Abstract
The Marfan syndrome (MFS) is strongly associated with aortic disease causing a high prevalence of prophylactic aortic surgery, aortic dissection, and sudden death. The aim of the present study was to evaluate mortality in a nationwide Danish MFS population diagnosed by the Ghent II criteria. In a register-based setting, we identified all Danish patients with MFS (n = 412, men n = 215) by assessment of their medical records. We established a gender and age matched control cohort based on 41,000 control patients (men n = 21,500). MFS cases risk time was 6,669 patient years. We applied Cox regression using each case and his/her control as one stratum, adjusting for age and calendar time. We found a significantly decreased lifespan of 50years compared with 60years among controls. The mortality hazard ratio among MFS compared with controls was significantly increased to3.6 (CI 2.8-4.7, p < 0.001); men 4.0 (CI 2.8-5.7, p < 0.001); women 3.2 (CI 2.1-4.8,p < 0.001). Aorta disease represented the main reason for the overall increased mortality with a hazard ratio of 194.6 (CI 67.4-561.7, p < 0.0001); men 208.7 (CI 53.8-809.1, p < 0.001); women 173.4 (CI 31.5-954.5, p < 0.001). In addition, an unexplained mortality due to respiratory ill...Continue Reading
Citations
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