Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)

Abstract

MD; for the GenTAC Investigators* Background--The risk of aortic complications associated with pregnancy in women with Marfan syndrome (MFS) is not fully understood.

Response by Kamel to Letter Regarding Article, “Pregnancy and the Risk of Aortic Dissection or Rupture: A Cohort-Crossover Analysis”

“…Furthermore, the risk of aortic complications may be lower provided that the underlying Marfan syndrome is recognized and appropriately managed before pregnancy, rather than being discovered at the time of a pregnancy-related aortic complication. 2 We also fully agree with Chun-Ka Wong and colleagues about the importance of a risk stratification system for personalized prediction of aortic complications during pregnancy. The data we used in our study 1 provide a reliable estimate of the heightened risk of aortic complications during pregnancy across the overall population of pregnant patients but are less useful for personalized risk prediction given the lack of certain clinical details.…”

Sex, pregnancy and aortic disease in Marfan syndrome

“…A recent retrospective study by Roman et al ., using data from the GenTAC registry, reported an 8-fold increased risk of type A or B aortic dissection in pregnant MFS women compared to never-pregnant MFS women [19]. This risk was especially increased during the immediate postpartum period.…”

Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study

“…8 Diagnostic criteria of MFS were revised in 2010 (revised Ghent nosology) to place more emphasis on causative genetic analyses, aortic root aneurysm/dissection, and ectopia lentis (EL). 2,9,10 Since the release of the revised Ghent nosology, there have been two cohort studies on pregnancy outcomes of MFS patients collected from multiple institutions 11,12 and five case series. [13][14][15][16][17] Collectively, these reports indicate that (1) pregnant MFS patients have a higher risk of AD compared with non-pregnant MFS patients, 11,12 and (2) pregnancy increases the risk of aortic complications in the long-term.…”

“…2,9,10 Since the release of the revised Ghent nosology, there have been two cohort studies on pregnancy outcomes of MFS patients collected from multiple institutions 11,12 and five case series. [13][14][15][16][17] Collectively, these reports indicate that (1) pregnant MFS patients have a higher risk of AD compared with non-pregnant MFS patients, 11,12 and (2) pregnancy increases the risk of aortic complications in the long-term. 13 However, further evaluation of pregnancy outcomes in patients with MFS diagnosed according to the revised Ghent nosology is still required to establish guidelines for management of pregnant patients with this syndrome.…”