کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5966514 1576158 2015 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Increased aortic tortuosity indicates a more severe aortic phenotype in adults with Marfan syndrome
ترجمه فارسی عنوان
افزایش گرگرفتگی آئورت نشان دهنده یک فنوتیپ شدید آئورت در بزرگسالان با سندرم مارفان است
کلمات کلیدی
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی کاردیولوژی و پزشکی قلب و عروق
چکیده انگلیسی

BackgroundPatients with Marfan syndrome (MFS) have a highly variable occurrence of aortic complications. Aortic tortuosity is often present in MFS and may help to identify patients at risk for aortic complications.Methods3D-visualization of the total aorta by MR imaging was performed in 211 adult MFS patients (28% with prior aortic root replacement) and 20 controls. A method to assess aortic tortuosity (aortic tortuosity index: ATI) was developed and reproducibility was tested. The relation between ATI and age, and body size and aortic dimensions at baseline was investigated. Relations between ATI at baseline and the occurrence of a clinical endpoint (aortic dissection, and/or aortic surgery) and aortic dilatation rate during 3 years of follow-up were investigated.ResultsATI intra- and interobserver agreements were excellent (ICC: 0.968 and 0.955, respectively). Mean ATI was higher in 28 age-matched MFS patients than in the controls (1.92 ± 0.2 vs. 1.82 ± 0.1, p = 0.048). In the total MFS cohort, mean ATI was 1.87 ± 0.20, and correlated with age (r = 0.281, p < 0.001), aortic root diameter (r = 0.223, p = 0.006), and aortic volume expansion rate (r = 0.177, p = 0.026). After 49.3 ± 8.8 months follow-up, 33 patients met the combined clinical endpoint (7 dissections) with a significantly higher ATI at baseline than patients without endpoint (1.98 ± 0.2 vs. 1.86 ± 0.2, p = 0.002). Patients with an ATI > 1.95 had a 12.8 times higher probability of meeting the combined endpoint (log rank-test, p < 0.001) and a 12.1 times higher probability of developing an aortic dissection (log rank-test, p = 0.003) compared to patients with an ATI < 1.95.ConclusionsIncreased ATI is associated with a more severe aortic phenotype in MFS patients.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: International Journal of Cardiology - Volume 194, 1 September 2015, Pages 7-12
نویسندگان
, , , , , , , , , , ,