Contemporary survival trends and aetiological characterization in non-ischaemic dilated cardiomyopathy

Contemporary survival trends and aetiological characterization in non-ischaemic dilated cardiomyopathy
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DOI:
10.1002/ejhf.1914
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发表时间:
2020-06-26
影响因子:
18.2
通讯作者:
Sinagra, Gianfranco
Sinagra, Gianfranco
中科院分区:
医学1区
文献类型:
--
作者:
Merlo, Marco;Cannata, Antonio;Sinagra, Gianfranco

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目的目前扩张型心肌病(DCM)的生存趋势尚不清楚。本研究的目的是调查扩张型心肌病患者的临床描述因素、生存趋势和病因学特征对预后的影响。方法与结果连续入选扩张型心肌病患者,按入选时间分为4组(1978~1984年、1985~1994年、1995~2004年、2005~2015年)。还分析了2005至2015年间登记的特定病因学DCM患者的子集。在平均12+/-8年的随访中,评估了1284名DCM患者(1978-1984年组52例,1985-1994年组326例,1995-2004年组379例,2005-2015年组527例)。尽管年龄较大(1978年至1984年、1985年至1994年、1995年至2004年和2005年至2015年组的平均年龄分别为51+/-15岁、43+/-15岁、45+/-14岁和52+/-15岁;P<0.001),但大多数基线临床特征在2005年-2015年组中有所改善,表明诊断时疾病阶段较晚。同样,在竞争风险分析中,所有结果参数的年度发生率随着时间的推移而逐渐减少(全球P<0.001)。在多变量分析中,最后一次登记与全因死亡率/心脏移植(HTX)/心脏辅助装置(VAD)植入(1.46个事件/100名患者/年)、心血管死亡/HTX/VAD植入(0.82个事件/100名患者/年)和心脏性猝死(0.15个事件/100名患者/年)独立相关。最后,在287例特定病因的扩张型心肌病患者中,与遗传性、心肌炎后遗症或特发性扩张型心肌炎患者相比,环境、毒性或可移除因素的患者的表型和预后似乎不同(P<0.001)。结论当代DCM患者的生存趋势显著改善,主要是由于心血管事件的减少。适当的病因学特征可能有助于DCM患者的预后。
Aim Contemporary survival trends in dilated cardiomyopathy (DCM) are largely unknown. The aim of this study is to investigate clinical descriptors, survival trends and the prognostic impact of aetiological characterization in DCM patients. Methods and results Dilated cardiomyopathy patients were consecutively enrolled and divided into four groups according to the period of enrolment (1978-1984; 1985-1994; 1995-2004; and 2005-2015). A subset of patients with DCM of specific aetiology, enrolled from 2005 to 2015, was also analysed. Over a mean follow-up of 12 +/- 8 years, 1284 DCM patients (52 in the 1978-1984 group, 326 in the 1985-1994 group, 379 in the 1995-2004 group, and 527 in the 2005-2015 group) were evaluated. Despite older age (mean age 51 +/- 15, 43 +/- 15, 45 +/- 14, and 52 +/- 15 years for the 1978-1984, 1985-1994, 1995-2004, and 2005-2015 groups, respectively;P < 0.001), most of the baseline clinical characteristics improved in the 2005-2015 group, suggesting a less advanced disease stage at diagnosis. Similarly, at competing risk analysis, the annual incidence of all outcome parameters progressively decreased over time (globalP < 0.001). At multivariable analysis, the last period of enrolment emerged as independently associated with a reduction in all-cause mortality/heart transplantation (HTx)/ventricular assist device (VAD) implantation (1.46 events/100 patients/year), cardiovascular death/HTx/VAD implantation (0.82 events/100 patients/year) and sudden cardiac death (0.15 events/100 patients/year). Lastly, in 287 patients with DCM of specific aetiology, patients with environmental, toxic, or removable factors appeared to have different phenotypes and prognosis compared to those with genetic, post-myocarditis, or idiopathic DCM (P < 0.001). Conclusions Contemporary survival trends in DCM significantly improved, mainly due to a reduction of cardiovascular events. Appropriate aetiological characterization might help in prognostication of DCM patients.