Barts Heart Centre, Institute for Cardiovascular Science, St Bartholomew's Hospital, University College London, London, United Kingdom.
Cardiology, Department of Experimental, Diagnostic, and Specialty Medicine, Alma Mater Studiorum University of Bologna, Bologna, Italy.
JAMA Cardiol. 2020 Jan 1;5(1):73-80. doi: 10.1001/jamacardio.2019.4534.
It is unclear whether hypertrophic cardiomyopathy (HCM) conveys excess mortality when compared with the general population.
To compare the survival of patients with HCM with that of the general European population.
DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study of 4893 consecutive adult patients with HCM presenting at 7 European referral centers between 1980 and 2013. The data were analyzed between April 2018 and August 2019.
Survival was compared using standardized mortality ratios (SMRs) calculated with data from Eurostat, stratified by study period, country, sex, and age, and using a composite end point in the HCM cohort of all-cause mortality, aborted sudden cardiac death, and heart transplant.
Of 4893 patients with HCM, 3126 (63.9%) were male, and the mean (SD) age at presentation was 49.2 (16.4) years. During a median follow-up of 6.2 years (interquartile range, 3.1-9.8 years), 721 patients (14.7%) reached the composite end point. Compared with the general population, patients with HCM had excess mortality throughout the age spectrum (SMR, 2.0, 95% CI, 1.48-2.63). Excess mortality was highest among patients presenting prior to the year 2000 but persisted in the cohort presenting between 2006 and 2013 (SMR, 1.84; 95% CI, 1.55-2.18). Women had higher excess mortality than men (SMR, 2.66; 95% CI, 2.38-2.97; vs SMR, 1.68; 95% CI, 1.52-1.85; P < .001).
Among patients referred to European specialty centers, HCM was associated with significant excess mortality through the life course. Although there have been improvements in survival with time, potentially reflecting improved treatments for HCM, these findings highlight the need for more research into the causes of excess mortality among patients with HCM and for better risk stratification.
与普通人群相比，肥厚型心肌病（HCM）是否会导致死亡率过高仍不清楚。
比较 HCM 患者的生存率与欧洲普通人群的生存率。
设计、地点和参与者：这是一项回顾性队列研究，纳入了 1980 年至 2013 年间在欧洲 7 个转诊中心就诊的 4893 例连续成年 HCM 患者。数据于 2018 年 4 月至 2019 年 8 月进行分析。
使用来自 Eurostat 的数据计算标准化死亡率比（SMR），根据研究期间、国家、性别和年龄对生存率进行分层，并使用 HCM 队列的全因死亡率、心源性猝死和心脏移植的复合终点来评估生存率。
4893 例 HCM 患者中，3126 例（63.9%）为男性，就诊时的平均（SD）年龄为 49.2（16.4）岁。中位随访 6.2 年（四分位间距，3.1-9.8 年）期间，721 例患者（14.7%）达到复合终点。与普通人群相比，HCM 患者在整个年龄范围内均存在死亡率过高的情况（SMR，2.0；95%CI，1.48-2.63）。在 2000 年前就诊的患者中，死亡率过高的情况最为严重，但在 2006 年至 2013 年就诊的患者中仍持续存在（SMR，1.84；95%CI，1.55-2.18）。女性的死亡率过高高于男性（SMR，2.66；95%CI，2.38-2.97；与 SMR，1.68；95%CI，1.52-1.85；P<.001）。
在转诊至欧洲专科中心的患者中，HCM 与全生命周期的死亡率过高显著相关。尽管随着时间的推移生存率有所提高，这可能反映了对 HCM 的治疗有所改善，但这些发现突出表明需要进一步研究 HCM 患者死亡率过高的原因，并需要更好的风险分层。
