儿童扩张型心肌病68例临床分析

Clinical assessment of dilated cardiomyopathy in 68 children

摘要:

目的：探讨扩张型心肌病（dilated cardiomyopathy，DCM）患儿的临床特征，提高对该病的认识。方法：回顾性分析重庆医科大学附属儿童医院2012年1月1日至2018年12月31日住院诊断为DCM患儿的临床资料，包括发病年龄、临床症状、辅助检查、预后情况等。结果：68例DCM患儿中男女比1∶1.125，平均发病年龄（86.6±47.6）个月，其中1～6岁儿童发病率最高（39.7%）；发病时心功能Ⅰ级2.9％，Ⅱ级26.5％，Ⅲ级38.2％，Ⅳ级32.4％；心脏超声检查示左室射血分数（left ventricular ejection fraction，LVEF）为（36.4±9.5）％，左室短轴缩短率（left ventricular fraction shortening，LVFS）为（20±5.3）％；随访1～84个月，其中好转30例（44.1％），恶化5例（7.4％），病情稳定14例（20.6％），死亡12例（17.6％），失访7例（10.3％）；第1年、第2年、第3年及第5年生存率分别为80.9％、69.4％、63.4％及62.9％；不同预后组间比较起病时LVEF及LVFS值差异具有统计学意义（LVEF：F=3.920，P=0.019；LVFS：F=4.720，P=0.005）；死亡组及恶化组室性心律失常发生率较其他组更高。结论：儿童DCM发病年龄较小，起病时心功能分级、LVEF值及室性心律失常与预后相关，长期随访对儿童DCM有重要意义。

Abstract:

Objective：To investigate clinical characteristics of dilated cardiomyopathy（DCM） of children，and improve the awareness of it. Methods：The clinical characteristics，including onset age，symptoms，examinations and prognosis，of patients diagnosed as DCM in Children’s Hospital of Chongqing Medical University，from January 1，2012 to December 31，2018，were retrospectively analyzed. Results：Totally，a sum of 68 children were enrolled，with a ratio of 1∶1.125 between male and female. The mean onset age was （86.6±47.6） months，and the incidence was highest in children aged 1 to 6 years，accounting for 39.7%. When onset，the functional class（FC） was evaluated as Ⅰ（2.9%），Ⅱ（26.5%），Ⅲ（38.2%） and Ⅳ（32.4%）. The echocardiography displayed that left ventricular ejection fraction（LVEF） was （36.4±9.5）%， and left ventricular shortening fraction（LVFS） was （20±5.3）%. Patients were followed up for 1 to 84 months，among which 30 cases（44.1%） were improved，5 cases（7.4%） deteriorated，14 cases（20.6%） were in stable condition，12 cases（17.6%） died，and 7 cases（10.3%） lost. The survival rates at the first，second，third and fifth year were 80.9%，69.4%，63.4% and 62.9%，respectively. The initial LVEF and LVFS varies，with a statistical difference，from groups with different prognosis（LVEF：F=3.920，P=0.019；LVFS：F=4.720，P=0.005）. The incidence of ventricular arrhythmias was higher in the death group and the worsening group than that in other groups. Conclusion：It concludes that DCM occurs in children at an early age. FC，LVEF and the incidence of ventricular arrythmias at baseline were significantly correlated with prognosis. Additionally，a long-term follow-up is important for children with DCM.