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【目的】进一步深化对心内膜弹力纤维增生症(endocardial fibroelastosis,EFE)临床诊治的认识。【方法】对本院儿科5年来收治的32例EFE病例,结合病史、临床表现、辅助检查等进行回顾性分析。【结果】婴儿EFE多以呼吸道感染,尤其是下呼吸道感染为首发表现,常伴有突发心力衰竭。辅助检查可见胸片、心电图及心脏超声的改变。洋地黄类等药物的规范治疗对预后有明显影响。【结论】对于反复心衰而不能单纯用感染及先心解释的病例,要考虑到EFE可能。心脏超声是诊断EFE的重要依据。能否长期足量服用洋地黄类药物是影响预后的关键。
【Objective】 To further deepen the understanding of the clinical diagnosis and treatment of endocardial fibroelastosis (EFE). 【Methods】 A total of 32 cases of EFE admitted to our hospital during the past five years were retrospectively analyzed with history, clinical manifestations and laboratory tests. 【Results】 Most infants with respiratory tract infection, especially lower respiratory tract infection as the first manifestation, often accompanied by sudden heart failure. Auxiliary examination shows chest X-ray, ECG and cardiac ultrasound changes. Digitalis drugs such as the standard treatment has a significant impact on prognosis. [Conclusion] For cases of recurrent heart failure that can not be explained solely by infection and congenital heart disease, EFE may be considered. Echocardiography is an important basis for the diagnosis of EFE. The long-term enough to take digitalis drugs is the key to prognosis.