CAJ | 학술논문

目的观察原发性心内膜弹力纤维增生症(endocardial fibroelastosis,EFE)患儿血浆脑利钠肽(brain natriuretic peptide,BNP)变化及卡维地洛的临床疗效,探讨治疗剂量、方案及安全性.方法 21例原发性EFE患儿分为2组,常规治疗组(n=10)及卡维地洛治疗组(n=11),治疗前及治疗后6个月进行多普勒超声心动图检测、观察血浆BNP;观察2组治疗前后血浆BNP水平、临床症状、心胸比例、心率的变化、心功能的改善、患儿对卡维地洛的耐受量及不良反应.结果原发性EFE患儿血浆BNP水平[(865±702)ng/L]明显高于正常对照组[(154±78)ng/L](P＜0.01),且心功能越差升高越明显,心功能Ⅱ、Ⅲ、Ⅳ级分别为(286±125)ng/L、(437±386)ng/L、(1673-s-859)ng/L;卡维地洛治疗后血浆BNP水平[(219±87)ng/L]低于常规组[(403±216)ng/L](P＜0.05),临床症状明显改善,患儿心胸比例降低,心率减慢,射血分数(EF)、短轴缩短率、平均左室周径缩短率显著提高(P＜0.01),左室收缩末期内径(P＜0.05)、左室质量、室间隔收缩末厚度明显降低(P＜0.01).结论原发性EFE患儿血浆BNP水平显著升高,与EF呈负相关;卡维地洛可以降低血浆BNP水平,明显抑制EFE患儿心室重塑,改善患儿的心功能;心功能Ⅱ～Ⅳ级原发性EFE患儿卡维地洛长期治疗安全有效.
목적 관찰원발성심내막탄력섬유증생증(endocardial fibroelastosis,EFE)환인혈장뇌리납태(brain natriuretic peptide,BNP)변화급잡유지락적림상료효,탐토치료제량、방안급안전성.방법 21례원발성EFE환인분위2조,상규치료조(n=10)급잡유지락치료조(n=11),치료전급치료후6개월진행다보륵초성심동도검측、관찰혈장BNP;관찰2조치료전후혈장BNP수평、림상증상、심흉비례、심솔적변화、심공능적개선、환인대잡유지락적내수량급불량반응.결과 원발성EFE환인혈장BNP수평[(865±702)ng/L]명현고우정상대조조[(154±78)ng/L](P＜0.01),차심공능월차승고월명현,심공능Ⅱ、Ⅲ、Ⅳ급분별위(286±125)ng/L、(437±386)ng/L、(1673-s-859)ng/L;잡유지락치료후혈장BNP수평[(219±87)ng/L]저우상규조[(403±216)ng/L](P＜0.05),림상증상명현개선,환인심흉비례강저,심솔감만,사혈분수(EF)、단축축단솔、평균좌실주경축단솔현저제고(P＜0.01),좌실수축말기내경(P＜0.05)、좌실질량、실간격수축말후도명현강저(P＜0.01).결론 원발성EFE환인혈장BNP수평현저승고,여EF정부상관;잡유지락가이강저혈장BNP수평,명현억제EFE환인심실중소,개선환인적심공능;심공능Ⅱ～Ⅳ급원발성EFE환인잡유지락장기치료안전유효.
Objective Endecardial fibroelastosis(EFE),a common pediatric cardiovascular disense.often results in chronic heart failure(CHF)and deatlL Clinical trials have shown that the regimen of combining β-adrenoreceptor blocker with traditional medicines against CHF can improve left ventricular function and prevent the ventricle from remodeling in patients with CHF.The present study aimad to observe the effect of carvedilol on concentration of plasma brain-type natriuretic peptide(BNP),and safety in children with EFE.Methotis Twenty-one children with EFE were randomly divided into two groups:(1)treated with traditional regimen(digoxin,prednisone and/or diuretics)(n=10);(2)treated with carvedilol plus traditional regimen(n=11).Measurement of plasma concentration of BNP by ELISA,cardiac function by ultrasound were performed before and after 6 months of treatment.The changes in clinical symptom,heart rate,heart funotion,side effect and maximal tolerance dose after treatment with earvedilol were observed.Results Plasma concentration of BNP was much higher in the group of patients with EFE [(865±702)ng/L]than that of control group[(154±78)ng/L](P＜0.01),and there was a positive correlation between plasma concentration of BNP and cardiac function classification.and cardiac function grades Ⅱ,Ⅲ,and Ⅳ correspondedto plasma concentration of BNP(286±125)ng/L,(437±386)ng/L,(1673±859)ng/L respectively in children with EFE.Compared with the group treated with traditional medicines,plasma concentration of BNP[(403±216)ng/L vs.(219±87)ng/L]significantly decreased,the clinical symptom was significantly improved,cardio-thoracic ratio(CTB)(0.60±0.05 vs.0.54±0.06)(P＜0.05)and heart rate[(115±20)bpm v8.(90±14)bpm](P＜0.01)decreased,Oection fraction(EF)(46.6%±13.4%vs.54.5%±12.9%),fractional shortening(2I.6%±8.1%vs.24.1%±7.5%),mean velocity of circumferential fiber shortening[(O.8±0.5)cir/s vs.(0.9±0.4)cir/s] were significantly increased (P ＜0. 01 ),left ventrieular end-systolic dimension [ (34. 0±8.6) mm vs. (32.2±9.1) mm] (P＜0.05.),left ventricular mass [(65.9±34.1)g vs. (65.9±34.1) g],interventricular septal thickness at end-systole [ ( 6. 0 ±1.0 ) mm vs ( 5.5±1.1 )mm ] were notably decreased ( P ＜ 0. 01 ) after treatment with carvedilol.Conclusion These data indicated that plasma concentration of BNP significantly increased in children with EFE, carvedilol can decrease plasma concentration of BNP,inhibit the remodeling of ventricle,significantly improve the cardiac function in children with EFE. Carvedilol is effective and safe in treatment of children with EFE.