Supravalvular Aortic Stenosis and the Risk of Premature Death Among Patients With Homozygous Familial Hypercholesterolemia.
Supravalvular Aortic Stenosis and the Risk of Premature Death Among Patients With Homozygous Familial Hypercholesterolemia.
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DOI：
10.1016/j.amjcard.2020.12.080
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发表时间：
2021-04-15
期刊：
影响因子：
--
通讯作者：
Yang, Ya
中科院分区：
文献类型：
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作者：
Zhang, Ruiying;Xie, Jinjie;Yang, Ya
纯合子家族性高胆固醇血症(HoFH)患者过早死亡的风险很高。主动脉瓣上狭窄(SVAS)是HoFH常见的主动脉根部病变。SVAS与HoFH患者过早死亡风险的关系尚未得到充分研究。本研究分析了97例HOFH患者，平均年龄14.7岁，来自汉族家族性高胆固醇血症的遗传和影像研究。在中位数(±SD)随访4.0(±4.0)年期间，40名(41.2%)参与者有SVAS，17名(17.5%)参与者死亡。非SVAS组和SVAS组过早死亡的比例分别为7.0%和32.5%。与非SVAS组相比，SVAS组在HoFH中的累积生存期较低(log-ranch检验，p
Patients with homozygous familial hypercholesterolemia (HoFH) have a high risk for premature death. Supravalvular aortic stenosis (SVAS) is a common and the feature lesion of the aortic root in HoFH. The relation between SVAS and the risk of premature death in patients with HoFH has not been fully investigated. The present study analysis included 97 HoFH patients with mean age of 14.7 (years) from the Genetic and Imaging of Familial Hypercholesterolemia in Han Nationality Study. During the median (±SD) follow-up 4.0 (±4.0) years, 40 (41.2%) participants had SVAS and 17 (17.5%) participants experienced death. The proportion of premature death in the non-SVAS and SVAS group was 7.0% and 32.5%, respectively. Compared with the non-SVAS group, SVAS group cumulative survival was lower in the HoFH (log-rank test, p