Radley-Smith R, Slavík Z, Spatenka J, Yacoub M H
Harefield Hospital, Middlesex a National Heart-Lung Institute, Londýn, Velká Británie.
Cesk Pediatr. 1992 May;47(5):257-9.
Authors reviewed available information concerning etiology and pathophysiology of dilated cardiomyopathy. Diagnostic criteria and current poor results of conservative treatment are discussed. Between August 1984 and August 1991 67 children with various types of cardiomyopathy underwent heart and heart and lung transplantation at Harefield Hospital, U.K. Dilated cardiomyopathy was the indication for transplantation in 47 patients (mean age at operation was 6.5 years, range 0.3-4.8 years). Eighty-one percent actuarial survival at 4 years after operation represents reasonable progress in the treatment of this severe acquired disease. Currently, early transplantation is recommended in children with dilated cardiomyopathy and with low shortening fraction of left ventricle (below 0.13), without any improvement after 3 months of conservative treatment, will familial trait of dilated cardiomyopathy and/or with severe myocardial fibrosis.
作者回顾了有关扩张型心肌病病因和病理生理学的现有信息。讨论了诊断标准和目前保守治疗效果不佳的情况。1984年8月至1991年8月期间,英国哈雷菲尔德医院有67例患有各种类型心肌病的儿童接受了心脏及心肺移植手术。扩张型心肌病是47例患者的移植指征(手术时的平均年龄为6.5岁,范围为0.3至4.8岁)。术后4年81%的精算生存率表明在这种严重的后天性疾病的治疗方面取得了合理进展。目前,对于患有扩张型心肌病、左心室缩短分数低(低于0.13)、经过3个月保守治疗无任何改善、有扩张型心肌病家族特征和/或有严重心肌纤维化的儿童,建议早期进行移植。