The natural history of Sydenham's chorea. Review of the literature and long- term evaluation with emphasis on cardiac sequelae

Fifty patients were examined an average of twenty-nine years after their initial episode of chorea. Seventeen (34 per cent) were found to have valvular heart disease. In 27 per cent of the patients who presented with "pure chorea" rheumatic valvular disease developed within thirty years. In 22 per cent of the patients with "pure chorea" valvular disease developed without recognized subsequent rheumatic manifestations. There appears to be no way to predict at the time of admission in which patient with "pure chorea" heart disease will develop. The absence of a significant murmur in no way implies freedom from future valvular damage; an organic murmur at the onset of chorea increases the probability of future valvular disease. No prognostic value could be obtained from initial electrocardiogram, chest roentgenogram or erythrocyte sedimentation rate. The number of recurrences had little influence on ultimate prognosis; the incidence of later heart disease appeared to increase slightly with the severity of the choreic episode. The presence of a soft, blowing fixed apical systolic murmur, the "P murmur," at the time of chorea carried no greater significance than presentation without a murmur. The ultimate appearance of heart disease after chorea was best correlated with the subsequent occurrence of other rheumatic manifestations. No neurological sequelae of chorea were found. A high incidence of emotional disturbances was found prior to the onset of chorea and a high incidence of psychoneurotic problems was apparent at the time of follow-up examination. Since no method is available to predict in which third of the patients with Sydenham's chorea heart disease will develop, it is suggested that all patients be given continuous antibiotic prophylaxis such as would be administered if they had acute rheumatic fever.