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Heritable thoracic aortic diseases: Preconception risk assessment and management

Heritable thoracic aortic diseases: Preconception risk assessment and management
Literature review current through: May 2024.
This topic last updated: Sep 15, 2023.

INTRODUCTION — Pregnancy in individuals at risk for thoracic aortic aneurysm or dissection is associated with elevated maternal and offspring risk.

Causes of thoracic aortic aneurysm include hereditable thoracic aortic diseases (HTADs), congenital conditions (including bicuspid aortic valve, Turner syndrome, coarctation of the aorta, and tetralogy of Fallot), hypertension, atherosclerosis, degenerative aortic disease, aortitis (inflammatory or infectious), and traumatic aortic injury. (See "Epidemiology, risk factors, pathogenesis, and natural history of thoracic aortic aneurysm and dissection".)

HTADs include [1]:

Syndromic disorders – These include Marfan syndrome [MFS], Loeys-Dietz syndrome [LDS], vascular Ehlers-Danlos syndrome, smooth muscle dysfunction syndrome, and other syndromes attributable to pathogenic variants in FLNA, BGN, or LOX.

and

Nonsyndromic HTADs (nsHTADs) – These include pathogenic variants in ACTA2, MYH11, PRKG1, MYLK, and familial thoracic aortic aneurysm without identified pathogenic variants in a known gene for HTAD.

Preconception risk assessment and preconception management in individuals with MFS, LDS, and nsHTAD is reviewed here. For pregnant individuals with these conditions who have not received preconception care, risk assessment and counseling are provided as soon as the patient presents for care.

Related issues are discussed separately:

Management during pregnancy and postpartum care for individuals with HTAD is discussed separately. (See "Heritable thoracic aortic diseases: Pregnancy and postpartum care".)

Management of pregnancy in individuals with vascular Ehlers-Danlos syndrome is discussed separately. (See "Overview of the management of Ehlers-Danlos syndromes".)

Management of pregnancy in individuals with bicuspid aortic valve-associated ascending aortic aneurysm is discussed separately. (See "Bicuspid aortic valve: Preconception and pregnancy care".)

Management of pregnancy in patients with coarctation of the aorta is discussed separately. (See "Management of coarctation of the aorta", section on 'Pregnancy'.)

Management of Turner syndrome in adults is discussed separately. (See "Management of Turner syndrome in adults".)

The genetics, pathogenesis, clinical manifestations, diagnosis, and management of MFS and related disorders are discussed separately. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders" and "Management of Marfan syndrome and related disorders".)

ROLE OF PREGNANCY HEART TEAM — Since individuals with HTAD are at moderate or high risk of complications during or following pregnancy, they should receive specialized evaluation, counseling, and management by a multidisciplinary pregnancy heart team prior to, during, and following pregnancy, including delivery and the postpartum period [2]. The pregnancy heart team should include, at a minimum, a cardiologist, maternal-fetal medicine specialist/obstetrician, cardiovascular surgeon, anesthesiologist, and provider with expertise in genetics (genetic counselor or geneticist) [1-3].

RISKS ASSOCIATED WITH PREGNANCY

Maternal risks — Pregnant patients with HTAD are at risk for cardiovascular (particularly aortic) complications and obstetric complications.

Cardiovascular complications

Aortic complications — Patients with HTAD are at increased risk for aortic aneurysm, dissection, and rupture during pregnancy and the postpartum period. Aortic root dilation can also lead to worsening aortic regurgitation. Complications can occur at any time during or after pregnancy, but are most often seen during the third trimester or postpartum period [4-8].

The association between pregnancy-related aortic dissection and HTAD was illustrated by a study of 29 individuals with aortic dissection during or within 12 weeks after pregnancy reported in the International Registry of Acute Aortic Dissection (IRAD) from 1998 to 2018 [6]. Twenty of these individuals (69 percent) had HTAD or a family history of disease of the aorta: 13 with MFS, two with LDS, two with bicuspid aortic valves, two with family history of aortic disease, and one with familial thoracic aortic aneurysm [6]. In 47 percent of cases, aortopathy was diagnosed only after aortic dissection.

Most data available about pregnancy-related aortic dissection with HTAD comes from individuals with MFS. There are few data on pregnancy for other HTADs, so the risk of aortic rupture or dissection during pregnancy and postpartum in individuals with these disorders is difficult to quantify.

