Mortality and Morbidity in Adults With Rheumatic Heart Disease.

Importance: Rheumatic heart disease (RHD) remains a public health issue in low- and middle-income countries (LMICs). However, there are few large studies enrolling individuals from multiple endemic countries. Objective: To assess the risk and predictors of major patient-important clinical outcomes in patients with clinical RHD. Design, Setting, and Participants: Multicenter, hospital-based, prospective observational study including 138 sites in 24 RHD-endemic LMICs. Main Outcomes and Measures: The primary outcome was all-cause mortality. Secondary outcomes were cause-specific mortality, heart failure (HF) hospitalization, stroke, recurrent rheumatic fever, and infective endocarditis. This study analyzed event rates by World Bank country income groups and determined the predictors of mortality using multivariable Cox models. Results: Between August 2016 and May 2022, a total of 13 696 patients were enrolled. The mean age was 43.2 years and 72% were women. Data on vital status were available for 12 967 participants (94.7%) at the end of follow-up. Over a median duration of 3.2 years (41 478 patient-years), 1943 patients died (15% overall; 4.7% per patient-year). Most deaths were due to vascular causes (1312 [67.5%]), mainly HF or sudden cardiac death. The number of patients undergoing valve surgery (604 [4.4%]) and HF hospitalization (2% per year) was low. Strokes were infrequent (0.6% per year) and recurrent rheumatic fever was rare. Markers of severe valve disease, such as congestive HF (HR, 1.58 [95% CI, 1.50-1.87]; P < .001), pulmonary hypertension (HR, 1.52 [95% CI, 1.37-1.69]; P < .001), and atrial fibrillation (HR, 1.30 [95% CI, 1.15-1.46]; P < .001) were associated with increased mortality. Treatment with surgery (HR, 0.23 [95% CI, 0.12-0.44]; P < .001) or valvuloplasty (HR, 0.24 [95% CI, 0.06-0.95]; P = .042) were associated with lower mortality. Higher country income level was associated with lower mortality after adjustment for patient-level factors. Conclusions and Relevance: Mortality in RHD is high and is correlated with the severity of valve disease. Valve surgery and valvuloplasty were associated with substantially lower mortality. Study findings suggest a greater need to improve access to surgical and interventional care, in addition to the current approaches focused on antibiotic prophylaxis and anticoagulation.

How Many Doses Make a Difference? An Analysis of Secondary Prevention of Rheumatic Fever and Rheumatic Heart Disease.

Background Acute rheumatic fever ( ARF ) and rheumatic heart disease cause substantial burdens worldwide. Long-term antibiotic secondary prophylaxis is used to prevent disease progression, but evidence for benefits of different adherence levels is limited. Using data from northern Australia, we identified factors associated with adherence, and the association between adherence and ARF recurrence, progression to rheumatic heart disease, worsening or improvement of rheumatic heart disease, and mortality. Methods and Results Factors associated with adherence (percent of doses administered) were analyzed using logistic regression. Nested case-control and case-crossover designs were used to investigate associations with clinical outcomes; conditional logistic regression was used to estimate odds ratios ( OR ) with 95% CIs Adherence estimates (7728) were analyzed. Being female, younger, having more-severe disease, and living remotely were associated with higher adherence. Alcohol misuse was associated with lower adherence. The risk of ARF recurrence did not decrease until ≈40% of doses had been administered. Receiving <80% was associated with a 4-fold increase in the odds of ARF recurrence (case-control OR : 4.00 [95% CI : 1.7-9.29], case-crossover OR : 3.31 [95% CI : 1.09-10.07]) and appeared to be associated with increased all-cause mortality (case-control OR : 1.90 [95% CI : 0.89-4.06]; case-crossover OR 1.91 [95% CI : 0.51-7.12]). Conclusions We show for the first time that increased adherence to penicillin prophylaxis is associated with reduced ARF recurrence, and a likely reduction in mortality, in our setting. These findings can motivate patients to receive doses since even relatively low adherence can be beneficial, and additional doses further reduce adverse clinical outcomes.

Predictive performance of six mortality risk scores and the development of a novel model in a prospective cohort of patients undergoing valve surgery secondary to rheumatic fever.

