| Objective:To analyze the clinical materials of children with endocardial fibroelastosis in the recent 5 years and summarize the characters of EFE, effects of different treatments and the factors of prognosis.Methods:A riview of 71 cases who admitted in the Shandong provincial hospital between Jan.2005.and Jun.2010 with primary EFE and of the following-up datas were carried out.The echocardiography and electrocardiogram were routinely performed during each outpatient visit.Results:1.The onset age was 1 months to 32 months (mean 7.3±5.5months) The reasons for being admitted into hospital were pneumonia or hear failure.2.The mainly abnormality of electrocardiogram were high voltage of left ventricle, and inversion of T wave.3.The cardiothoracic rate (CTR) was higher than normal.4..All the patients are the kind of dilated heart mainly in left ventricle and the thickened endocardium were find only in left ventricle especially in lateral wall and inferoposterior wall. The left ventricle ejection fraction(LVEF) was (37.6±12.1) and the thickness of endocardium was (0.37±0.07) cm. And significant negative correlation and partial negative correlation (removing sex and onset age) are both presented between the LVEF and the thickness of endocardium (r=0.0592,p<0.01) 5.51 patients have been followed up between 5 months and 70 months in the outpatient and the age in the end of follow-up was 10 months～8 years and 6 months. The total curative rate is 17.6%(9/51) and the average time to recover is (35.2±16.8) months.6.During the follow-up 8 patients (8/51) aged 1-7 months died, and the age of dead is significant younger than the survival's (3.5±2.1m VS 8.8±6.4m,p<0.05),The left ventricular ejection fraction(LVEF) of dead and survival are (36.40±5.1)% and (38.0%±13.1)% (p=0.79) respectively and the thickness of endocardium of dead and survival are 0.34±0.071cm,0.37±0.076cm (p=0.36),respectively. The age of dead was (4.8±2.82) months, the span was 1.6 months to 7.7 months.7.24 cases were with normal Left ventricle ejection fraction (LVEF) value at the end of follow-up and the time span that LVEF becomes to normal is 0-48 months,(16.15%±13.43% months in average)and the endocardium was not normal at the same time. The cases whose endocardial thickness became normal at the end of follow-up were only 3 and the time to return to normal are 6 months,1 year and 3 years respectively.8.The LVEF and the thickness of endocardium of 21 cases treated withβblockers were improved significantly in all the point-in-times during the follow-up.9.8 cases were treated with IVIG, no death was happened. The LVEF was improved in the first therapeutic year [(38.0±10.30)% vs (55.3±6.68)%, p<0.01]and the thickness of the endocardium in the end of the first therapeutic year was thinner the beginning.Conclusions:1. The abnormal endocardium was only find in the left ventricle and is more significant in the lateral wall and inferoposterior wall of the left ventricle the LVEF gets better as the thickness gets thinner.2. High voltage of left ventricle and inversion of T wave are the main abnormality.3.The symptoms were improved firstly, then the LVEF, and endocardium was the last to be normal. 4. The younger of onset age the more risk of suffering dead. The dead age is younger than 8months.5.βreceptor blocker could improve the LVEF and relive the thickness of endocardium, and it is safe to patients.6.The use of IVIG can improve the LVEF significantly during short time, no death was happened.7.The effect of long-time regular treatment is good with no side-effect. |
PDF Full Text Request