The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporarymanagement of adult patients with cardiomyopathies
The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporarymanagement of adult patients with cardiomyopathies
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DOI：
10.1093/eurheartj/ehx819
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发表时间：
2018-05-21
影响因子：
39.3
通讯作者：
Maggioni, Aldo P.
中科院分区：
文献类型：
--
作者：
Charron, Philippe;Elliott, Perry M.;Maggioni, Aldo P.
目的：欧洲观察性研究计划的心肌病登记是一项前瞻性、观察性和多国登记研究，包括4种心肌病亚型的连续患者：肥厚型心肌病（HCM）、扩张型心肌病（DCM）、致动脉瘤性右心室心肌病（ARVC）和限制型心肌病（RCM）。我们报告的基线特征和管理的成年人登记registration.Methods和结果共3208例患者在18个国家的69个中心参加[HCM（n = 1739）; DCM（n = 1260）; ARVC（n = 143）;和RCM（n = 66）]。在诊断时的年龄、家族性疾病史、持续性室性心律失常史、磁共振成像或基因检测的使用以及植入起搏器方面，观察到心肌病亚型之间的差异（P < 0.001）。与先证者相比，亲属的诊断年龄较低（P < 0.001），但症状和发病率相似。与长期治疗期相比，试验期患者（入组更多专家中心）的家族性疾病发生率更高（P < 0.001），更常诊断为罕见基础疾病（P < 0.001），更常植入除颤器（P = 0.023）。比较四个地理区域，来自南欧的患者更频繁地患有家族性疾病（P < 0.001），在家庭筛查中更常被诊断（P < 0.001），更常诊断为罕见的基础疾病结论通过对心肌病患者的特点和治疗提供当代观察资料，登记册为评价准则执行情况提供了一个平台。与现有的建议，以及一些建议，以改善在欧洲的医疗保健提供潜在的差距进行了讨论。
Aims The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry.Methods and results A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001).Conclusion By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.