Objective To summarize the clinical characteristics of pediatric hypertrophic cardiomyopathy and analyze its etiology for providing guidance for early identification,diagnosis and prognosis.Methods Fifty-two cases of pediatric hypertrophic cardiomyopathy admitted to Guangzhou Women and Children's Medical Center from January 2012 to June 2018 were retrospectively analyzed and summarized from the aspects of age,gender,family history,clinical features,auxiliary examination,etiology,drug efficacy and disease outcome.Results (1) There were 52 cases in this group including 36 males and 16 females.The ages of patients ranged from 15 days to 14 years (with mean age of 27.7 months,median 6.5 months).A total of 34 patients (65.4％) were followed up for 1-78 months (mean 30.6 months).Echocardiography showed 52 cases of left ventricular wall thickening (100％),21 cases of double ventricular hypertrophy (40.4％),18 cases of left ventricular outflow tract obstruction (34.6％),and 18 cases of hepatic enzyme elevation (34.6％).The etiology of 11 cases was clear (21.2％),including 7 cases of type Ⅱ glycogen accumulation,3 cases of Noonan syndrome and 1 case of primary carnation deficiency.No routine heart transplantation was performed at the end of follow-up,and 12 patients (35.3％) died,7 cases of whom died in infancy.Conclusions Children with hypertrophic cardiomyopathy have a relatively young age,so it is necessary to search for the etiology actively,carry out disease risk assessment,and conduct personalized management and treatment.

目的 探讨小儿缩窄性心包炎的临床及病理学特征。 方法 对广州医科大学附属广州市妇女儿童医疗中心2003年1月至2015年12月诊断为缩窄性心包炎患儿的临床资料及病理学检查结果进行回顾性分析。 结果 缩窄性心包炎患儿共15例，年龄1岁4个月～14岁，平均6岁。病程2周～36个月。临床表现主要有腹胀(11例)、水肿(9例)、气促(7例)、腹痛(7例)、乏力(5例)、咳嗽(3例)、尿少(1例)等。心脏彩超检查提示双心房扩大7例，三尖瓣反流6例，二尖瓣反流5例，心包膜增厚4例，下腔静脉增宽3例，右心房内实质性占位性病变1例；心脏CT发现心包增厚10例，心包钙化4例。经外科手术治疗10例，手术方式为心包剥脱术，1例曾因疑诊缩窄性心包炎行心包开窗术。10例手术患儿总住院时间11～57 d，平均29 d；术后重症监护病房住院时间3～10 d，平均5.5 d；术后恢复顺利，无死亡病例。术后心包病理学检查结果均为非特异性炎症，表现为纤维结缔组织增生、玻璃样变、小血管增生、淤血及出血、纤维素渗出或沉积、淋巴细胞或中性粒细胞浸润及钙化灶。随访1～2年，除1例因复发再次手术，其余患儿心功能均改善，临床症状消失，生长发育恢复正常。 结论 小儿缩窄性心包炎临床症状不典型，超声心动图检查阳性率低，心脏CT诊断符合率高，病理学多为非特异性炎症，心包剥脱术是主要治疗手段，对于疑诊病例，心包开窗术不失为一种可取的过渡性治疗。