多巴酚丁胺负荷超声心动图在评价小儿扩张型心肌病预后中的价值

目的 探讨超声心动图参数与多巴酚丁胺负荷试验在评价小儿扩张型心肌病（DCM）预后中的作用及临床价值。方法 DCM患儿31例，分为两组：I组（16例）为病情好转组；Ⅱ组（15例）为病情恶化、死亡组。正常组30例。行超声心动图、多巴酚丁胺超声心动图负荷试验检查。结果 全部病例随访3个月－4年（Ⅰ组患儿6例失访，10例目前仍存活，病例稳定；Ⅱ组8例患儿病情无明显好转，3例院内死亡，4例1年内死亡）。正常组分别与DCMI、II组比较，超声心动图参数：射血分数（EF）、短轴缩短率（Fs）、肺动脉压力（PAMP）、肺毛细血管楔压（PCWP）、左心室壁应力（LVWS）、舒张期左心室后壁与左心室内径比值（PWd/Dd）、心室收缩末期容量指数（ESVI）和收缩压与心室收缩末期容量指数（SP／ESVI）、ESVI均有显著性意义（P＜0．01、P＜0．001），DCM两组比较，除PCWP和LVWSII外差异均有显著意义（P＜0．05、P＜0．001）。多巴酚丁胺超声心动图负荷试验正常组反应增强，而DCM组反应性均差，但两组比较，Ⅱb组（7例）反应较Ⅱa组（8例）反应更低。且Ⅱb组生存时间与负荷后左室功能参数EF、Fs的反应性呈正相关(r分别为0．88，0．75，P＜0．01、P＜0．05）。结论 DCM小儿有以下表现者预后差：①EF、Fs和PWd/Dd显著减低，可能提示心肌细胞的减少和纤维的增生；②多巴酚丁胺负荷试验无明显反应；③肺动脉压力和左心室壁应力明显增高。

Prognostic and clinical value of echocardiography and dobutamine stress test in children with dilated cardiomyopathy

Objective　To explore the prognostic and clinical value of echocardiography and dobutamine stress test in children with dilated cardiomyopathy (DCM). Methods　Thirty-one children with DCM were divided into two groups: group I(16 cases), to be good prognosis; group II(15 cases), to be poor prognosis. Tirty cases of normal children were designed to be control group. Echocardiography and dobutamine stress test were examined in the patients (15 cases, group Ⅱa:8 cases; group Ⅱb:7 cases) to measure the following parameters: ejection fraction (EF), fractional shortening (Fs), the ratio of diastolic left ventricular wall thickness and diastolic left ventricular diameter (PWd/Dd), pulmonary artery mean pressure (PAMP), left ventricular wall stress (LVWS ), pulmonary capillary wedge pressure (PCWP), ratio of contractive press and end contract volume index (SP/ESVI), ESVI,et al. Meanwhile, the prognosis of the DCM patients were surveyed.Results　The echocardiography parameters of EF, Fs, PAMP, PCWP, LVWS, PDd/Dd, ESVI, SP/ESVI and ESVI were significantly different between DCM group I, II and control group (P<0.01,P<0.001). Moreover, there were significant difference of the above parameters except PCWP and LVWS II between DCM group I and group II (P<0.05,P<0.001). Control group showed strong response to dobutamine stress test, however, DCM group had poor response, especially in group Ⅱb (7 cases). The survival time of group Ⅱb was positively correlated with parameters of EF, Fs response of left ventricle after load.Conclusions　The DCM children who had the following presentation would be poor prognosis: ①The significant decrease of EF, Fs and PWd/Dd , especial PWd/Dd, might indicate the reduce of myocardial cell and the hyperplasia of fibrosis. ②The patient showed poor response to dobutamine stress test. ③The pulmonary pressure and LVWS were significantly increased.