Thirty-year outcome in children with hypertrophic cardiomyopathy based on the type
Thirty-year outcome in children with hypertrophic cardiomyopathy based on the type
复制标题
肥厚型心肌病儿童三十年结局（基于类型）
DOI：
10.1016/j.jjcc.2022.07.016
复制
发表时间：
2022
影响因子：
2.5
通讯作者：
Kurosaki Kenichi
中科院分区：
文献类型：
--
作者：
Tsuda Etsuko;Ito Yuki;Kato Yoshiaki;Sakaguchi Heima;Ohuchi Hideo;Kurosaki Kenichi
BackgroundWe reviewed the long-term outcome of children with hypertrophic cardiomyopathy（HCM）based on the type.MethodsWe reviewed the medical records of 100 patients（male 54 female 46）with HCM at our hospital between 1977 and 2015. Kaplan-Meier法计算生存率和无心脏事件生存率。结果诊断时的年龄范围为0 ~ 15岁，中位数为8年。努南综合征和肥厚性梗阻性心肌病（HOCM）、特发性HCM（i-HCM）和继发性HCM（s-HCM）的患者数量分别为13、13、65和9例。24例患者出现扩张期HCM。24例患者中有19例（79%）死于心力衰竭，2例接受了心脏移植。24例患者中有8例（33%）患有s-HCM。发生扩张期的中位年龄为15岁，从首次诊断到扩张期的中位间隔为8年。从诊断出扩张期到死亡的中位时间为1.6年。15岁左右猝死6例，植入型心律转复除颤器11例。20年生存率如下：努南综合征84%; HOCM 82%; i-HCM 71%;和s-HCM 17%。总体而言，10年、20年和30年的生存率分别为83%（95%置信区间73-89）、69%（58-78）和63%（50-74）。10年、20年和30年的总体无心脏事件生存率分别为57%（47-67）、39%（31-50）和32%（21-44），分别为ConclusionThe long-term outcome in children with HCM is poor，and the outcome of s-HCM is very poor.扩张期的发生使HCM患者的结局恶化。猝死和d-HCM多发生在15岁左右。
BackgroundWe reviewed the long-term outcome of children with hypertrophic cardiomyopathy (HCM) based on the type.MethodsWe reviewed the medical records of 100 patients (male 54 female 46) with HCM at our hospital between 1977 and 2015. The survival and cardiac event-free survival rates were calculated by the Kaplan–Meier method.ResultsThe age at the time of the diagnosis ranged from 0 to 15 years with a median of 8 years. The number of patients with Noonan syndrome and hypertrophic obstructive cardiomyopathy (HOCM), idiopathic HCM (i-HCM), and secondary HCM (s-HCM) was 13, 13, 65, and 9 respectively. A dilated phase of HCM occurred in 24 patients. Nineteen (79 %) of the 24 patients died of heart failure, and two underwent a heart transplantation. Eight (33 %) of the 24 patients had s-HCM. The median age when a dilated phase occurred was 15 years old, and the median interval from the initial diagnosis to the dilated phase was 8 years. The median time from the diagnosis of a dilated phase to death was 1.6 years. Sudden death and implantable cardioverter defibrillator implantations occurred in 6 and 11 patients at around 15 years old, respectively. The 20-year survival rates were as follows: Noonan syndrome 84 %; HOCM 82 %; i-HCM 71 %; and s-HCM 17 %. Overall, the survival rates at 10, 20, and 30 years were 83 % (95 % confidence interval 73–89), 69 % (58–78), and 63 % (50–74), respectively. The overall cardiac event-free survival rates at 10, 20, and 30 years were 57 % (47–67), 39 % (31–50), and 32 % (21–44), respectively.ConclusionThe long-term outcome in children with HCM was poor, and the outcome of s-HCM was very poor. The occurrence of a dilated phase worsened the outcome in HCM patients. Sudden death and d-HCM often occurred at around 15 years old.