Clinical analysis of 52 cases of pediatric hypertrophic cardiomyopathy / 中华实用儿科临床杂志

ABSTRACT

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.