A number of observations on Marfan syndrome have been made in previous IRAD studies ( 5- 8). The rationale and structure of IRAD have been published previously ( 4). The International Registry of Acute Aortic Dissection (IRAD) represents an investigational collaboration that has collected information on unselected consecutive cases of acute aortic dissection occurring at 30 aortic referral centers in 10 countries since January 1, 1996. Nevertheless, aortic dissection remains the leading cause of morbidity and mortality in these patients ( 2). Surgical replacement of the dilated aortic root and ascending aorta has significantly increased the life expectancy of patients with Marfan syndrome ( 2, 3). Aortic root dilatation/dissection is in fact one of the cardinal features of Marfan syndrome, according to the revised Ghent criteria ( 1). In most patients, the connective tissue disorder leads to abnormalities of the aortic wall, causing progressive aortic dilatation, thus increasing the risk of acute aortic dissection. Marfan syndrome is a heritable disorder of the fibrillin 1 ( FBN1) gene, which encodes the connective tissue protein fibrillin-1. Such a high rate of reinterventions highlights the need for careful surveillance and treatment for patients with Marfan syndrome surviving the acute phase of aortic dissection. Follow-up data for type A and B dissections combined show an estimated five-year survival rate of 80.1% and an estimated reintervention rate of 55.3% in patients with Marfan syndrome. The Marfan syndrome cohort that was treated with open surgery for type B dissection seemed to do especially well, with a 0% mortality rate (n=27). In contrast, the in-hospital mortality of Marfan syndrome patients with type B dissection appears to be lower than that of patients without Marfan syndrome. The in-hospital mortality in type A dissection was not significantly different in patients with or without Marfan syndrome, despite the differences in age and comorbidities and the lower incidence of aortic rupture in the Marfan syndrome cohort. ![]() ![]() We noted significantly larger diameters of the aortic annulus and root in the Marfan syndrome cohort, but no larger diameters more distally. 63.0☑4.0 years P<0.001) and in general had fewer comorbidities, although they more frequently had a known aortic aneurysm and history of prior cardiac surgery. Patients with Marfan syndrome presented at a significantly younger age compared to patients without Marfan syndrome (38.2☑3.2 vs. Between January 1996 and May 2017, the International Registry on Acute Aortic Dissections has collected information on a total of 6,424 consecutive patients with acute aortic dissection, including 258 individuals with a diagnosis of Marfan syndrome.
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