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Objective: To analyse clinical presentation of rheumatic fever (RF), with special emphasis on cardiac involvement, electrocardiographic and echocardiographic findings and the outcome of the cases referred to Maria Pia Children's Hospital from January 1990 to September 1999., Methods: We retrospectively analyzed the clinical files of all cases referred to pediatric cardiology clinics with the suspicion of acute RF (Group 1) or with rheumatic valvular disease and heart failure (Group 2). In group 1 we studied the following: age and sex distribution, year of diagnosis, presence of Jones criteria treatment and outcome. In group 2 we analysed provenance, age of initial onset of RF, age of cardiology referral, treatment and outcome., Results: Thirteen cases were identified, 8 in groups 1 and 5 in group 2. Group 1 included 3 girls and 2 boys, mean age of 10 years. The diagnosis of RF was based in the presence of 2 major and 1 minor manifestation (4/8), 1 major and 2 minor manifestations (1/8) and chorea in 3 cases associated with clinical carditis in one and subclinical carditis in another. Colour Doppler echocardiography showed pathological mitral regurgitation jet in 6 cases, associated with aortic regurgitation in 2 and dilatation of left ventricle in 3. All were treated with penicillin associated with anti-inflammatory drugs in 5 and haloperidol in 3. Group 2 included 3 girls and 2 boys, mean age 9.56 years. Four were from African countries (Angola and Guinea), and one came from the north of Portugal. The elapsed time between the initial acute attack and cardiology referral varied from 5 months to 3 years. All presented severe mitral insufficiency associated with aortic and/or tricuspid valve lesions, and heart failure. All five underwent valve surgery. The secondary prophylaxis was recommended in every patient. There was a recurrence in a child who had interrupted chemoprophylaxis. The patients from African countries were lost for follow-up., Conclusions: RF still remains a problem in present times, with serious cardiac sequela in African countries. Colour Doppler echocardiography is a valuable tool for the detection of pathological valvular regurgitation and subclinical carditis if strict criteria are used. The need for appropriate treatment of streptococcal pharyngitis and secondary prophylaxis is emphasized.