MFS – For individuals with MFS, the risk of aortic dissection associated with pregnancy has been estimated as approximately 2 to 4 percent [4,9-13]. In a report from the GenTAC registry of 227 pregnancies in 94 individuals with MFS, the rate of aortic dissection (type A or B) was higher during pregnancy and the postpartum period than during nonpregnancy time periods (5.4 versus 0.6 per 100 person-years; rate ratio 8.4, 95% CI 3.9-18.4) [3].

ACTA2 – While there is much less information for nonsyndromic HTAD, a retrospective study examined the risk associated with 137 pregnancies in 53 individuals with ACTA2, a gene associated with thoracic aortic aneurysm and aortic dissection [14]. Eight patients (6 percent of pregnancies; 20 percent of patients) experienced an acute aortic dissection during the third trimester or postpartum period. One patient had a myocardial infarction prior to aortic dissection. Of note, none of the patients with aortic dissection were diagnosed with ACTA2 mutation prior to their dissections, despite a family history of dissection in six patients.

Risk factors for aortic complications — Data on risk factors for aortic complications come largely from studies of individuals with MFS:

Aortic size

Type A dissection – Individuals with MFS with an aortic root diameter >40 mm (particularly >45 mm) and/or rapidly increasing aortic root size (≥3 mm per year) are at greater risk of ascending aortic (type A) dissection and adverse cardiovascular outcomes, but there is some risk of dissection even with an aortic root diameter <40 mm [1,3,6,13,15-17]. In a review of 36 cases of aortic dissection associated with pregnancy in individuals with MFS, eight cases occurred with an aortic root diameter ≤40 mm [17]. The risk of dissection or other serious complications such as endocarditis or heart failure during pregnancy has been estimated to be approximately 1 percent in patients with MFS with an aortic root diameter ≤40 mm [9,10].

Type B dissection – Unlike type A dissections, type B aortic dissections are often not associated with significant thoracic aortic dilation, and no predictive factors have been identified to date [6].

Prior aortic dissection or aortic root replacement – Patients with a prior aortic dissection or prophylactic aortic root replacement are considered at high risk for aortic dissection during pregnancy, but there are insufficient data to quantify the risk since only case reports are available in this population of patients [18-20].

Long-term effects of pregnancy – In addition to the risk of complications during pregnancy, limited data suggest that pregnancy may increase the long-term rate of aortic dilation in patients with MFS. One small study of females 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 [18]. In a larger study of females with MFS, aortic root size was significantly larger among those who had experienced pregnancy compared with controls who had not been pregnant, although baseline aortic root sizes were similar [15]. In addition, in a subset of females 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.

The increased risk of aortic dilation and aortic complications during pregnancy may be due to increased arterial wall stress associated with the hypervolemic and hyperdynamic circulatory state and/or hormonal effects on aortic wall composition [4,21].

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). The 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" and "Management of heart failure during pregnancy".)

Obstetric complications — Patients with HTAD are at risk for obstetric complications including postpartum hemorrhage, though only limited data are available, as illustrated by the following examples [22].

MFS – 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 (34 versus 14 percent; odds ratio [OR] 3.29, 95% CI 1.30-8.34), with a markedly elevated risk of postpartum hemorrhage (28 versus 4 percent; OR 8.46, 95% CI 2.52-28.38) [23].

LDS – In a study of 20 pregnancies in 13 individuals with LDS, postpartum hemorrhage occurred in 33 percent of 18 live births [24].

Offspring risks — Pregnancy in patients with HTAD is associated with fetal and neonatal risks. There is also a risk of transmission from affected parent to child. HTADs are generally autosomal dominant conditions with a 50 percent risk of transmission to offspring, with the potential for intrafamilial phenotypic variability. (See 'Counseling' below and "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)

MFS – Limited data are available on fetal and neonatal complications associated with pregnancy in patients with HTAD. A literature review of case reports and series published between 2005 and 2015 included 1112 pregnancies in 852 patients with MFS [16]. The fetal mortality rate was 5.6 percent and 41 percent of births were cesarean deliveries.

In a retrospective multicenter study of 111 completed pregnancies among 63 patients with MFS, there was a high rate of premature deliveries (15 percent), mainly due to preterm prelabor 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 prelabor rupture of membranes) [25].

In the above-referenced series of 29 pregnancies in 21 patients with MFS, babies born to mothers with MFS were delivered 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 percent in the MFS group versus 6 percent in the controls) [23]. Similar findings were observed in a population-based cohort [26].

LDS – In the above-cited study of 18 live births from 20 pregnancies in 13 individuals with LDS, 50 percent of deliveries were preterm and 78 percent were elective cesarean deliveries [24].

Case reports have described prenatal diagnosis of aortic dilation or congenital heart disease as early manifestations of LDS [27-30]. (See "Management of Marfan syndrome and related disorders".)

