Aortic Dissection in Pregnant Women With Marfan Syndrome

For women with Marfan syndrome (MFS), pregnancy-related type B aortic dissection was observed to be an unpredictable complication, according to results of a study published in the Journal of the American College of Cardiology.

The Cornell Aortic Aneurysm Registry was populated with medical records dating from 1982 and 2020 from 2 adult cardiologists. All patient records were reviewed for possible Marfan syndrome, women were stratified on the basis of pregnancy status, and clinical outcomes were assessed.

A total of 169 women were included in this analysis; 74 had between 1 and 8 documented pregnancies resulting in 112 live births. Women who were considering pregnancy were counseled and aortic dimensions were assessed. Pregnancies were determined to be “sanctioned” if the maternal prepartum aortic root measured £4.5 cm in diameter.

Women who were ever pregnant and never pregnant were aged 49.6±15.0 and 40.5±14.4 years at their last visit (P <.001), and 80.3% and 48.9% (P <.001), respectively, had a documented family history of Marfan syndrome. All other demographic and noncardiovascular phenotypic features were similar among patient groups.

Rates of aortic dissection were not significantly different among ever-pregnant and never-pregnant women (31% vs 23%; P =.25). However, women who were never pregnant were found to be at increased risk for age-related elective aortic surgery (P =.0028).

Fewer women who were ever pregnant vs those who were never pregnant had aortic root dilation (mean, 87.8% vs 96.8%; P =.02) and were at increased age at aortic surgery (mean, 41.7 vs 32.5 years; P =.003), respectively. Although not reaching significance, the study authors reported a trend for women who were ever pregnant to undergo aortic dissection at a later age (mean, 44.9 vs 37.9 years; P =.07).

The average prepartum aortic dimension was 3.9 (interquartile range [IQR], 3.8-4.0) cm, intrapartum was 4.0 (IQR, 3.7-4.2) cm, and postpartum was 3.9 (IQR, 3.6-4.2) cm. In general, the use of beta-blockers during pregnancy was associated with 0.3 cm smaller aortic diameters (P <.005).

Vascular complications occurred among 5 women during the peripartum period. Type A dissections occurred among 2 women who were unaware of having Marfan syndrome, type B dissections occurred among 2 women who were aware of their diagnosis but were not receiving care for Marfan syndrome, and a left anterior descending artery dissection occurred in 1 woman who knew of her diagnosis and was on medication for Marfan syndrome.

During the pregnancy period, rates of aortic dissection were significantly higher among all women (rate ratio [RR], 5.1; 95% CI, 2.0-12.9; P =.0001) and among women who had ever been pregnant (RR, 4.7; 95% CI, 1.7-12.7; P =.0007).

This study was limited as systematic data were not uniformly available for all patients.

These data indicated that women with Marfan syndrome were found to be at increased risk for aortic dissection during the peripartum period but not overall compared with never-pregnant women. Prepregnancy counseling likely mitigates the risk for type A dissection, but type B dissection may be a more unpredictable outcome.

Reference

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