The presence of heart-M protein cross-reactive T-cell clones in rheumatic heart lesions suggests their direct involvement in the pathogenesis of this disease. The dissection of protective and pathogenic epitopes of streptococcal M protein is an important step in allowing the development of a safe anti-streptococcal synthetic vaccine.

BACKGROUND The incidence of rheumatic heart disease is great in Brazil. We analyzed the distribution of human leukocyte (HLA) antigens in a Brazilian population sample with rheumatic fever or rheumatic heart disease, with the aim of better understanding the mechanisms involved. METHODS AND RESULTS HLA class I (A, B, and C) and class II (DR and DQ) antigen distribution was studied in 40 patients with diagnosis of rheumatic fever or rheumatic heart disease and compared with a control group of 617 healthy individuals for class I typing, from which 118 were drawn for class II typing. A strong correlation between rheumatic fever and rheumatic heart disease and HLA-DRw53 (72.9% in the disease group versus 39% in the control group: p = 0.00061, relative risk, 4.2; etiologic fraction, 0.43) was found. We also found an increase in the frequency of HLA-DR7 (57.5% in the disease group versus 26.3% in control group: p = 0.00715; relative risk, 3.8; etiologic fraction, 0.56). HLA class I and HLA-DQ typing did not point to any association with these diseases. CONCLUSIONS HLA-DR7 and HLA-DRw53 are markers for susceptibility to rheumatic fever and rheumatic heart disease in Brazil. These results could be explained by genetic differences resulting from racial or geographical diversity.

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.

Interruption of pregnancy is usually recommended for pregnant women with Eisenmenger's syndrome. We studied 13 pregnancies in 12 women with this syndrome, who decided to carry on with their pregnancy despite recommendation for therapeutic abortion. The mean age was 27 years. Five patients had ventricular septal defect; two, persistent ductus arteriosus; one, a combination of both; two, atrial septal defect; one, atrioventricular septal defect and one patient a combination of ventricular and atrial septal defects. Mean systolic and diastolic arterial pulmonary pressures were 112.7 and 61.7, mmHg, respectively. There were three spontaneous abortions, one premature labour at 23 weeks of gestation and two maternal deaths during the 23 and 27 weeks of gestation. Seven patients who reached the end of the second trimester were hospitalized until delivery and received heparin (20,000 to 40,000 units per day) and oxygen therapy. Caesarean section was performed in all patients as a result of worsening maternal or fetal clinical condition during the third trimester of gestation. all the mothers were discharged from hospital but one of them died on the 30th day post-partum. Five of the eight infants were premature, three were small babies for gestational age and all were discharged from hospital with the exception of one who died 48 h after birth. In conclusion, although pregnancy should be discouraged in women with Eisenmenger's syndrome, it can be successful. In this study, prolonged bed rest, the use of heparin and oxygen therapy presumably positively influenced maternal and infant outcomes.

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