of rheumatic fever, although often disabling, runs its course in a matter of weeks, and death during such an attack is rare nowadays in the developed world. When fatalities do occur, they are due to intractable rheumatic carditis. Detailed clinical descriptions of ARF are available in numerous reviews (Bisno, 1982, 1985; Stollerman, 1975; Whitnack and Bisno, 1980).
From a public health standpoint, the intense concern with ARF stems from its propensity to inflict permanent heart damage. Such damage usually takes the form of chronic, deforming rheumatic valvulitis leading to insufficiency and stenosis of the mitral or aortic valves or both and occasionally of the valves of the right heart as well. Moreover, patients who have suffered a single attack of ARF are highly susceptible to recurrent attacks following immunologically significant GrAS upper respiratory infections. As the rheumatic attacks tend to be mimetic, individuals who experienced carditis with the first attack are likely to experience progressive heart damage with succeeding episodes.
The long-term prognosis of ARF is closely correlated with the cardiac status during the acute attack. This was shown most conclusively in the joint United Kingdom/United States collaborative study (United Kingdom and United States Joint Report, 1965), wherein some 494 children under the age of 16 were studied; about 70 percent of these were followed up over many years. Among patients free of carditis during their acute attack, only 6 percent had residual heart disease. Patients with no preexisting heart disease who experienced mild carditis during their acute attack (i.e., apical systolic murmur without pericarditis or heart failure) had a relatively good prognosis in that only about 30 percent had heart murmurs 19 years later. About 40 percent of subjects with apical or basal diastolic murmurs and 70 percent of subjects with congestive heart failure or pericarditis or both during their acute attacks had residual rheumatic heart disease. The prognosis was worse in patients with preexisting heart disease and in those who experienced recurrent attacks of ARF.
Thus, as a general rule, patients who do not experience carditis during an initial attack of ARF and who are protected from recurrent rheumatic attacks will not go on to develop rheumatic heart disease. Individuals with “pure” chorea represent an exception to this rule, because 25 percent of them may go on to develop rheumatic heart disease.
Although the figures vary widely, in most modern studies about 50 percent of patients diagnosed as having ARF experience some carditis during the acute attack. One might estimate that some 33 to 50 percent of such patients will be left with residual rheumatic heart disease of varying severity. Thus, perhaps one-quarter of all ARF patients develop chronic cardiac involvement. In many of these patients, the involvement is characterized by severe valvulitis with chronic congestive heart failure. A sizeable number of patients eventually require valve replacement or die from the effects of rheumatic heart disease.