小儿心内膜弹力纤维增生症血浆脑钠肽/N端脑钠肽前体与左室射血分数的相关性研究

Analysis of relationship between BNP / NT pro-BNP and LVEF in patients with endocardial fibroelastosis

目的:通过对心内膜弹力纤维增生症(endocardial fibroelastosis,EFE)患儿血浆脑钠肽(brain natrium peptide,BNP)、N端脑钠肽前体(N-terminal pro-BNP,NT-pro-BNP)的动态观察以及其与左室射血分数(left ventricular ejection fraction,LVEF)的相关性,来评估其在EFE转归和预后判定中的价值。方法:(1)病例选择:选自中国医科大学附属盛京医院2009年1月初至2013年4末住院的EFE患儿共46例,所有患儿均符合EFE相关诊断标准。(2)药物治疗:所有患儿均在强心、利尿并口服糖皮质激素基础上加用丙种球蛋白治疗,对治疗6个月疗效不佳者,加服环磷酰胺口服。(3)随访项目:血清离子、心肌标记物、肝肾功能和BNP、NT-pro-BNP、心电图、心脏超声加左心功能测定。结果:全组46例患儿,男24例,女22例,平均发病年龄9个月,平均随访时间24个月。全组初诊EFE时血浆BNP、NT-pro-BNP中位数及上下四分位间距分别为:BNP 2 037.5(387.2,4 489.0)pg·ml-1,NT-pro-BNP 6 565.5(3 696.5,9 992.2)pg·ml-1。随心功能恢复其值逐渐下降,分别于治疗6个月、12个月时其中位数值降至正常范围。全组LVEF中位数于治疗6个月后升高至50%以上,于24个月时恢复正常范围(60%)。血浆BNP及NT-pro-BNP水平与LVEF均呈负相关,回归方程分别为Y=-71.2X+3 451,r=-0.46和Y=-136.30X+8 509.04,r=-0.70。结论:血浆BNP、NT-pro-BNP水平在EFE治疗前均升高,且与LVEF呈负相关;BNP、NT-pro-BNP检测可结合患儿临床症状及心脏超声、心电图等共同应用于EFE的疗效和预后的评估。

Objective：To evaluate the significant of BNP/NT-pro-BNP and the relationship between them and the LVEF in patients with endocardial fibroelastosis （EFE）. Methods：（1） Choose patients：From January 2009 to April 2013,total 46 EFE children were selected. （2） Treatment：Gamma globulin intravenous IVIG（intravenous immunoglobulin）,at the same time took captopril, prednisone or medrol,digoxin,uragogue.To those patients who had taken medicine regularly over 6 months and LVEF were still less than 30% additionally took cyclophosphamide （CTX）. （3） Follow-up：cardiac functional grading, BNP/NT-pro-BNP, the electrolyte concentration, myocardial enzyme, troponin T, liver and kidney function test, ECG, pulse Doppler electrocardiograph（PDE） including LVEF and left ventricular diastolic diameter（LVED） were recorded at each time. Results：The patients were male 24 and female 22, the average age was 9 months.The median of BNP/NT-pro-BNP levels were 2037.5（387.2, 4489.0）pg·ml^-1 and 6565.5（3 696.5, 9992.2）pg·ml^-1 before treatment. It recovered at 6 and 12 months. The correlation between BNP/NT-pro-BNP and LVEF were negative. The relevant regression equation between BNP and LVEF was Y=-71.2X＋3 451, r=-0.46, while between NT-pro-BNP and LVEF was Y=-136.30X＋8509.04,r=-0.70. Conclusions：BNP/NT-pro-BNP increase before treatment of EFE. There are negative correlation between BNP/NT-pro-BNP and LVEF. The former and clinical symptoms,PDE,ECG can be used to evaluate the cardiac function in EFE patients.