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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Aortic diameter >or = 5.5 cm is not a good predictor of type A aortic dissection: observations from the International Registry of Acute Aortic Dissection (IRAD).

Pape LA, Tsai TT, Isselbacher EM, Oh JK, O'gara PT, Evangelista A, Fattori R, Meinhardt G, Trimarchi S, Bossone E, Suzuki T, Cooper JV, Froehlich JB, Nienaber CA, Eagle KA,

University of Massachusetts Medical School, 55 Lake Ave N, Worcester, MA 01655, USA. linda.pape@umassmed.edu.

BACKGROUND: Studies of aortic aneurysm patients have shown that the risk of rupture increases with aortic size. However, few studies of acute aortic dissection patients and aortic size exist. We used data from our registry of acute aortic dissection patients to better understand the relationship between aortic diameter and type A dissection. METHODS AND RESULTS: We examined 591 type A dissection patients enrolled in the International Registry of Acute Aortic Dissection between 1996 and 2005 (mean age, 60.8 years). Maximum aortic diameters averaged 5.3 cm; 349 (59%) patients had aortic diameters <5.5 cm and 229 (40%) patients had aortic diameters <5.0 cm. Independent predictors of dissection at smaller diameters (<5.5 cm) included a history of hypertension (odds ratio, 2.17; 95% confidence interval, 1.03 to 4.57; P=0.04), radiating pain (odds ratio, 2.08; 95% confidence interval, 1.08 to 4.0; P=0.03), and increasing age (odds ratio, 1.03; 95% confidence interval, 1.00 to 1.05; P=0.03). Marfan syndrome patients were more likely to dissect at larger diameters (odds ratio, 14.3; 95% confidence interval, 2.7 to 100; P=0.002). Mortality (27% of patients) was not related to aortic size. CONCLUSIONS: The majority of patients with acute type A acute aortic dissection present with aortic diameters <5.5 cm and thus do not fall within current guidelines for elective aneurysm surgery. Methods other than size measurement of the ascending aorta are needed to identify patients at risk for dissection.

Published 4 September 2007 in Circulation, 116(10): 1120-7.
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Marfan Syndrome Research Today Archive:

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Marfan Syndrome Books

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