原发性心内膜弹力纤维增生症预后相关因素分析

An analysis of the factors related to the prognosis of primary endocardial fibroelastosis

摘要:

目的：探讨原发性心内膜弹力纤维增生症（endocardial fibroelastosis，EFE）的临床特征及影响其预后的相关因素。方法：纳入2008年1月至2018年4月在重庆医科大学附属儿童医院心内科住院的EFE患儿150例（男52例，女98例），收集其起病年龄及体质量、临床症状、个人史、辅助检查等。其中出院后随访92例，根据随访时心功能及辅助检查结果分为缓解组（n=75）与恶化组（n=17），采用卡方检验、U检验进行统计学分析，采用logistic回归分析起病年龄及体质量、母孕期感染史、初诊心功能、心胸比、是否丙种球蛋治疗、随访时间与预后的关系。结果：①150例患儿起病中位年龄为163 d，中位体质量为7 kg，71.33%的患者2周内有前驱感染，72%以呼吸道症状起病，71.33%心功能Ⅰ级到Ⅱ级，28.67%心功能Ⅲ级，31.33%初诊心胸比≥0.70。心脏超声心内膜厚度中位数为2.5 mm，左室舒张期末内径（left ventricular end-diastolic diameter，LVDd）平均值为42.09 mm，左室短轴缩短率（left ventricular fraction shortening，LVFS）中位数为18%，左室射血分数（left ventricular ejection fraction，LVEF）平均值为38%。55.33%病毒抗体阳性。②92例出院后规律随访患儿中，缓解组与恶化组间起病年龄、随访时间、心胸比差异均有统计学意义（均P＜0.05）。性别、母孕期感染史、初诊心功能分级、丙种球蛋白治疗在两组间差异均无统计学意义（均P ＞0.05）。采用二元logistic回归矫正性别、起病年龄、有无母孕期感染史、初诊心功能分级、是否丙种球蛋白治疗等混杂因素后，初诊心胸比小于0.7的患儿缓解概率明显升高（OR=6.89，95%CI=1.54~25.68），规律随访时间小于2年的患儿缓解概率明显降低（OR=0.11，95%CI=0.03~0.49）。结论：EFE多发于婴幼儿，大部分患儿有前驱感染史或母孕期有感染史。初诊时心胸比小于0.7及随访时间大于2年为该病缓解的保护因素。

Abstract:

Objective：To investigate the clinical features of primary endocardial fibroelastosis（EFE） and the factors affecting its prog－nosis. Methods：A total of 150 children with EFE（52 males and 98 females） who were hospitalized in the Department of Cardiology in Children’s Hospital of Chongqing Medical University from January 2008 to April 2018 were enrolled. The age，body weight，clinical symptoms，personal history，and auxiliary examinations of the children at disease onset were collected. Among these patients，92 were followed up after discharge from the hospital and were divided into remission group（n=75） and deterioration group（n=17） according to their cardiac function and auxiliary examination results at follow-up. The chi-square test and U test were used for statistical analysis. Logistic regression was used to analyze the association of age and body weight of the patients at disease onset，maternal infection his－tory，cardiac function，and cardiothoracic ratio at initial diagnosis，status of gamma-globulin treatment，and duration of follow-up with prognosis. Results：The median age and median body weight of the 150 children at disease onset were 163 days and 7 kg，respectively；71.33% of the patients had a prodromal infection within two weeks，72% had respiratory symptoms as their earliest manifestations，71.33% had a cardiac function of grade Ⅰ-Ⅱ，28.67% had a cardiac function of grade Ⅲ，and 31.33% had a cardiothoracic ratio of ≥0.70 at initial diagnosis. The median endocardial thickness，mean LVDd，median LVFS，and mean LVEF were 2.5 mm，42.09 mm，18%，and 38%，respectively. Fifty-five point three-three percent of the patients had positive viral antibody test results. Among the 92 children being regularly followed up，there were signifi－cant differences in age at disease onset，duration of follow-up，and cardiothoracic ratio between the remission group and the deterioration group（P<0.05），but there were no significant differences in sex，maternal infection history，cardiac functional grading at initial diagnosis，or status of gamma-globulin treatment between the two groups（P>0.05）. After correction of the confounding factors such as sex，age at disease onset，maternal infection history，cardiac functional grading at initial diagnosis，and status of gamma-globulin treatment using binary logistic regression，the percentage of remitted children with a cardiothoracic ratio of <0.7 at initial diagnosis increased significantly（OR=6.89，95%CI=1.54 to 25.68），and the percentage of remitted children with a regular follow-up of <2 years decreased significantly（OR=0.11，95%CI=0.03 to 0.49）. Conclusion：EFE is prevalent in infants and young children，most of whom have a history of prodromal infection or maternal infection. An initial cardiothoracic ratio of <0.7 and a follow-up of >2 years are protective factors for remission of the disease.