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Aortic Surgery Outcomes of Marfan Syndrome and Ehlers-Danlos Syndrome Patients at Teaching and Non-Teaching Hospitals
Courtenay Holscher, MD, Hanaa Dakour Aridi, MD, Satinderjit Locham, MD, Caitlin W. Hicks, MD, MS, Joseph K. Canner, MHS, Mahmoud Malas, MD, MHS, James H. Black, III, MD.
Johns Hopkins University, Baltimore, MD, USA.

Objective: Despite improvements in prevention and operative techniques, aortic aneurysm repair remains a high risk operation for patients with Marfan syndrome (MFS) and Ehlers-Danlos syndrome (EDS). The goal of this study was to examine differences in characteristics and outcomes of patients with MFS or EDS undergoing aortic aneurysm repair at teaching versus non-teaching hospitals.
Methods: We used National Inpatient Sample data to study characteristics and outcomes of patients with MFS or EDS undergoing open or endovascular aortic aneurysm repair from 2000 to 2014. Chi-square and t-tests were used to compare patients who underwent repair at teaching versus non-teaching hospitals.
Results: Of 3482 patients undergoing aortic aneurysm repair during the study period, 2974 (85%) underwent repair at a teaching hospital. Patients who underwent repair at a teaching hospital were slightly younger than those who underwent repair at a non-teaching hospital (38 vs 43 years, p=0.003) but otherwise were similar in gender (29% vs 28% female), race (70% vs 78% white), and connective tissue disorder diagnosis (97% vs 97% MFS). There was no difference in anatomy (17% vs 19% abdominal, 67% vs. 66% thoracic, and 15% vs 15% thoracoabdominal) or type of repair (5% vs 6% endovascular) between teaching and non-teaching hospitals respectively, however patients presenting to non-teaching hospitals were more likely to have a dissection (49% vs. 38%, p=0.02). There was no difference in mortality (4% vs 6%) or length of stay (median 8 days vs. 7 days) between teaching and non-teaching hospitals (Table). There was also no difference in hemorrhage (47% vs 43%), renal (12% vs 14%), or neurologic (5% vs 6%) complications between teaching and non-teaching hospitals respectively, although teaching hospitals had a trend toward fewer pulmonary complications (9% vs 16%, p=0.06, Table).
Conclusion: Marfan or Ehlers-Danlos syndrome patients who undergo aortic aneurysm repair have their operations predominantly at teaching hospitals, but those patients who undergo repair at non-teaching hospitals do not have worse mortality or morbidity despite a higher incidence of dissection. This data suggests transfer of MFS or EDS patients should be considered selectively if surgical support is not available at the facility of the index presentation.

Table. Outcomes of Marfan and Ehlers-Danlos patients
Teaching (n=2974)Non-Teaching (n=508)p-value
Mortality (n (%))191 (6%)23 (4%)0.48
Complications (n (%))
Hemorrhage1406 (47%)218 (43%)0.46
Wound Infection117 (4%)24 (5%)0.75
Cardiac902 (30%)173 (34%)0.54
Pulmonary270 (9%)80 (16%)0.06
Renal353 (12%)69 (14%)0.61
Neurologic147 (5%)28 (6%)0.82
Gastrointestinal80 (3%)24 (5%)0.24
Length of stay (median (IQR))8 days (5-13)7 days (5-12)0.31
Total charges (mean (SE))$158,774 ($9834)$165,546 ($14765)0.71


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