Cardiovascular and obstetrical outcomes among delivering patients with Marfan or Loeys-Dietz syndrome: a retrospective analysis by hospital delivery setting

Abstract

BackgroundPregnancy is a high-risk time for patients with Marfan syndrome (MFS) or Loeys-Dietz (LDS) syndrome due to risk for cardiovascular complications including risk of aortic dissection. Little is known about differences in obstetric and cardiac outcomes based on delivery hospital setting (academic/academic-affiliated versus community medical centers). ObjectiveThis study aimed to evaluate obstetric and cardiac outcomes of patients with MFS or LDS based on delivery hospital setting. Study DesignThis is a secondary analysis of a retrospective, observational cohort study of singleton pregnancies among patients with a diagnosis of MFS or LDS from 1990 to 2016. Patients were identified through the Marfan Foundation, Loeys-Dietz Syndrome Foundation, or Cardiovascular Connective Tissue Clinic at Johns Hopkins Hospital. Data were obtained via self-reported obstetric history and verified by review of medical records. Nonparametric analysis was performed via Fisher's Exact Test and Wilcoxon rank-sum tests. Results273 deliveries among patients with MFS or LDS were included in this analysis. More patients who had a known diagnosis prior to delivery for either MFS or LDS delivered at an academic hospital as compared community hospital (78.6% vs. 59.9%, p=0.001). Patients with MFS or LDS who delivered at academic centers were more likely to have an operative vaginal delivery compared to those at community centers (23.7% vs 8.6%, p=0.002). When indications for cesarean delivery were assessed, connective tissue disease was the primary reason for indication for mode of delivery at community centers compared to academic centers (55.6% vs 43.5%, p=0.02). There were higher rates of cesarean for arrest of labor and/or malpresentation at community hospitals compared to academic centers (23.6% vs 5.3%, p=0.01). There were no differences between groups in regards to method of anesthesia for delivery. Among those with a known diagnosis of MFS or LDS prior to delivery, there were increased operative vaginal delivery rates at academic hospitals compared to community hospitals (27.2% vs 15.1%, p=0.03; Table 2). More patients with an aortic root measuring ≥4 cm pre-pregnancy or postpartum delivered at academic centers compared to community centers (33.0% vs. 10.2%, p=0.01), but there were no significant differences in median size of the aortic root during pregnancy or on postpartum assessment between delivery locations. Cardiovascular complications were rare: eight patients who delivered at academic centers and seven patients who delivered at community centers had an aortic dissection either in pregnancy or the postpartum period (p=0.79). ConclusionPatients with MFS/LDS and more severe aortic phenotypes were more likely to deliver at academic hospitals. Those who delivered at academic hospitals had higher rates of operative vaginal delivery. Despite lower frequencies of aortic root diameter >4.0 cm, those delivered at community hospitals had higher rates of cesarean delivery for the indication of MFS/LDS. Optimal delivery management of these patients requires further prospective research.