Pregnancy-Related Aortic Complications in Marfan Syndrome
- Preconception counseling for women with Marfan syndrome is important and requires careful assessment of aortic dimensions.
- Marfan syndrome is associated with an unpredictable risk of type B dissection. The aortic diameters remained stable during pregnancy in the women with Marfan syndrome with aortic diameters of 4.5 cm or less.
What are the pregnancy-associated vascular complications in Marfan syndrome (MFS)?
This was a retrospective chart review of patients with MFS managed by two cardiologists at Cornell (1982–2020). Clinical, imaging, and pregnancy-related history and vascular complications were collected and compared in never versus ever-pregnant women.
Of 169 women with MFS, there were 74 ever-pregnant women with 112 live births. There were no significant differences between never-pregnant versus ever-pregnant women in rates of aortic dissection (23% vs. 31%; p = 0.25) or elective aortic repair (47% vs. 36%; p = 0.16). In 54 observed pregnancies with serial imaging available, the aortic root dimensions remained largely stable (mean prepartum diameter was 3.9 cm), with four women having aortic diameters >4.5 cm. Type A aortic dissection occurred in two women during the third trimester (previously unaware of their diagnosis; one had root 5.6 cm). Type B dissections occurred in two women in the early postpartum period (unknown dimensions of the descending aorta). Spontaneous coronary artery dissection occurred in one woman in the early postpartum period. Rates of aortic dissection were five-fold higher in the pregnancy versus nonpregnancy period.
The authors concluded: 1) pregnancy-related type A dissection only occurred in patients unaware of their diagnosis; 2) type B dissection remains unpredictable; 3) those with prepartum diameters between 4.0 and 4.5 cm demonstrated stable aortic dimensions throughout pregnancy.
Counseling women with a diagnosis of MFS about risk of aortic dissection during pregnancy is challenging due to lack of prospective, systematic imaging, and data collection. The authors of this study reported women with MFS and aortic root up to 4.5 cm remained stable during pregnancy. This was a small number of women, but an important finding because of conflicting guidelines. The 2010 American Heart Association/American College of Cardiology guidelines suggest elective surgery prior to pregnancy if the aorta is >4.0 cm (Class IIa; Level of Evidence [LOE] C). The 2018 European Society of Cardiology guidelines suggest elective surgery prior to pregnancy if the aorta is >4.5 cm (Class IIa; LOE C). Since aortic surgery carries risk, especially with comorbidities such as obesity, the real-life conversation about elective surgery sometimes concludes with women being told not to become pregnant. This study provides some reassurance that women with MFS and aorta measurements <4.5 cm remained stable during pregnancy; however, preconception risk assessment and careful monitoring remain important. Additionally, the risk of type B dissection is notable and needs further study. For long-term family planning, the lifetime risk of aortic dissection should also be included in preconception discussions. Last, although a previously reported experimental mouse model suggested higher risk of dissection in mice related to oxytocin and lactation, there was no association between breastfeeding and aortic dissection in this cohort.
Clinical Topics: Cardiac Surgery, Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Prevention, Vascular Medicine, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement
Keywords: Aneurysm, Dissecting, Aorta, Thoracic, Counseling, Dissection, Diagnostic Imaging, Heart Defects, Congenital, Marfan Syndrome, Obesity, Postpartum Period, Pregnancy, Pregnant Women, Primary Prevention, Risk Assessment, Elective Surgical Procedures, Vascular Diseases, Women
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