Causes of Mortality in the Marfan Syndrome (from a Nationwide Register Study)
Causes of Mortality in the Marfan Syndrome (from a Nationwide Register Study)
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DOI：
10.1016/j.amjcard.2018.06.034
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发表时间：
2018-10-01
影响因子：
2.8
通讯作者：
Gravholt, Claus H.
中科院分区：
文献类型：
--
作者：
Groth, Kristian A.;Stochholm, Kirstine;Gravholt, Claus H.
马凡综合征 (MFS) 与主动脉疾病密切相关，导致预防性主动脉手术、主动脉夹层和猝死的发生率很高。本研究的目的是评估根据根特 II 标准诊断的丹麦全国 MFS 人群的死亡率。在基于登记的环境中，我们通过评估患者的医疗记录来识别所有患有 MFS 的丹麦患者（n = 412，男性 n = 215）。我们基于 41,000 名对照患者（男性 n = 21,500）建立了性别和年龄匹配的对照队列。 MFS 病例的风险时间为 6,669 患者年。我们应用 Cox 回归，将每个病例及其对照作为一个层，并根据年龄和日历时间进行调整。我们发现对照组的寿命显着缩短 50 年，而对照组的寿命为 60 年。与对照组相比，MFS 的死亡风险比显着增加至 3.6（CI 2.8-4.7，p < 0.001）；男性 4.0（CI 2.8-5.7，p < 0.001）；女性 3.2（CI 2.1-4.8，p < 0.001）。主动脉疾病是总体死亡率增加的主要原因，风险比为 194.6 (CI 67.4-561.7, p < 0.0001)；男性 208.7（CI 53.8-809.1，p < 0.001）；女性 173.4 (CI 31.5-954.5, p < 0.001)。此外，呼吸系统疾病导致的不明原因死亡并非由气胸引起。排除心血管和呼吸系统死亡原因，我们没有发现任何迹象表明 MFS 与其他原因导致的死亡率增加有关。 (C) 2018 Elsevier Inc. 保留所有权利。
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 50 years compared with 60 years among controls. The mortality hazard ratio among MFS compared with controls was significantly increased to 3.6 (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 illness was not attributed to pneumothorax. Excluding cardiovascular and respiratory causes of death, we found no indication that MFS is associated with increased mortality for other reasons. (C) 2018 Elsevier Inc. All rights reserved.