Siddiqi Hasan K, Luminais Steven N, Montgomery Dan, Bossone Eduardo, Dietz Harry, Evangelista Arturo, Isselbacher Eric, LeMaire Scott, Manfredini Roberto, Milewicz Dianna, Nienaber Christoph A, Roman Mary, Sechtem Udo, Silberbach Michael, Eagle Kim A, Pyeritz Reed E
Department of Internal Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Department of Internal Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Am J Cardiol. 2017 Mar 1;119(5):785-789. doi: 10.1016/j.amjcard.2016.11.021. Epub 2016 Dec 2.
Marfan syndrome (MFS) is an autosomal dominant connective tissue disease associated with acute aortic dissection (AAD). We used 2 large registries that include patients with MFS to investigate possible trends in the chronobiology of AAD in MFS. We queried the International Registry of Acute Aortic Dissection (IRAD) and the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) registry to extract data on all patients with MFS who had suffered an AAD. The group included 257 patients with MFS who suffered an AAD from 1980 to 2012. The chi-square tests were used for statistical testing. Mean subject age at time of AAD was 38 years, and 61% of subjects were men. AAD was more likely in the winter/spring season (November to April) than the other half of the year (57% vs 43%, p = 0.05). Dissections were significantly more likely to occur during the daytime hours, with 65% of dissections occurring from 6 a.m. to 6 p.m. (p = 0.001). Men were more likely to dissect during the daytime hours (6 a.m. to 6 p.m.) than women (74% vs 51%, p = 0.01). These insights offer a glimpse of the times of greatest vulnerability for patients with MFS who suffer from this catastrophic event. In conclusion, the chronobiology of AAD in MFS reflects that of AAD in the general population.
马凡综合征(MFS)是一种常染色体显性遗传性结缔组织疾病,与急性主动脉夹层(AAD)相关。我们利用两个包含马凡综合征患者的大型登记处,来研究马凡综合征中急性主动脉夹层时间生物学的可能趋势。我们查询了国际急性主动脉夹层登记处(IRAD)和基因触发胸主动脉瘤及心血管疾病登记处(GenTAC),以提取所有发生急性主动脉夹层的马凡综合征患者的数据。该组包括1980年至2012年间发生急性主动脉夹层的257例马凡综合征患者。采用卡方检验进行统计学分析。急性主动脉夹层发生时患者的平均年龄为38岁,61%为男性。急性主动脉夹层在冬季/春季(11月至4月)比一年中的其他时间更易发生(57%对43%,p = 0.05)。夹层明显更易在白天发生,65%的夹层发生在上午6点至下午6点(p = 0.001)。男性比女性更易在白天(上午6点至下午6点)发生夹层(74%对51%,p = 0.01)。这些见解让我们得以一窥患有这种灾难性疾病的马凡综合征患者最易发病的时间。总之,马凡综合征中急性主动脉夹层的时间生物学与普通人群中急性主动脉夹层的情况相符。