-risk patients to weekly follow-up with imaging during the first few weeks for high-risk patients. Patients should be alerted about the increased risk of aortic dissection postpartum and encouraged to present promptly for evaluation and imaging should symptoms of persistent chest or back discomfort occur.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Aortic dissection and other acute aortic syndromes" and "Society guideline links: Marfan syndrome" and "Society guideline links: Management of cardiovascular diseases during pregnancy".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topic (see "Patient education: Marfan syndrome (The Basics)")

SUMMARY AND RECOMMENDATIONS

Preconception care for patients with Marfan syndrome (MFS) includes assessment of maternal, fetal, and neonatal risk and counseling by a multidisciplinary pregnancy heart team, which includes a cardiologist, maternal-fetal medicine specialist/obstetrician, anesthesiologist, and geneticist. Assessment includes aortic and cardiac imaging and may also include prenatal genetic testing, after discussion of the potential benefits and limitations of such testing. (See 'Role of pregnancy heart team' above.)

If a pregnant patient with MFS has not received preconception evaluation and counseling, an assessment of risks and counseling should be undertaken by the pregnancy heart team as soon as the patient presents for care.

Any patient with MFS who is contemplating pregnancy should have a screening transthoracic echocardiogram for assessment of aortic root and ascending aorta dimensions and imaging of the entire aorta by computed tomography (CT) or magnetic resonance (MR) imaging. For pregnant patients with MFS who have not undergone preconception evaluation, MR without gadolinium contrast is the preferred modality for imaging of the entire aorta during pregnancy. (See 'Evaluation of risk' above and 'Preconception counseling and management' above.)

A multidisciplinary approach at a center with experience in management of MFS and access to maternal fetal medicine, specialized imaging, and cardiac/aortic surgical intervention is recommended for all patients with MFS who become pregnant. (See 'Management during pregnancy' above.)

Among patients with MFS, an aortic root diameter >40 mm identifies a group with increased risk of aortic dissection and other complications during and after pregnancy. (See 'Evaluation of risk' above.)

For patients with MFS with an aortic root diameter ≥45 mm, we suggest elective repair prior to conception (Grade 2C). Limited supporting data are available and there is a risk of aortic dissection in the remaining aorta during subsequent pregnancies. (See 'Management of high risk features' above.)

All pregnant patients with MFS require regular cardiovascular follow-up during pregnancy with the frequency of follow-up and imaging individualized. (See 'Monitoring' above.)

For pregnant patients with MFS, we suggest beta blocker therapy (Grade 2C). We prefer labetalol or metoprolol during pregnancy. The goal of beta blocker therapy is to minimize progressive aortic dilation and the risk of aortic dissection, although limited supporting data are available in this setting. (See 'Medical therapy' above.)

Strict blood pressure control is recommended for all pregnant patients with MFS. (See 'Medical therapy' above.)

The method of delivery should be carefully considered and individualized for patients with MFS by a multidisciplinary care team. Ideally, all MFS patients should deliver at a center that has emergency cardiac surgery services. (See 'Delivery' above.)

For Type A (ascending aorta) dissection during the first or second trimester, urgent surgical repair with fetal monitoring when feasible is preferred. (See 'Aortic dissection' above.)

For Type A dissection during the third trimester, urgent cesarean delivery followed by aortic repair is suggested. (See 'Aortic dissection' above.)

Intervention for Type B aortic dissection is occasionally needed during pregnancy; nonsurgical methods may be the safest approach for mother and fetus, but the decision regarding approach should be individualized. (See 'Aortic dissection' above.)

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