[0:00–0:32]
On-Screen Title: Dr. Nupoor Narula; Cardiologist; Director, Women’s Heart Program; NewYork-Presbyterian/Weill Cornell Medicine
On-Screen Title: Redefining pregnancy-associated vascular risks in Marfan syndrome
Dr. Nupoor Narula: Marfan syndrome is an inherited connective tissue disorder with a prevalence of approximately 1 in 5,000. It is caused by mutations in the fibrillin-1 gene. The overall clinical phenotype can be highly variable. However, aortic aneurysms, predominantly involving the aortic root, are highly prevalent and individuals are more susceptible to both types A and types B aortic dissection.
[0:33–0:58]
Dr. Nupoor Narula: Now, the United States guidelines had recommended that for women with Marfan syndrome contemplating pregnancy, prepartum aortic dimensions greater than 4.0 centimeters would proceed to elective aortic surgery prior to pregnancy. The European guidelines recommended that in women with Marfan syndrome contemplating pregnancy, a prepartum aortic root of greater than 4.5 centimeters should prompt elective aortic intervention.
[0:59–1:24]
Dr. Nupoor Narula: Now, these numbers in the cardiology community are highly distinct. It’s like saying someone is five foot tall versus six foot tall. So we harnessed our large Cornell Aortic Aneurysm Registry, one of the largest single-center aneurysm registries internationally with serial and systematic imaging data. This included a group of very well-characterized Marfan syndrome women by the Ghent diagnostic nosologies, whose pregnancies were followed systematically with serial imaging data.
[1:25 –1:55]
Dr. Nupoor Narula: Our results demonstrated that women with Marfan syndrome, in whom we have serial and systematic imaging data, demonstrated stability of aortic dimensions throughout the peripregnancy period. This included women who had prepartum aortic dimensions between 4 and 4.5 centimeters. There were five women in whom we noted peripregnancy-related aortic complications. Two women had type A dissection, which both occurred in the third trimester. Both of these women were unaware of their diagnosis of Marfan syndrome prior to pregnancy.
[1:56–2:17]
Dr. Nupoor Narula: There were two type B dissections that occurred early in the postpartum period, both in women who were aware of their diagnosis of Marfan syndrome prior to pregnancy and who had nonprohibitive or nonsurgical aortic dimensions. There was also one isolated coronary artery dissection that occurred early in the postpartum period in a woman that was aware of her diagnosis of Marfan syndrome prior to pregnancy.
[2:18–2:42]
Dr. Nupoor Narula: The cumulative incidence of having an aortic dissection was similar in never- versus ever-pregnant women. And this should be noted. So our results were a rationale to update the 2002 United States aortic disease guidelines for the management of pregnancy in Marfan syndrome. It is now recommended that women with prepartum aortic dimensions of 4.5 centimeters undergo elective aortic surgery prior to pregnancy.
[2:43–3:18]
Dr. Nupoor Narula: The big takeaway from our paper is that type B dissections and Marfan syndrome are unpredictable. This has really paved the way for our latest data, which was published in the Journal of the American College of Cardiology in November of 2023, in which we showed that type B aortic dissection in this patient cohort really represents more severe phenotype in the setting of longer life expectancy.
On-Screen Title: Dr. Narula’s study supported the US guidelines update for pregnancy management in Marfan syndrome
[END]