GENERAL PRECONCEPTION CARE — Preconception care for patients with HTAD includes assessment of maternal, fetal, and neonatal risk and counseling by a multidisciplinary pregnancy heart team [1-3]. If a pregnant patient with HTAD has not received preconception evaluation and counseling, an assessment of risk and counseling should be undertaken by the pregnancy heart team as soon as the patient presents for care.

Risk assessment — A key task of preconception care is the identification of conditions such as HTAD associated with increased risk during pregnancy. Most individuals with aortic dissection related to pregnancy have an underlying aortopathy (heritable or nonheritable) but the aortopathy is commonly not recognized until after aortic dissection [6]. Lack of knowledge of the diagnosis of HTAD prior to pregnancy is a risk factor for adverse pregnancy outcomes [3,13]. (See "The preconception office visit".)

Preconception (or earliest possible) assessment of risk in an individual with HTAD includes a history (including family history of aortic dissection or sudden death attributable to aortic disease) and cardiovascular imaging and may also include prenatal genetic testing, after discussion of the goals as well as potential benefits, limitations, and cost of such testing. (See "Genetic counseling: Family history interpretation and risk assessment" and "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders", section on 'Role of genetic testing' and "Genetic testing".)

Baseline cardiovascular imaging — Individuals with HTAD who are contemplating pregnancy should undergo the following imaging prior to conception (or if not obtained preconception, at the earliest possible time during pregnancy).

Transthoracic echocardiogram – A screening transthoracic echocardiogram is performed for assessment of aortic root, ascending aorta dimensions, and assessment of possible associated valve or myocardial disease. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders", section on 'Diagnosis of MFS'.)

Additional imaging – Imaging to assess the vascular system from head through pelvis by computed tomography or magnetic resonance imaging (MRI) should be performed if such imaging has not been completed within the year prior to the time that the patient is contemplating pregnancy for patients with LDS and select patients with MFS. For pregnant patients who have not undergone preconception evaluation and thus require baseline imaging during pregnancy, MRI without gadolinium contrast is generally the preferred modality for elective 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 lactating patients".)

Follow-up cardiovascular imaging during pregnancy is discussed below. (See "Heritable thoracic aortic diseases: Pregnancy and postpartum care", section on 'Monitoring during pregnancy'.)

Prenatal genetic testing — Discussion of the availability and limitations of prenatal diagnostic testing (of an embryo or fetus) should be included in preconception counseling. Prenatal diagnostic testing is feasible if a pathogenic variant has been identified in the family.

However, the severity of disease in a child who inherits a pathogenic variant (eg, in the FBN1 gene) is somewhat unpredictable; on average the phenotype of an affected child broadly reflects the severity of disease seen in the affected parent, but extreme intrafamilial clinical variability may occur. Analysis of the gene of interest from cells or tissue obtained during preimplantation genetic testing (PGT) using in vitro fertilization techniques, or with chorionic villus sampling during the first trimester of pregnancy or amniocentesis during the second trimester, can be informative. However, such analyses are time consuming, costly (generally not covered by insurance carriers), and carry a very low but measurable risk to the embryo or fetus. Resources for genetic testing are discussed in more detail elsewhere. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders", section on 'Role of genetic testing' and "Genetic testing".)

Counseling — Preconception (or earliest possible) counseling by the pregnancy heart team should include discussion of maternal and offspring risks and management options [1,2]. (See 'Maternal risks' above and 'Offspring risks' above.)

This counseling should also include discussion of the risk of transmission from an affected parent to a child, phenotypic variability, and the availability of PGT or prenatal diagnostic testing [1]. (See "Genetics, clinical features, and diagnosis of Marfan syndrome and related disorders".)

Preconception discussion of options for managing the above risks should include potential alternatives to pregnancy including childlessness, adoption, use of a gestational carrier (who carries a pregnancy with an embryo conceived using egg and sperm of the prospective parents), and gestational surrogate (who carries a pregnancy with an embryo conceived using the surrogate's egg).

PRECONCEPTION 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.

Aortic dilation — As noted above, patients with HTAD, particularly those with aortic dilation or dissection, are at increased risk for aortic complications during or following pregnancy; options include preconception elective aorta repair or forgoing pregnancy. While the risk of ascending aortic dissection is reduced with prophylactic aortic root replacement, there is a risk of aortic dissection in the remaining aorta during subsequent pregnancies [9,18,20]. The available data are insufficient to estimate this risk.

