Abstract 12904: National Trends in Aortic Aneurysm and Dissections in Patients With Marfans and Ehlers Danlos Syndrome

Abstract

Introduction: Marfans (MFS) and Ehlers Danlos syndrome (EDS) predispose patients to acute aortic dissections and aneurysms. Given their low incidence, there are limited large cohort data on mortality. Hypothesis: Type of connective tissue disorder will predict mortality in patients with Marfans or Ehlers Danlos Syndrome. Methods: The National Inpatient Sample (NIS) was reviewed for adults (>18 years) with MFS and EDS between 2010-2017 via ICD9 and 10 diagnosis codes. Procedure codes were used to distinguish patients undergoing open aortic repair or TEVAR (thoracic endovascular aortic repair) for either a type A aortic dissection or an aortic aneurysm. NIS weights were used to project the 20% NIS volume to national volumes. Univariable logistic regression was used to determine independent predictors of mortality. Results: A total of 19,567 patients were identified. The incidence of MFS and EDS was 18 and 22.4 per 100k, respectively. After applying the inclusion criteria, there were 2,553 MFS and 180 EDS patients. EDS patients were more likely older (50 vs 42, P<0.001) and women (47% vs 33%, P<0.001). MFS patients were more likely to have a type A aortic dissection (44% vs 31%, P<0.001). However, in the total cohort, aortic aneurysm was more frequent (56%). EDS patients were more likely to undergo TEVAR (2.8% vs 1.0%, P=0.03). MFS patients were more likely to have a complication of acute kidney injury (P=0.02) whereas EDS patients were more likely to have bowel ischemia (P=0.05). The majority of patients (89%) were treated at urban teaching hospitals. There was no statistical difference in mortality between the MFS and EDS cohorts (4.6% vs 2.8%, P=0.26). In logistic regression for death, aortic aneurysm (HR 0.14, P<0.001) and elective admission (OR 0.28, P=0.003) were protective for death whereas aortic dissection was a predictor for mortality (OR 7.31, P<0.001). Type of connective tissue disease or sex were not significant predictors. Conclusions: National level estimates show low mortality for patients with MFS and EDS presenting to the hospital with an aortic dissection or aneurysm. Differences in age and sex can guide aortic surveillance for these patient populations, leading to potentially decreased risk of acute aortic dissections and hospital mortality.