原发性心内膜弹力纤维增生症心脏舒张功能障碍及其与收缩功能障碍的关系

Diastolic myocardial dysfunction and its relationship with systolic myocardial dysfunction in primary endocardial fibroelastosis

目的:探讨原发性心内膜弹力纤维增生症(EFE)患儿是否存在主动舒张功能障碍及其与收缩功能障碍的关系。方法:EFE患儿44例,正常对照组50例。超声常规测量左室舒张末内径(LVEDD)、左房内径(LAD)、左室射血分数(LVEF)、左室舒张末容积(LVEDV)、E峰流速(E)、A峰流速(A)和左室等容舒张时间(IVRT)。结果:仅有舒张功能障碍7例,收缩功能障碍和舒张功能障碍并存37例。主动舒张功能障碍,即IVRT≥65 m s者17例,IVRT<65 m s 27例,两组比较,前者LVEF高、LAD小、E低、A高和E/A大(P<0.05)。IVRT≥65 m s时,控制变量LVEF情况下,即IVRT与LVEDD呈正相关(P<0.05),未发现LVEF与E/A或E相关(P>0.05)。以IVRT 70 m s为分界点分成两组,IVRT≥70 m s组较<70 m s组LVEDD大(P<0.05),而LVEF无显著差异。结论:EFE存在主动舒张功能障碍,随着舒张功能障碍加重,收缩功能下降加剧。

Objective To investigate diastolic myocardial dysfunction and it＇s relationship with systolic myocardial dysfunction in endocardial fibroelastosis（EFE）. Methods Cardiac structure, left ventriclar diastolic and systolic function in 44 children with endocardial fibroelastosis and 50 normal subjects were analyzed by echocardiography. Left ventricular end-diastolic dimension （LVEDD）, left atrial dimension （LAD）, left ventricular ejection fraction （LVEF）, left ventricular end-diastolic volume （LVEDV）, E peak velocity （VE）, A peak velocity （VA）, isovolumic relaxing time （IVRT） were assessed. Results According to the result of the echocardiography,7 children only had diastolic dysfunction, 37 children had both diastolic and systolic dysfunction. Systolic dysfunction was diagnosed according to IVRT. Systolic dysfunction group whose IVRT≥65 ms including 17 children and IVRT 〈 65 ms group including 27 children. In the comparison of the two groups, the former had a higher LVEF, lower LAD, down regulated VE, up regulated VA and E/A ratio. IVRT had significant positive correlation with LVEDD when LVEF was controlled （ P 〈 0. 05 ）, no significant difference was observed between LVEF and E/A or E （P 〉 0. 05 ）. If IVRT = 70 ms was set up as a separation point, the group with a IVRT ≥70 ms had a higher LVEDD than that with a IVRT 〈 70 ms （ P 〈 0.05 ）, but no significant difference was found in LVEF. Conclusion Diastolic myocardial dysfunction systolic exists in endocardial fibroelastosis. IVRT has a significantly positive correlation with LVEDD, systolic dysfunction reduced dramatically with diastolic heart failure severity.