The maximal aortic root/ascending aorta diameter thresholds for preconception intervention vary depending on the diagnosis (MFS, nonsyndromic HTAD [nsHTAD], or LDS) and genotype. Among patients with LDS, the threshold aortic diameter for repair is lower for TGFBR1, TGFBR2, or SMAD3 variants than for TGFB2- and TGFB3-related LDS variants because studies have suggested that patients with TGFBR1, TGFBR2, and SMAD3 variants have more severe aortopathy with aortic dissection risk at lower aortic diameters [6,31-34]. Based on the limited available evidence on aortic risk, patients with MFS and most patients with nsHTAD are managed similarly. The following recommendations for preconception aortic repair are similar to those in the 2022 American College of Cardiology/American Heart Association aortic guidelines [1].

Aortic root/ascending aorta diameter <40 mm

MFS, LDS, or genotypes of nsHTAD (other than ACTA2 and MYLK) – For patients with one of these diagnoses with a maximal aortic root/ascending aorta diameter <40 mm, there is no indication for elective preconception aortic repair.

Exception for nsHTAD with ACTA2 or MYLK – Aortic dissection associated with pregnancy has occurred at small aortic diameters (<40 mm) in individuals with nsHTAD with ACTA2 or MYLK pathogenic variants [35]. For individuals with nsHTAD with ACTA2 or MYLK pathogenic variants, a decision on whether to proceed with preconception prophylactic aortic surgery is based upon factors including the molecular diagnosis, family history, and aortic diameter growth rate.

Aortic root/ascending aorta diameter ≥40

MFS or nsHTAD and aortic diameter of 40 to 45 mm – For patients with either of these diagnoses with an aortic root or ascending aortic diameter of 40 to 45 mm, options include elective repair prior to conception, or proceeding with pregnancy without elective repair. The presence of one or more risk factors for aortic complications (such as rapid ascending aortic diameter growth of ≥3mm/year or family history of aortic dissection or sudden death attributable to aortic disease) favors aortic repair prior to conception.

Alternatively, some experts advise against pregnancy for patients with MFS or nsHTAD with an aortic diameter >40 mm and a family history of aortic dissection or sudden death attributable to aortic disease [2].

LDS with pathogenic variants in TGFBR1, TGFBR2, or SMAD3 and aortic diameter ≥40 mm – For patients with LDS with pathogenic variants in TGFBR1, TGFBR2, or SMAD3 with an aortic root/ascending aorta diameter ≥40 mm, we suggest aortic surgical repair prior to conception. Alternatively, some experts advise against pregnancy in this setting. There is insufficient information to provide a recommendation for individuals with LDS and a pathogenic variant in SMAD2.

LDS with aortic diameter >40 mm and family history of aortic dissection or sudden death – For patients with LDS, family history of dissection or sudden death, and aortic diameter >40 mm, some experts advise against pregnancy [2].

Aortic root/ascending aorta diameter ≥45 mm

MFS or nsHTAD and aortic diameter >45 mm – For patients with MFS or nsHTAD with a maximal aortic root/ascending aorta diameter >45 mm (or >27 mm/m2), we suggest elective aortic surgical repair prior to conception. Alternatively, some experts advise against pregnancy in patients with MFS or nsHTAD 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 [2]. Counseling regarding alternatives to pregnancy is discussed above. (See 'Counseling' above.)

LDS with pathogenic variants in TGFB2 or TGFB3 and aortic diameter ≥45 mm For patients with LDS with pathogenic variants in TGFB2 or TGFB3 with an aortic root/ascending aorta diameter ≥45 mm, we suggest aortic surgical repair prior to conception. Alternatively, some experts advise against pregnancy in this setting [2].

Aortic dissection — For patients with aortic dissection or prior history of dissection, we advise against pregnancy. This approach is consistent with the 2018 ESC pregnancy heart disease guidelines [2]. For patients who develop aortic dissection during pregnancy, management is discussed separately. (See "Heritable thoracic aortic diseases: Pregnancy and postpartum care", section on 'Management of aortic dissection'.)

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

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".)

SUMMARY AND RECOMMENDATIONS

Multidisciplinary care – Multidisciplinary preconception, pregnancy, and postpartum care for patients with heritable thoracic aortic disease (HTAD) should be provided by a pregnancy heart team at a center with experience in management of HTAD (multidisciplinary aortic team), and access to maternal fetal medicine, specialized imaging, and cardiac/aortic surgical intervention is recommended for all patients with HTAD. The pregnancy heart team should include, at a minimum, a cardiologist, maternal-fetal medicine specialist/obstetrician, anesthesiologist, cardiovascular surgeon, and provider with expertise in genetics (genetic counselor or geneticist). (See "Heritable thoracic aortic diseases: Pregnancy and postpartum care", section on 'Role of pregnancy heart team'.)

