| OBJECTIVE: To research the data of in-patient department and follow-up of patients who had been diagnosed with endocardial fibroelastosis(EFE) retrospectively. And we analyzed and discussed the incidence, treatment and prognosis of patients with EFE. METHODS: We collected data of 31 cases who had been diagnosed with endocardial fibroelastosis(EFE) in Lanzhou University Second Hospital(Children’s Hospital of Gansu Province) from June 2007 to December 2015, and processed it by statistical analysis. RESULTS: 1. There were thirty-one patients being diagnosed with EFE in the first visit in the in-patient department, 19 boys(61.30%), 12 girls(38.70%),the average age was 4.71±3.19 mon(2~16 mon), and 26 of 31 cases were less than six months old. One girl had family history about EFE. 2. The chest X-ray examinations of all 31 cases revealed the enlargement of the heart, in which almost was expansion of left atrium and ventricle. The cardiothoracic ratio was between 0.58~0.70( 0.64±0.03). 3. All patients had electrocardiography. In the ECGs(electrocardiogram), the left atrium and left ventricle with high voltage in different degrees, T waves inversion and ST-T change were most common in every case, no arrhythmia. 4. Echocardiograms of 31 cases all showed left ventricular endocardial thickening, involving the mitral valve and aortic valve in some patients. The thickness of endocardium was between 3.10~5.50 mm, and the average value was 3.89±0.47 mm。5. There were 26 cases in the first in-patient in the conventional treatment group, six died, 6 lost. Fourteen cases had finished at least thirty-six months follow-up, and three cases had finished more than sixty months follow-up. The duration of follow-up in all the other 14 patients was between 38~70 months. The cure rate of one year was 1/15(6.67%), and one year ’s improvement rate was 3/15(20.00%). The cure rate of three, five years was 3/14(21.43%) and 1/3(33.33%), respectively. The improvement rate of 3, 5 years was 9/14(64.28%) and 2/3(66.67%), respectively. With the treatment time increasing, the endocardial thickness gradually decreased, LVEF and LVFS gradually increased, and cardiothoracic ratio decreased. 6. There were 5 cases diagnosed with EFE in the first visit in the IVIG group. All the five cases had finished IVIG infusion on time, and there was no case died and lost. The duration of follow-up was between 12~36 months. All five patients reached the criterion of improvement. The thickness of endocardium, LVEF, LVFS, and cardiothoracic ratio, which compared with the onset of EFE, had improved significantly. 7. LVEF in IVIG group was higher than that in the conventional treatment group after twelve months ’ treatment, 51.80±1.30 vs. 45.53±5.34( P < 0.001).8. There was a negative correlation between the thickness of endocardium and LVFS, r=-0.435,P=0.014. There was a positive correlation between the thickness of endocardium and cardiothoracic ratio, r= 0.375,P=0.038. CONCLUSIONS: 1. Primary endocardial fibroelastosis was most common in infants and young children, and mainly occurred in less than 6 months of age. The symptoms of heart failure were the main reason for visit, and most patients were susceptible to pulmonary infection. Very few cases had family history of EFE. 2.The duration of treatment for EFE needs a long time. Adhering to the long-term regular treatment is necessary. 3.Intravenous immunoglobulin has a certain therapeutic effect on the improvement of cardiac systolic function in patients with EFE. 4.There was a negative correlation between the thickness of endocardium and cardiac systolic function in the patients of EFE, and a positive correlation between the thickness of endocardium and the size of heart. |
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