Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries
Sudden death in cardiac sarcoidosis: an analysis of nationwide clinical and cause-of-death registries
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DOI：
10.1093/eurheartj/ehz428
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发表时间：
2019-10-01
影响因子：
39.3
通讯作者：
Kupari, Markku
中科院分区：
文献类型：
--
作者：
Ekstrom, Kaj;Lehtonen, Jukka;Kupari, Markku
目的-本研究是为了评估心脏性猝死（SCD）的作用，从心脏结节病（CS）的表现和死亡率之间的方法和结果-我们共分析了351例CS从1998年到2015年在芬兰。有262例患者临床诊断和治疗CS，27例患者最初终生诊断为巨细胞心肌炎，后来转化为CS，62例在尸检时发现，并通过筛选来自国家死因登记处的>820 000份死亡证明来确定。总病例系列包括253名女性和98名男性，就诊时平均年龄为52岁。高度房室传导阻滞是CS最常见的首发症状（n = 147，42%），其次是心力衰竭（n = 58，17%）、非预期致死性（n = 38）或流产（n = 12）SCD（14%）和持续性室性心动过速（n = 48，14%）。在38例出现致死性SCD的病例中，有4例在尸检时发现严重冠状动脉疾病伴CS。在截至2015年底记录的所有死亡中，64%（n = 54/84）是CS的非预期SCD，这些SCD在生命中一直保持沉默或无法进行所有诊断。Kaplan-Meier估计（95%CI）症状发作后5年生存率为85%（80-90%），10年生存率为76%（68- 84%.Conclusion致死性和流产性SCD共占CS临床表现的14%。近三分之二的CS死亡是由心脏中未诊断的肉芽肿引起的。
Aims The present study was done to assess the role of sudden cardiac death (SCD) among the presenting manifestations of and fatalities from cardiac sarcoidosis (CS).Methods and results We analysed altogether 351 cases of CS presenting from year 1998 through 2015 in Finland. There were 262 patients with a clinical diagnosis and treatment of CS, 27 patients with an initial lifetime diagnosis of giant cell myocarditis that was later converted to CS, and 62 cases detected at autopsy and identified by screening >820 000 death certificates from the national cause-of-death registry. The total case series comprised 253 females and 98 males aged on average 52 years at presentation. High-grade atrioventricular block was the most common first sign of CS (n = 147, 42%) followed by heart failure (n = 58, 17%), unexpected fatal (n = 38) or aborted (n = 12) SCD (14%), and sustained ventricular tachycardia (n = 48, 14%). Severe coronary artery disease was found at autopsy concomitant with CS in four of the 38 cases presenting with fatal SCD. Of all deaths recorded till the end of 2015, 64% (n = 54/84) were unexpected SCDs from CS that had either been silent during life or defied all attempts at diagnosis. The Kaplan-Meier estimate (95% CI) of survival from symptom onset was 85% (80-90%) at 5 years and 76% (68-84%) at 10 years.Conclusion Together fatal and aborted SCD constitute 14% of the presenting manifestations of CS. Nearly two-thirds of all fatalities from CS are caused by undiagnosed granulomas in the heart.