Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan.
Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, Japan.
J Cardiol. 2022 Dec;80(6):557-562. doi: 10.1016/j.jjcc.2022.07.016. Epub 2022 Aug 10.
We reviewed the long-term outcome of children with hypertrophic cardiomyopathy (HCM) based on the type.
We 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.
The 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.
The 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.
我们根据肥厚型心肌病（HCM）的类型回顾了儿童患者的长期预后。
我们回顾了 1977 年至 2015 年期间我院 100 例 HCM 患者（男 54 例，女 46 例）的病历。通过 Kaplan-Meier 法计算生存率和无心脏事件生存率。
诊断时年龄为 0 至 15 岁，中位数为 8 岁。患有 Noonan 综合征和肥厚型梗阻性心肌病（HOCM）、特发性肥厚型心肌病（i-HCM）和继发性肥厚型心肌病（s-HCM）的患者分别为 13、13、65 和 9 例。24 例患者出现扩张期 HCM。24 例患者中，19 例（79%）死于心力衰竭，2 例接受心脏移植。8 例（33%）为 s-HCM。扩张期发生的中位年龄为 15 岁，从初始诊断到扩张期的中位间隔为 8 年。从扩张期诊断到死亡的中位时间为 1.6 年。6 例和 11 例患者分别在 15 岁左右发生猝死和植入式心脏复律除颤器植入。20 年生存率如下：Noonan 综合征 84%；HOCM 82%；i-HCM 71%；s-HCM 17%。总体而言，10、20 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、20 和 30 年的无心脏事件生存率分别为 57%（47-67%）、39%（31-50%）和 32%（21-44%）。
HCM 儿童的长期预后较差，s-HCM 的预后非常差。扩张期的发生使 HCM 患者的预后恶化。猝死和扩张型 HCM 常发生在 15 岁左右。
