Department of Epidemiology Research, Statens Serum Institut, Artillerivej 5, Copenhagen S, Denmark
Department of Epidemiology Research, Statens Serum Institut, Artillerivej 5, Copenhagen S, Denmark.
Eur Heart J. 2014 Aug 21;35(32):2158-64. doi: 10.1093/eurheartj/ehu157. Epub 2014 Apr 16.
To quantify the association between myotonic dystrophy (DM) and cardiac disease in a nationwide cohort.
We identified a nationwide cohort of 1146 DM patients (period 1977-2011) using the National Patient Registry (NPR) and a subcohort of 485 patients who had undergone genetic testing for DM1. Information on incident cardiac diseases was obtained from the NPR. We estimated standardized incidence ratios (SIRs) of cardiac disease compared with the background population, overall and according to selected diagnostic subgroups (cardiomyopathy, heart failure, conduction disorders, arrhythmias, and device implantation). In the DM cohort, SIR for any cardiac disease was 3.42 [95% confidence interval (CI) 3.01-3.86]; for a cardiac disease belonging to the selected subgroups 6.91 (95% CI: 5.93-8.01) and for other cardiac disease 2.59 (95% CI: 2.03-3.25). For a cardiac disease belonging to the selected subgroups, the risk was particularly high in the first year after DM diagnosis [SIR 15.4 (95% CI: 10.9-21.3)] but remained significantly elevated in subsequent years [SIR 6.07 (95% CI: 5.11-7.16]). The risk was higher in young cohort members [e.g. 20-39 years: SIR 18.1 (95% CI: 12.3-25.8)] compared with older [e.g. 60-79 years: SIR 3.99 (95% CI: 2.98-5.23)] but remained significantly increased in all age categories. Results were similar in separate analyses of the genetically confirmed DM1 patients.
Myotonic dystrophy is strongly associated with cardiac disease. The risk is pronounced in the young and remains elevated throughout life, stressing the importance of lifelong cardiac follow-up from time of DM diagnosis.
在全国范围内的队列中定量研究肌强直性营养不良（DM）与心脏病的关联。
我们使用国家患者登记处（NPR）确定了一个全国性的 DM 患者队列（1977 年至 2011 年），共 1146 例患者，同时还确定了 485 例经过 DM1 基因检测的患者亚组。从 NPR 中获取了关于新发心脏病的信息。我们总体上以及根据选定的诊断亚组（心肌病、心力衰竭、传导障碍、心律失常和器械植入），估计了心脏病的标准化发病比（SIR）。在 DM 队列中，任何心脏病的 SIR 为 3.42（95%置信区间[CI]：3.01-3.86）；属于选定亚组的心脏病 SIR 为 6.91（95%CI：5.93-8.01），其他心脏病的 SIR 为 2.59（95%CI：2.03-3.25）。属于选定亚组的心脏病，在 DM 诊断后的第一年风险特别高[SIR 15.4（95%CI：10.9-21.3）]，但在随后的几年中仍显著升高[SIR 6.07（95%CI：5.11-7.16）]。年轻队列成员的风险较高[例如，20-39 岁：SIR 18.1（95%CI：12.3-25.8）]，而年长队列成员的风险较低[例如，60-79 岁：SIR 3.99（95%CI：2.98-5.23）]，但在所有年龄组中，风险仍然显著增加。在对单独的基因确认 DM1 患者的分析中，结果相似。
肌强直性营养不良与心脏病密切相关。这种风险在年轻人中尤为明显，且终生存在，这凸显了从 DM 诊断开始进行终生心脏随访的重要性。
