Marfan Syndrome Research - Genetics, Causes, Symptoms, Treatment

Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.


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Fate of the residual distal and proximal aorta after acute type a dissection repair using a contemporary surgical reconstruction algorithm.

Geirsson A, Bavaria JE, Swarr D, Keane MG, Woo YJ, Szeto WY, Pochettino A

Division of Cardiothoracic Surgery, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, USA.

BACKGROUND: In this study, we evaluated the long-term results of our contemporary, standardized surgical management algorithm for repair of acute type A aortic dissections. Prior reports have analyzed heterogeneous techniques and populations. METHODS: From 1993 to 2004, 221 consecutive patients underwent repair of acute type A aortic dissection at our aortic center. Hemiarch repair was performed in 97.7% (216 of 221), and total arch in 2.3% (5 of 221). Of these, 72.9% (161 of 221) underwent aortic valve resuspension, and 27.1% (60 of 221) had aortic root replacement. RESULTS: In-hospital mortality for a primary operation was 12.7% (28 of 221). Actuarial survival was 79.2% at 1 year, 62.8% at 5 years, and 46.3% at 10 years. Significant risk factors for decreased survival included prior stroke, cerebral malperfusion, and length of cardiopulmonary bypass. Freedom from proximal reoperation after aortic valve resuspension was 94.6% at 5 years and 76.8% at 10 years, with cardiac malperfusion as the main risk factor. Freedom from distal reoperation was 87.6% at 5 years and 76.4% at 10 years, with Marfan syndrome, age, and extent of dissection as significant risk factors for reoperation. In-hospital mortality was 18.2% (2 of 11) after proximal reoperation and 31.2% (5 of 16) after distal reoperation. CONCLUSIONS: We report improved long-term durability of our proximal root repair, with cardiac malperfusion as a significant risk factor. Marfan disease, younger age, and DeBakey type I dissection are risk factors for distal reoperation. To further improve long-term outcome, means to prevent progression of distal aortic disease need to be developed.

Published 26 November 2007 in Ann Thorac Surg, 84(6): 1955-64; discussion 1955-64.
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Marfan Syndrome - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References

Marfan Syndrome - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References