BACKGROUND: Mortality prediction after cardiac procedures is an essential tool in clinical decision making. Although rheumatic cardiac disease remains a major cause of heart surgery in the world no previous study validated risk scores in a sample exclusively with this condition. OBJECTIVES: Develop a novel predictive model focused on mortality prediction among patients undergoing cardiac surgery secondary to rheumatic valve conditions. METHODS: We conducted prospective consecutive all-comers patients with rheumatic heart disease (RHD) referred for surgical treatment of valve disease between May 2010 and July of 2015. Risk scores for hospital mortality were calculated using the 2000 Bernstein-Parsonnet, EuroSCORE II, InsCor, AmblerSCORE, GuaragnaSCORE, and the New York SCORE. In addition, we developed the rheumatic heart valve surgery score (RheSCORE). RESULTS: A total of 2,919 RHD patients underwent heart valve surgery. After evaluating 13 different models, the top performing areas under the curve were achieved using Random Forest (0.982) and Neural Network (0.952). Most influential predictors across all models included left atrium size, high creatinine values, a tricuspid procedure, reoperation and pulmonary hypertension. Areas under the curve for previously developed scores were all below the performance for the RheSCORE model: 2000 Bernstein-Parsonnet (0.876), EuroSCORE II (0.857), InsCor (0.835), Ambler (0.831), Guaragna (0.816) and the New York score (0.834). A web application is presented where researchers and providers can calculate predicted mortality based on the RheSCORE. CONCLUSIONS: The RheSCORE model outperformed pre-existing scores in a sample of patients with rheumatic cardiac disease.

Psychiatric comorbidity increases mortality in immune-mediated inflammatory diseases.

OBJECTIVE: We determined the association between any common mental disorder (CMD: depression, anxiety disorder, bipolar disorder) and mortality and suicide in three immune-mediated inflammatory diseases (IMID), inflammatory bowel disease (IBD), multiple sclerosis (MS) and rheumatoid arthritis (RA), versus age-, sex- and geographically-matched controls. METHODS: Using administrative data, we identified 28,384 IMID cases (IBD: 8695; MS: 5496; RA: 14,503) and 141,672 matched controls. We determined annual rates of mortality, suicide and suicide attempts. We evaluated the association of any CMD with all-cause mortality and suicide using multivariable Cox regression models. RESULTS: In the IMID cohort, any CMD was associated with increased mortality. We observed a greater than additive interaction between depression and IMID status (attributable proportion 5.2%), but a less than additive interaction with anxiety (attributable proportion -13%). Findings were similar for MS and RA. In IBD, a less than additive interaction existed with depression and anxiety on mortality risk. The IMID cohort with any CMD had an increased suicide risk versus the matched cohort without CMD. CONCLUSION: CMD are associated with increased mortality and suicide risk in IMID. In MS and RA, the effects of depression on mortality risk are greater than associations of these IMID and depression alone.

The WHF Roadmap for Reducing CV Morbidity and Mortality Through Prevention and Control of RHD.

Rheumatic heart disease (RHD) is a preventable non-communicable condition that disproportionately affects the world's poorest and most vulnerable. The World Heart Federation Roadmap for improved RHD control is a resource designed to help a variety of stakeholders raise the profile of RHD nationally and globally, and provide a framework to guide and support the strengthening of national, regional and global RHD control efforts. The Roadmap identifies the barriers that limit access to and uptake of proven interventions for the prevention and control of RHD. It also highlights a variety of established and promising solutions that may be used to overcome these barriers. As a general guide, the Roadmap is meant to serve as the foundation for the development of tailored plans of action to improve RHD control in specific contexts.

Long-Term Outcomes From Acute Rheumatic Fever and Rheumatic Heart Disease: A Data-Linkage and Survival Analysis Approach.

BACKGROUND: We investigated adverse outcomes for people with acute rheumatic fever (ARF) and rheumatic heart disease (RHD) and the effect of comorbidities and demographic factors on these outcomes. METHODS: Using linked data (RHD register, hospital, and mortality data) for residents of the Northern Territory of Australia, we calculated ARF recurrence rates, rates of progression from ARF to RHD to severe RHD, RHD complication rates (heart failure, endocarditis, stroke, and atrial fibrillation), and mortality rates for 572 individuals diagnosed with ARF and 1248 with RHD in 1997 to 2013 (94.9% Indigenous). RESULTS: ARF recurrence was highest (incidence, 3.7 per 100 person-years) in the first year after the initial ARF episode, but low-level risk persisted for >10 years. Progression to RHD was also highest (incidence, 35.9) in the first year, almost 10 times higher than ARF recurrence. The median age at RHD diagnosis in Indigenous people was young, especially among males (17 years). The development of complications was highest in the first year after RHD diagnosis: heart failure incidence rate per 100 person-years, 9.09; atrial fibrillation, 4.70; endocarditis, 1.00; and stroke, 0.58. Mortality was higher among Indigenous than non-Indigenous RHD patients (hazard ratio, 6.55; 95% confidence interval, 2.45-17.51), of which 28% was explained by comorbid renal failure and hazardous alcohol use. RHD complications and mortality rates were higher for urban than for remote residents. CONCLUSIONS: This study provides important new prognostic information for ARF/RHD. The residual Indigenous survival disparity in RHD patients, which persisted after accounting for comorbidities, suggests that other factors contribute to mortality, warranting further research.

