Abstract 19006: Peripartum Aortic Dissection in Women With Marfan Syndrome
Purpose: There is a known increased risk of aortic dissection (AD) in Marfan Syndrome (MFS) during pregnancy, although quantification of that risk has been difficult to obtain. The purpose of this study was to assess the incidence of AD in pregnant women with MFS and to compare the risk in MFS to other known risk factors.
Methods: The Texas Inpatient Public Use Data File (TIPUDF) contains administrative data on the majority of Texas hospital discharges. Inclusion criteria were all TIPUDF discharges 1999-2012 associated with pregnancy, including antepartum status, delivery, or postpartum status using designated ICD9 codes. Patients with MFS were then identified, as were patients with aortic dissection or rupture. MFS patient admissions were reviewed for readmission and repeat pregnancy by matching zip code, age, and race. Diagnoses of Turner Syndrome (TS), Ehlers-Danlos Syndrome (EDS), bicuspid aortic valve (BAV), coarctation, other aortic valve disorders, atherosclerosis, hypertension (HTN), and cocaine use were also identified.
Results: Of >37 million admissions, 5286576 were associated with pregnancy. Among those, there were 208 admissions in 152 patients with MFS, representing 171 pregnancies. Aortic dissection was coded in 41 admissions, representing 38 total ADs. Six ADs were in MFS, for an incidence in MFS and pregnancy of 3.5%. No AD was reported in TS, EDS, or BAV, although 2 events were in patients with isolated aortic valve disorders. The majority of the MFS AD events were post-partum (n=5), 3 of which were on an admission coincident with delivery. The site of AD was thoracic or thoracoabdominal in 5 (85%), with 1 limited to the abdomen, compared to abdominal site only in 40% of the non-MFS pregnant women with AD. After controlling for HTN and other known risk factors, there was a high risk for AD in MFS pregnancies compared to non-MFS pregnancies (OR >1000 [95%CI >999 to >999]. All MFS AD patients survived, although overall mortality in AD with pregnancy was 10.5%.
Conclusions: Although AD is rare in pregnancy, it is common in pregnant women with MFS, often occurring postpartum. Careful reproductive planning and close cardiac monitoring during pregnancy, but also after delivery in MFS are warranted.
- © 2013 by American Heart Association, Inc.