National trends in thoracic aortic aneurysms and dissections in patients with Marfans and Ehlers Danlos syndrome.

Abstract

Connective tissue disorders predispose patients to earlier aortic dissections and aneurysms. However, there is limited large cohort data given its low incidence. The National Inpatient Samplewas searched for all adults with Marfans (MFS) and Ehlers Danlos (EDS) disease between 2010 and 2017. ICDcodes were used to select those with a type A aortic dissection or aneurysm. There was a total of 19,567 cases, giving the estimated incidence of MFS and EDS of 18 and 22.4 per 100k people, respectively. After inclusion criteria, there were 2553 MF and 180 EDS patients. There was no statistical difference in mortality between the MFS and EDS cohorts (4.6% vs. 2.8%, p = .26). EDS patients were more likely to undergo a TEVAR procedure (2.8% vs. 1.0%, p = .03). MF patients were more likely to have a complication of acute kidney injury (p = .02). EDS patients were more likely older (50 vs. 42, p < .001) and female (47% vs. 33%, p < .001). MFS patients were more likely to have a type A aortic dissection (44% vs. 31%, p < .001). The majority (89%) of patients were treated at urban teaching hospitals. On univariable logistic regression, aortic dissection was a predictor for mortality (odds ratio7.31, p < .001). The type of connective tissue disease was not a significant predictor. National level estimates show low mortality for patients with MF or ED presenting to the hospital with aortic dissection or aneurysm. The differences in age and gender can guide surveillance for these patient populations, leading to more elective admissions and reduced hospital mortality.