Preconception care – Preconception care for patients with Marfan syndrome (MFS) and other HTADs includes assessment of maternal, fetal, and neonatal risk and counseling by a multidisciplinary pregnancy heart team. (See 'Role of pregnancy heart team' above and 'Risks associated with pregnancy' above.)

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

Risk assessment Among patients with HTAD, an aortic root diameter >40 mm identifies a group with increased risk of aortic dissection and other complications during and after pregnancy. Additional risk factors include rapid ascending aortic diameter growth (≥3 mm/year) and family history of aortic dissection or sudden death attributable to aortic disease. (See 'Risk assessment' above.)

Preconception aortic imaging Any patient with HTAD 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 or magnetic resonance imaging (MRI). (See 'Baseline cardiovascular imaging' above.)

Preconception management – The threshold for prepregnancy intervention varies depending on the diagnosis, genotype, and clinical features. Limited supporting data are available, and there is a risk of aortic dissection in the remaining aorta during pregnancy and postpartum. (See 'Aortic dilation' above.)

The thresholds for preconception intervention and counseling based upon maximal aortic root/ascending aorta diameter vary depending on the diagnosis (MFS, nonsyndromic HTAD [nsHTAD], or LDS) and genotype.

Aortic root/ascending aorta diameter <40 mm

MFS, LDS, or genotypes of nsHTAD (other than ACTA2 and MYLK) – For patients with one of these diagnoses with a maximal aortic root/ascending aorta diameter <40 mm, there is no indication for elective preconception aortic repair.

Exception for nsHTAD with ACTA2 or MYLK – Aortic dissection associated with pregnancy has occurred at small aortic diameters (<40 mm) in individuals with nsHTAD with ACTA2 or MYLK pathogenic variants [35]. For individuals with nsHTAD with ACTA2 or MYLK pathogenic variants, a decision on whether to proceed with preconception prophylactic aortic surgery is based upon factors including the molecular diagnosis, family history, and aortic diameter growth rate.

Aortic root/ascending aorta diameter ≥40

MFS or nsHTAD with aortic diameter of 40 to 45 mm – For patients with either of these diagnoses with an aortic root or ascending aortic diameter of 40 to 45 mm, options include elective repair prior to conception, or proceeding with pregnancy without elective repair. The presence of one or more risk factors for aortic complications (such as rapid ascending aortic diameter growth of ≥3mm/year or family history of aortic dissection or sudden death attributable to aortic disease) favors aortic repair prior to conception.

Alternatively, some experts advise against pregnancy for patients with MFS or nsHTAD with an aortic diameter >40 mm and a family history of aortic dissection or sudden death attributable to aortic disease [2].

LDS with pathogenic variants in TGFBR1, TGFBR2, or SMAD3 with aortic diameter ≥40 mm – For patients with LDS with pathogenic variants in TGFBR1, TGFBR2, or SMAD3 with an aortic root/ascending aorta diameter ≥40 mm, we suggest aortic surgical repair prior to conception (Grade 2C). Alternatively, some experts advise against pregnancy in this setting. There is insufficient information to provide a recommendation for individuals with LDS and a pathogenic variant in SMAD2.

LDS with aortic diameter >40 mm and family history of aortic dissection or sudden death – For patients with LDS, family history of dissection or sudden death, and aortic diameter >40 mm, some experts advise against pregnancy.

Aortic root/ascending aorta diameter ≥45 mm

MFS or nsHTAD with aortic diameter >45 mm – For patients with MFS or nsHTAD with a maximal aortic root/ascending aorta diameter >45 mm (or >27 mm/m2), we suggest elective aortic surgical repair prior to conception (Grade 2C). Alternatively, some experts advise against pregnancy in patients with MFS or nsHTAD 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 [2]. Counseling regarding alternatives to pregnancy is discussed above. (See 'Counseling' above.)

LDS with pathogenic variants in TGFB2 or TGFB3 with aortic diameter ≥45 mm For patients with LDS with pathogenic variants in TGFB2 or TGFB3 with an aortic root/ascending aorta diameter ≥45 mm, we suggest aortic surgical repair prior to conception (Grade 2C). Alternatively, some experts advise against pregnancy in this setting.

Aortic dissection – For patients with aortic dissection or prior history of aortic dissection, we advise against pregnancy. (See 'Counseling' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Michael J Wright, MBChB, MSc, who contributed to earlier versions of this topic review.

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Topic 141928 Version 3.0

References

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