Camargo P R, Snitcowsky R, da Luz P L, Mazzieri R, Higuchi M L, Rati M, Stolf N, Ebaid M, Pileggi F
Instituto do Coraçao, Divisao Clínica, Sao Paulo, Brazil.
Pediatr Cardiol. 1995 Mar-Apr;16(2):61-8. doi: 10.1007/BF00796819.
Among 68 children with severe dilated cardiomyopathy, 43 (aged 10 months to 15 years) presented with active myocarditis, diagnosed by endomyocardial biopsy. They were divided into four treatment groups: I, controls: 9 patients submitted to conventional treatment (digitalis, diuretics, and vasodilators) for 8.1 +/- 0.7 (SD) months; II, prednisone: 12 patients received conventional therapy plus prednisone; III, azathioprine: 16 patients submitted to conventional therapy plus prednisone and azathioprine; IV, cyclosporine: 13 patients treated with conventional therapy plus prednisone and cyclosporine. Immunosuppressive therapy was maintained for a mean of 8.4 +/- 1.2 months. They were submitted to noninvasive (electrocardiogram, chest radiograph, Doppler echocardiogram, and radioisotopic scintigraphy) and invasive (hemodynamic) studies. In the control group only 2 of 9 patients showed clinical and hemodynamic improvement and 1 of 4, histologic regression of the myocarditis. Among patients submitted to conventional therapy plus prednisone, 3 of 12 presented clinical and hemodynamic improvement; 2 of 5 also showed histologic regression of inflammatory process. By contrast, patients treated with azathioprine or cyclosporine associated with prednisone had significantly better results: 13 of 16 and 10 of 13 patients, respectively, had clinical and hemodynamic improvement; all 6 patients in the azathioprine group and all 4 patients in the cyclosporine group had histologic regression of the myocarditis. Two patients in the prednisone group, one in the azathioprine group, and one in the cyclosporine group died during treatment, in cardiogenic shock. In our experience immunosuppressive therapy with azathioprine or cyclosporine associated with prednisone improves the prognosis of children with active myocarditis and severe ventricular dysfunction.
在68例患有严重扩张型心肌病的儿童中，43例（年龄在10个月至15岁之间）表现为活动性心肌炎，通过心内膜心肌活检确诊。他们被分为四个治疗组：I组，对照组：9例患者接受常规治疗（洋地黄、利尿剂和血管扩张剂）8.1±0.7（标准差）个月；II组，泼尼松组：12例患者接受常规治疗加泼尼松；III组，硫唑嘌呤组：16例患者接受常规治疗加泼尼松和硫唑嘌呤；IV组，环孢素组：13例患者接受常规治疗加泼尼松和环孢素。免疫抑制治疗平均持续8.4±1.2个月。他们接受了非侵入性（心电图、胸部X线、多普勒超声心动图和放射性核素闪烁扫描）和侵入性（血流动力学）检查。在对照组中，9例患者中只有2例显示临床和血流动力学改善，4例中有1例心肌炎组织学消退。在接受常规治疗加泼尼松的患者中，12例中有3例出现临床和血流动力学改善；5例中有2例也显示炎症过程的组织学消退。相比之下，接受硫唑嘌呤或环孢素联合泼尼松治疗的患者结果明显更好：16例患者中有13例、13例患者中有10例分别出现临床和血流动力学改善；硫唑嘌呤组的所有6例患者和环孢素组的所有4例患者心肌炎组织学消退。泼尼松组有2例患者、硫唑嘌呤组有1例患者和环孢素组有1例患者在治疗期间死于心源性休克。根据我们的经验，硫唑嘌呤或环孢素联合泼尼松的免疫抑制治疗可改善患有活动性心肌炎和严重心室功能障碍儿童的预后。
