A long-term follow-up study on the clinical treatment of 75 cases with primary endocardial fibroelastosis / 中华儿科杂志

ABSTRACT

<p><b>OBJECTIVE</b>To summarize and analyze the effects of treatment and prognosis of infants with endocardial fibroelastosis (EFE) in different states of the illness undergone relevant therapies, and to understand the roles of different treatments for improving the prognosis of the disease.</p><p><b>METHODS</b>Data of 75 cases with EFE admitted into Anzhen Hospital Affiliated to Capital Medical University from August 1984 to June 2006 were analyzed retrospectively.</p><p><b>RESULTS</b>(1) Of the 75 cases with EFE (40 males and 35 females), with the onset age ranged from 20-days to two years and eight months, 69 cases were treated normally and followed up in the Outpatient Department of the Hospital after discharge, the follow-up rate was 92%, with the follow-up span from six months to 23 years (5.7 years in average). During the follow-up, six cases (8.7%) died. (2) The total curative rate of EFE patients was 46.4% (32/69), while the improvement rate was 40.6% (28/69), the total rate of the cure and improvement was 87%. (3) The average value of ejection fraction (EF) of left ventricle of all the patients returned to normal two years after treatment (EF value was 55.86 ± 2.85), the percentage of patients with normal left ventricle EF at 1 year, 3 years, 5 years and 10 years after treatment was 42.6% (26/61), 64.4% (29/45), 70.7% (29/41) and 84.6% (22/26), respectively. The average value of cardiothoracic (C/T) ratio became normal three years after treatment through X ray examination (0.50 ± 0.01), however the average value of the LVDD had not been returned to normal 3 years after treatment. At 1 year, 3 years, 5 years and 10 years after treatment, the proportion of patients with normal LVDD was 0% (0/61), 13.3% (6/45), 53.7% (22/41) and 84.6% (22/26), respectively. (4) The average value of EF became normal one year after treatment in the glucocorticoid group (EF value 58.44 ± 5.10) in 37 cases scored < 22 at the first visit, while the average value of C/T normalized two years after treatment (0.50 ± 0.00); The average value of EF became normal three years after treatment in the glucocorticoid plus cyclophosphamide group (EF 57.33 ± 3.43) in 29 cases scored < 22 at the first visit, however the average value of the C/T and the LVDD did not return to normal 3 years after treatment. (5) Use of IVIG reduced the percentage of patients who received cyclophos-phamide. (6) The recovery of intimal thickness was slow in EFE patients, the span was four years on the average (1 - 8 years), the percentage of patients whose endocardium became normal 1 year, 3 years, 5 years and 10 years after treatment was 9.85% (6/61), 22.2% (10/45), 51.2% (21/41), 100% (29/29).</p><p><b>CONCLUSION</b>The long-term continuous normal treatment of patients with EFE showed good therapeutic effects. For severe and refractory cases, immunotherapy must be strengthened and maintained for longer time. For those who clinically recovered, the quantity of activity should be restricted after the treatment is discontinued, and the re-examination should be done timely for further management.</p>