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Favorable effects of immunosuppressive therapy in children with dilated cardiomyopathy and active myocarditis
P R Camargo1, R Snitcowsky, P L da Luz
1Instituto do Coraçao, Divisao Clínica, Sao Paulo, Brazil.
Insights
Immunosuppressive therapy with azathioprine or cyclosporine combined with prednisone significantly improves outcomes for children with active myocarditis and severe ventricular dysfunction, showing better clinical and histological results than conventional treatment.
Area of Science:
- Pediatric Cardiology
- Immunosuppressive Therapy
- Myocarditis Research
Background:
- Severe dilated cardiomyopathy in children can be associated with active myocarditis.
- Conventional treatments show limited efficacy in improving clinical and hemodynamic parameters.
Purpose of the Study:
- To evaluate the efficacy of immunosuppressive therapy in children with severe dilated cardiomyopathy and active myocarditis.
- To compare outcomes between conventional treatment and immunosuppressive regimens.
Main Methods:
- A study involving 43 children diagnosed with active myocarditis via endomyocardial biopsy.
- Four treatment groups: conventional therapy, conventional plus prednisone, conventional plus prednisone and azathioprine, conventional plus prednisone and cyclosporine.
- Assessment through noninvasive and invasive hemodynamic studies.
Main Results:
- Conventional therapy yielded minimal improvement (2/9 patients).
- Prednisone alone showed modest improvement (3/12 patients).
- Azathioprine (13/16) and cyclosporine (10/13) combined with prednisone demonstrated significantly better clinical, hemodynamic, and histological outcomes.
Conclusions:
- Immunosuppressive therapy, particularly with azathioprine or cyclosporine alongside prednisone, markedly improves prognosis in pediatric active myocarditis.
- These combined immunosuppressive strategies offer a superior therapeutic approach for severe ventricular dysfunction in children.
Abstract:
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.