Acute rheumatic fever (ARF), an auto-immune response to a group A Streptococcus infection and precursor to rheumatic heart disease (RHD), remains endemic in many socio-economically disadvantaged settings. A Global Resolution on ARF and RHD was recently adopted at the 71st World Health Assembly where governments committed to improving efforts to prevent and control ARF and RHD. To inform these efforts, the objectives of this study were to examine associations between childhood ARF in the UK between 1958 and 1969 and a range of environmental and social factors. Of 17 416 children from the nationally representative birth cohort of the National Child Development Study, ARF was reported in 23 children during early childhood (between birth and the 7-year follow-up) and in 29 additional children during middle childhood (between the 7- and 11-year follow-ups). Risk factors associated with ARF in both early and middle childhood were: a large family size; attendance at a private nursery or class; a history of nephritis, kidney or urinary tract infections; and a history of throat or ear infections. Risk factors for ARF in early childhood alone were families with fathers in a professional or semi-professional occupation and families who moved out of their local neighbourhood. Risk factors in late childhood alone included overcrowding and free school meals. These data suggest that prevention strategies in ARF endemic settings may be enhanced by targeting, for example, new members entering a community and children in environments of close contact, such as a nursery or shared bedrooms.

Acute rheumatic fever (ARF) continues to produce a significant burden of disease in New Zealand (NZ) Māori and Pacific peoples. Serious limitations in national surveillance data mean that accurate case totals cannot be generated. Given the changing epidemiology of ARF in NZ and the major national rheumatic fever prevention programme (2012–2017), we updated our previous likely true case number estimates using capture–recapture analyses. Aims were to estimate the likely true incidence of ARF in NZ and comment on the changing ability to detect cases. Data were obtained from national hospitalisation and notification databases. The Chapman Estimate partially adjusted for bias. An estimated 2342 likely true new cases arose from 1997 to 2015, giving a mean annual incidence of 2·9 per 100 000 (95% CI 2·5–3·5). Compared with the pre-intervention (2009–2011) baseline incidence (3·4 per 100 000, 95% CI 2·9–4·0), the likely true 2015 incidence declined 44%. Large gaps in data completeness are slowly closing. During the period 2012–2015, 723 cases were identified; 83·8% of notifications were matched to hospitalisation data, and 87·2% of hospitalisations matched to notifications. Despite this improvement, clinicians need to remain aware of the need to notify atypical patients. A possible unintended consequence of the national ARF prevention programme is increased misdiagnosis.

In New Zealand, efforts to control acute rheumatic fever (ARF) and its sequelae have focused on school-age children in the poorest socioeconomic areas; however, it is unclear whether this approach is optimal given the strong association with demographic risk factors other than deprivation, especially ethnicity. The aim of this study was to estimate the stratum-specific risk of ARF by key sociodemographic characteristics. We used hospitalization and disease notification data to identify new cases of ARF between 2010 and 2013, and used population count data from the 2013 New Zealand Census as our denominator. Poisson logistic regression methods were used to estimate stratum-specific risk of ARF development. The likelihood of ARF development varied considerably by age, ethnicity and deprivation strata: while risk was greatest in Māori and Pacific children aged 10–14 years residing in the most extreme deprivation, both of these ethnic groups experienced elevated risk across a wide age range and across deprivation levels. Interventions that target populations based on deprivation will include the highest-risk strata, but they will also (a) include groups with very low risk of ARF, such as non-Māori/non-Pacific children; and (b) exclude groups with moderate risk of ARF, such as Māori and Pacific individuals living outside high deprivation areas.

