Kleinert S, Weintraub R G, Wilkinson J L, Chow C W
Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
J Heart Lung Transplant. 1997 Dec;16(12):1248-54.
The true incidence and prognosis of myocarditis in children with acute dilated cardiomyopathy (DCM) at presentation remains uncertain. This study examines the incidence of lymphocytic myocarditis in a consecutive cohort of children with acute DCM at presentation and outcome after dual therapy immunosuppression with cyclosporine and steroids.
Twenty-nine consecutive children with acute DCM underwent early endomyocardial biopsy. Children with "definite" myocarditis comprised group I (n = 9) and were treated with cyclosporine and prednisolone. Group II (n = 2) had "borderline" myocarditis, and group III (n = 18) nonspecific histologic findings. Outcome was assessed by echocardiographic measurement of left ventricular end-diastolic dimension and fractional shortening, with follow-up endomyocardial biopsy in group I subjects.
Myocardial inflammation with or without myocardial necrosis (groups I and II) was present in 38% of all cases. There were no initial clinical, electrocardiographic, or echocardiographic features to distinguish patients in group I from patients in group III. At presentation, the mean +/- SEM left ventricular end-diastolic dimension and fractional score-Z scores of group I patients were 4.6 +/- 1.7 and -5.1 +/- 0.8, respectively, compared with 0.8 +/- 0.3 and -0.9 +/- 0.4, respectively, at withdrawal of immunosuppression (p < 0.001 for both). Both of these parameters did not differ significantly from normal controls at least follow up. Two group I patients had a biopsy-proven relapse after withdrawal of therapy that responded to reinstitution of immunosuppression. At latest follow-up, all nine group I patients had regained normal left ventricular function compared with four of 18 group III patients (p < 0.001).
Lymphocytic myocarditis is frequent in children with dilated cardiomyopathy and cannot be predicted from noninvasive investigations. The use of cyclosporine and steroids is associated with a favorable outcome, and a controlled trial of dual therapy immunosuppression in children is therefore warranted.
急性扩张型心肌病（DCM）患儿初诊时心肌炎的真实发病率及预后仍不确定。本研究调查了初诊时患有急性DCM的连续队列儿童中淋巴细胞性心肌炎的发病率以及环孢素和类固醇双重免疫抑制治疗后的结局。
29例连续的急性DCM患儿接受了早期心内膜心肌活检。患有“确诊”心肌炎的患儿组成I组（n = 9），接受环孢素和泼尼松龙治疗。II组（n = 2）有“临界”心肌炎，III组（n = 18）有非特异性组织学表现。通过超声心动图测量左心室舒张末期内径和缩短分数来评估结局，I组受试者进行随访心内膜心肌活检。
所有病例中38%存在伴有或不伴有心肌坏死的心肌炎症（I组和II组）。没有初始临床、心电图或超声心动图特征可区分I组和III组患者。初诊时，I组患者的平均±标准误左心室舒张末期内径和分数缩短Z评分分别为4.6±1.7和-5.1±0.8，而免疫抑制治疗撤药时分别为0.8±0.3和-0.9±0.4（两者p均<0.001）。至少随访时这两个参数与正常对照组无显著差异。两名I组患者在治疗撤药后经活检证实复发，再次给予免疫抑制治疗有效。在最近一次随访时，所有9名I组患者左心室功能恢复正常，而18名III组患者中有4名恢复正常（p<0.0
