During pregnancy, women with Marfan syndrome (MFS) were found to have stable aortic dimensions when aortic root diameters were between 4.0 cm and 4.5 cm, according to study results published in the Journal of the American College of Cardiology. These findings provide a rationale for updating current United States Guidelines for elective aortic root replacement prior to pregnancy in women with MFS.
In this retrospective chart review of women with MFS conducted by 2 cardiologists at Cornell University between 1982 and 2020, clinical, imaging, and pregnancy-related history and vascular complications were compared between nulligravid and gravid women.
Of 169 women with MFS, 44% had a total of 152 pregnancies with 112 live births. Baseline characteristics were similar between the nulligravid and gravid groups, though nulligravid women were significantly younger and had an increased likelihood of thoracic skeletal abnormalities. Of the 74 gravid women, only 65% were aware of their MFS diagnosis prior to their first pregnancy.
The rates of elective aortic repair and aortic dissection were similar between the nulligravid and gravid groups (47.4% vs 36.5%, respectively; P =.16; 23.2% vs 31.1%; P =.25, respectively). Of note, both elective aortic repair and dissection occurred at a younger age among women in the nulligravid group vs those in the gravid group.
Among the 54 observed pregnancies with partial or complete imaging available, the aortic root dimensions remained largely stable with a mean prepartum diameter of 3.9 cm (interquartile range, 3.8-4.0), including in women with prepartum diameters between 4.0 cm and 4.5 cm. In addition, 4 women had aortic diameters greater than 4.5 cm.
A total of 5 women experienced vascular complications during the peripartum period: 2 were unaware of their MFS diagnosis and experienced type A dissections in the third trimester; 2 were aware of their MFS diagnosis and experienced type B dissections in the early postpartum period but dimensions of the descending aorta were unknown; and 1 who was aware of her MFS diagnosis with a prepartum aortic root diameter of 3.9 cm experienced spontaneous coronary artery dissection in the early postpartum period.
There was a 5-fold increase in aortic dissection rates in the pregnancy vs nonpregnancy period (rate ratio [RR], 5.1; 95% CI, 2.0-12.9; P =.0001), with similar increased rates observed on analysis of only women in the gravid group (RR, 4.7; 95% CI, 1.7-12.7; P =.0007).
Limitations of this study included the lack of systematic data on the use of β-blockers, type of delivery, fetal birth weight, and lactation.
Pregnancy-related “type B dissection remains an unpredictable complication and warrants further study,” the researchers noted.
Given that findings from this study support the safety of pregnancy in women with MFS with prepartum aortic root diameters up to 4.5 cm, the researchers proposed that the US guidelines be updated to reflect European guidelines that recommend women with MFS to undergo elective aortic repair prior to pregnancy if the root diameter is greater than 4.5 cm.
Narula N, Devereux RB, Malonga GP, Hriljac I, Roman MJ. Pregnancy-related aortic complications in women with Marfan syndrome. J Am Coll Cardiol. 2021;78(9):870-879. doi:10.1016/j.jacc.2021.06.034