Between January, 2009 and December, 2013, 84 patients were identified who underwent isolated mitral valve surgery in Jamaica at The University Hospital of the West Indies and The Bustamante Hospital for Children. The most common pathology requiring surgery was rheumatic heart disease, accounting for 84% of the procedures performed. The majority of patients had regurgitation of the mitral valve (67%), stenosis of the mitral valve (22%), and mixed mitral valve disease (11%). The most common procedure performed was replacement of the mitral valve (69%), followed by mitral valve repair (29%). Among the patients, one underwent closed mitral commissurotomy. The choice of procedure differed between age groups. In the paediatric population (<18 years of age), the majority of patients underwent repair of the mitral valve (89%). In the adult population (18 years and above), the majority of patients underwent mitral valve replacement (93%). Overall, of all the patients undergoing replacement of the mitral valve, 89% received a mechanical valve prosthesis, whereas 11% received a bioprosthetic valve prosthesis. Of the group of patients who underwent mitral valve repair for rheumatic heart disease, 19% required re-operation. The average time between initial surgery and re-operation was 1.2 years. Rheumatic fever and rheumatic heart disease remain significant public health challenges in Jamaica and other developing countries. Focus must remain on primary and secondary prevention strategies in order to limit the burden of rheumatic valvulopathies. Attention should also be directed towards improving access to surgical treatment for young adults.

Rheumatic fever (RF) is an important public health problem in New Zealand (NZ). There are three sources of RF surveillance data, all with major limitations that prevent NZ generating accurate epidemiological information. We aimed to estimate the likely RF incidence using multiple surveillance data sources. National RF hospitalization and notification data were obtained, covering the periods 1988–2011 and 1997–2011, respectively. Data were also obtained from four regional registers: Wellington, Waikato, Hawke's Bay and Rotorua. Coded patient identifiers were used to calculate the proportion of individuals who could be matched between datasets. Capture–recapture analyses were used to calculate the likely number of true RF cases for the period 1997–2011. A range of scenarios were used to correct for likely dataset incompleteness. The estimated sensitivity of each data source was calculated. Patients who were male, Māori or Pacific, aged 5–15 years and met the Jones criteria, were most likely to be matched between national datasets. All registers appeared incomplete. An average of 113 new initial cases occurred annually. Sensitivity was estimated at 80% for the hospitalization dataset and 60% for the notification dataset. There is a clear need to develop a high-quality RF surveillance system, such as a national register. Such a system could link important data sources to provide effective, comprehensive national surveillance to support both strategy-focused and control-focused activities, helping reduce the incidence and impact of this disease. It is important to remind clinicians that RF cases do occur outside the well-characterized high-risk group.

Rheumatic fever is a public health problem of universal distribution, predominantly affecting individuals in developing countries. In individuals less than 20 years of age, pure mitral regurgitation is the most commonly found condition in chronic rheumatic valve disease. In the present study, rheumatic mitral regurgitation was assessed in children and adolescents, addressing its clinical (duration of the disease, symptoms, use of benzathine penicillin, and number of outbreaks of the acute phase of rheumatic fever), electrocardiographic (left atrium abnormality and/or left ventricle hypertrophy) and echocardiographic characteristics (left atrium and ventricle measurements, ejection fraction and pulmonary artery pressure), as well as plasma dose of N-terminal portion of the brain natriuretic peptide through electrochemiluminescence immunoassay. Fifty-three patients were studied. The patients had moderate (41.5%) or severe (58.5%) rheumatic mitral regurgitation; had not undergone surgery; were not in the acute phase of the disease; and were being treated at a paediatric cardiology reference hospital in Northeastern Brazil. Mean patient age was 10.6 years (minimum of 3 and maximum of 19 years). With the exception of the ejection fraction, the echocardiographic variables had a significant correlation to the natriuretic peptide, demonstrating that this hormone reflects the haemodynamic consequences of mitral regurgitation. It was concluded that cardiac remodelling that occurs in rheumatic mitral regurgitation in children and adolescents leads to the production of the brain natriuretic peptide, which could be used as a complementary diagnostic tool in the follow-up of such patients.

We have conducted, in two stages, a descriptive and comparative analysis of the diagnostic and evolutionary clinical aspects of rheumatic fever. The descriptive analysis was based on a sample of 226 patients seen between 1976–1986. The forms and frequency of the manifestations of rheumatic fever were studied, as well as the difficulties encountered in diagnosing mild cases and the risk of making a hasty diagnosis due to the high prevalence of the disease. Subsequent to the establishment of an outpatient clinic for referral of patients with rheumatic fever, as part of a study and control program for the disease, 98 patients were seen in the period 1988–1991. In order to compare the profile of the manifestations in the acute phase with evolutionary aspects over the course of the disease, a subgroup of 61 children, treated in the period 1983–986 was selected to obtain equivalence in size of sample and time of follow-up (p = 0.08). The comparative analysis revealed that no modifications have occurred over the period of study in the profile of the manifestations during the acute phase, and no statistically significant differences have occurred in age at first attack, severity ofcarditis, or in the type ofvalvar and articular involvement. Evolutionary data after rigid control of primary and secondary prevention, however, showed a marked reduction in the incidence of recurrence (p = 0.036), the frequency of hospital admission (p = 0.001) and deaths (p = 0.024). The main factors modifying the evolution of the disease were analyzed. The action of the health team had a fundamental importance in the control of recurrence. This fact is especially important for developing countries, where a reduction of the prevalence of the disease can only be achieved through major structural socioeconomic changes in the community.

