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  • Title: Presentation, surgical intervention, and long-term survival in patients with Marfan syndrome.
    Author: Aranson NJ, Patel PB, Mohebali J, Lancaster RT, Ergul EA, Clouse WD, Conrad MF, Patel VI.
    Journal: J Vasc Surg; 2020 Aug; 72(2):480-489. PubMed ID: 32085956.
    Abstract:
    OBJECTIVE: Patients with Marfan syndrome (MFS) often present with acute catastrophic aortic events at a young age and have a shortened life span. This study examines the impact of presentation and demographics on late survival in patients with MFS. METHODS: Adults with confirmed MFS in our thoracic aortic center dataset were identified and statistical analysis performed to identify the incidence and predictors of aortic interventions and late mortality. RESULTS: We identified 301 patients with a MFS initial diagnosis at age 17 years (interquartile range, 4-30 years) with presentation into our thoracic aortic center at 21 years (interquartile range, 8-34 years). The average follow-up in our center was 10 ± 10 years. Clinical features were 41% male, 86% white race, coronary artery disease 28%, hypertension 40%, peripheral vascular disease 19%, and anti-impulse agent in 51% (β-blocker, angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, calcium channel blocker). Distribution of operative aortic pathology was isolated to the ascending aorta (70%) and descending aorta (8%). One hundred seventy-eight patients (59%) required primary aortic surgery (36% emergent). Primary procedures were cardiac (aortic valve/root) in nature in 94%. Seventy-four patients (42%) required multiple aortic procedures at a mean of 9.2 ± 6.9 years, involving the thoracoabdominal aorta in 65%, thoracic aorta in 37%, and abdominal aorta in 21%. Patients who required multiple aortic procedures were more likely (P < .05) to have coronary artery disease (50% vs 30%), and peripheral vascular disease (43% vs 18%). Multiple aortic procedures were also more likely (P < .05) in patients who developed de novo distal dissection (14% vs 0%), had prior dissection (47% vs 18%), or unknown MFS at the time of the initial procedure (27% vs 63%). Multivariable analysis identified prior dissection as an independent predictor of need for emergent surgery (odds ratio, 13.20; 95% confidence interval, 4.64-37.30; P < .05), as well as additional aortic surgery (odds ratio, 4.42; 95% confidence interval, 1.87-10.50; P < .05). Kaplan-Meier analysis showed similar 10-year survival with or without aortic interventions (82% with vs 89% without; P = .08). Late survival was decreased in patients undergoing emergent initial procedures (66% vs 89%; P < .01), as well as those undergoing multiple operations (74% vs 86%; P = .03). CONCLUSIONS: These data indicate that, in the modern era, the mode of presentation and need for multiple procedures have a detrimental impact on late survival. Additionally, the presence of acute or chronic dissection predicts the need for additional aortic procedures during follow-up.
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