Aortic Surgery Outcomes of Marfan Syndrome and Ehlers-Danlos Syndrome Patients at Teaching and Nonteaching Hospitals.

BACKGROUND: Despite improvements in prevention and management, 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 nonteaching hospitals. METHODS: We used the National Inpatient Sample to study patients with MFS or EDS undergoing open or endovascular aortic aneurysm repair from 2000 to 2014. RESULTS: Of 3487 patients (MFS = 3375, EDS = 112), 2974 (85%) had repair at a teaching hospital. Patients who underwent repair at a teaching hospital were slightly younger than those who underwent repair at a nonteaching hospital (38 vs. 43 years, P < 0.01) but otherwise were similar in gender (29% vs. 28% female), race (70% vs. 78% white), and connective tissue disorder diagnosis (97% vs. 97% MFS, all P ≥ 0.1). There were no differences in anatomy (17% vs. 19% abdominal, 67% vs. 66% thoracic, and 15% vs. 15% thoracoabdominal, all P ≥ 0.1) or type of repair (5% vs. 5% endovascular), but patients at nonteaching hospitals were more likely to have a dissection (49% vs. 38%, P = 0.02). There was no difference in perioperative mortality (4% vs. 6%, P = 0.5) or length of stay (median 8 days vs. 7 days, P = 0.3) between teaching and nonteaching hospitals. There was also no difference in hemorrhagic (47% vs. 43%), pulmonary (9% vs. 16%), renal (12% vs. 14%), or neurologic (5% vs. 6%) complications between teaching and nonteaching hospitals, respectively (all P ≥ 0.05). In analysis stratified by anatomic extent of repair, there was a lower prevalence of pulmonary complications in thoracic aorta repairs at teaching hospitals (8.1% vs. 18.4%, P = 0.01) but a higher prevalence of hemorrhage in abdominal aortic repairs at teaching hospitals (45.6% vs. 20.6%, P = 0.04) as compared with nonteaching hospitals. CONCLUSIONS: Patients with MFS and EDS who undergo aortic aneurysm repair have their operations predominantly at teaching hospitals, but those patients who undergo repair at nonteaching hospitals do not have worse mortality or morbidity despite a higher incidence of dissection.

The Marfan syndrome: implications for athletes and their echocardiographic assessment.

Sudden death of competitive athletes is rare. These deaths challenge the perception that trained athletes represent the healthiest segment of modern society. The increasing frequency of such reported deaths worldwide and the visibility of the issue is underlined by the high-profile nature of each case. The majority of these deaths have been due to a variety of undiagnosed cardiovascular diseases. Marfan syndrome is a heritable disorder of the connective tissue that can hold life threatening consequences, especially for the athletic population. This paper will aim to review cardiovascular pathophysiology and assessment in relation to Marfan syndrome with particular reference to echocardiography and the athletic population.