We describe an eleven-year-old boy with severe retardation in physical growth and severe rheumatic valvar disease. At surgery, a sample of the atrial appendage showed Aschoff nodules, although no antecedent of rheumatic fever was recorded. The patient was HLA-DR5 positive. The case emphasizes the strong association of malnutrition with severe rheumatic heart disease in northeast Brazil.

We studied 56 children echocardiographically, ages four to 15 years (mean nine years) with acute rheumatic fever to determine the extent of cardiac involvement. All had a clinical diagnosis of carditis. Mitral regurgitation was always present and was associated with aortic regurgitation in 53.6% and tricuspid regurgitation in 32.1%. Mitral valvar prolapse was observed in 18 patients 5(32.1%) and could not be differentiated from myxomatous prolapse in 10 (17.8%). Cordal rupture was detected in seven patients (12.5%), three of whom required surgical treatment. Other findings concerning the mitral valve were vegetations in two patients (3.6%), commissural fusion without stenosis in 36(64.3%), and mitral stenosis in four (7.1%). Heart failure was usually secondary to valvitis rather than myocarditis, and led to surgical treatment in seven patients. The myocardial function was depressed in only two patients. In both, the ejection function returned to normal after medical treatment. Echocardiography provides important information on the involvement of the heart in acute rheumatic fever, helping to determine prognosis and the results of treatment.

A total of 246 consecutive patients were seen with the diagnosis of acute rheumatic fever (and/or rheumatic heart disease) and were followed for 587.7 patient years. The episode of acute rheumatic fever was the first in 64 of these patients, whereas recurrent acute rheumatic fever was seen in 26 and the other 156 patients had chronic rheumatic carditis. At presentation, those suffering an initial attack had less frequent and less severe carditis when compared to those suffering recurrent infection (p<0.05). Improvement in carditis during follow-up was noted in those having an initial attack (p<0.1), while deterioration occurred following recurrent infection (p<0.01), and no change was noted for those with chronic infection. Recurrences of acute rheumatic fever were most frequent in those presenting with their initial infection (21%) or reinfection (35%), and dropout from follow-up was highest in the group with first infection (38%) compared to those with recurrent infection (15%) and chronic carditis (25%). Non-recognition of the first episode of acute rheumatic fever and failure of secondary prophylaxis were found to be the major contributors to the observed increased pool of recurrent and chronic rheumatic heart disease. We conclude that, in the absence of programmed primary prophylaxis of acute rheumatic fever, the best chance of controlling the progression of carditis or affecting cure is to recognize the first episode ofacute rheumatic fever and then ensure strict adherence to secondary prophylaxis. Since the prognosis of recurrent carditis is poor, the best management of moderate to severe recurrent carditis is early reparative valvar surgery wherever possible.

In recent years, rheumatic fever has been declining in the industrialized countries, and has became a rare disease. In developing areas, nonetheless, the sequels of its cardiac involvement have important implications from the stance of public health, and this disease is still the main cause of heart disease in children and young adults. From a historical perspective, the long-term prevention and the control of socioeconomic problems have been proven to be effective, and it is these measures which have contributed to the decline of the disease in developed countries. But, due to its present high prevalence around the world, and the potential for his resurgence, this disease remains an unsolved problem. Furthermore, the complete understanding of its pathophysiology remains a challenge. In this presentation, we will discuss our experience with epidemiological, clinical and prophylatic aspects of this enigmatic disease, and the strategies we have developed to study and control heumatic fever in Minas Gerais, Brazil.