Mortality and hospitalisation costs of rheumatic fever and rheumatic heart disease in New Zealand.

AIMS: To estimate the annual mortality and the cost of hospital admissions for acute rheumatic fever (ARF) and rheumatic heart disease (RHD) for New Zealand residents. METHODS: Hospital admissions in 2000-2009 with a principal diagnosis of ARF or RHD (ICD9_AM 390-398; ICD10-AM I00-I099) and deaths in 2000-2007 with RHD as the underlying cause were obtained from routine statistics. The cost of each admission was estimated by multiplying its diagnosis-related group (DRG) cost weight by the national price for financial year 2009/2010. RESULTS: There were on average 159 RHD deaths each year with a mean annual mortality rate of 4.4 per 100, 000 (95% confidence limit 4.2, 4.7). Age-adjusted mortality was five- to 10-fold higher for Maori and Pacific peoples than for non-Maori/Pacific. The mean age at RHD death (male/female) was 56.4/58.4 for Maori, 50.9/59.8 for Pacific and 78.2/80.6 for non-Maori, non-Pacific men and women. The average annual DRG-based cost of hospital admissions in 2000-2009 for ARF and RHD across all age groups was $12.0 million (95% confidence limit $11.1 million, $12.8 million). Heart valve surgery accounted for 28% of admissions and 71% of the cost. For children 5-14 years of age, valve surgery accounted for 7% of admissions and 27% of the cost. Two-thirds of the cost occurs after the age of 30. CONCLUSIONS: ARF and RHD comprise a burden of mortality and hospital cost concentrated largely in middle age. Maori and Pacific RHD mortality rates are substantially higher than those of non-Maori/Pacific.

Understanding rheumatic fever.

Through a comprehensive review of the recent findings on rheumatic fever, we intend to propose a new physiopathologic model for this disease. A Medline search was performed for all articles containing the terms rheumatic fever or rheumatic heart disease in title or abstract from 1970 to 2011. Best evidence qualitative technique was used to select the most relevant. The scientific interest on rheumatic fever has notably diminished throughout the twentieth century as evidenced by the comparison of the proportion of articles in which RF was a subject in 1950 (0.26%) and today (0.03%) [Pubmed]. However, RF remains a major medical and social problem in the developing world and in the so-called hotspots, where it still causes around 500.000 deaths each year, not too different from the pre-antibiotic era. The role of genetic factors in RF susceptibility is discussed. Familiar aggregation, similarity of disease patterns between siblings, identical twin, and HLA correlation studies are evidence for a genetic influence on RF susceptibility. The suspect-involved genes fall mainly into those capable of immunologic mediation. Molecular mimicry explains the triggering of RF, but an intense and sustained inflammation is needed to cause sequels. Also, RF patients vary greatly in terms of symptoms. It is likely that a genetic background directing immune response towards a predominantly Th1 or Th2 pattern contributes to these features. The recent findings on rheumatic fever provide important insight on its physiopathology that helps understanding this prototype post-infectious autoimmune disease giving insights on other autoimmune conditions.

First-episode versus recurrent acute rheumatic fever: is it different?

BACKGROUND: Recurrent episodes of acute rheumatic fever (RF) can lead to rheumatic heart disease with considerable disability and mortality in children. RF can recur in the absence of secondary prophylaxis. The differences in clinical manifestations and outcome between first-episode and recurrent RF have been less studied. METHODS: A cross-section of patients under 14 years was studied for 2 years (2003-2005) in order to compare the clinical, laboratory, echocardiographic profile and outcome of first-episode RF with recurrent attacks, and risk factors for recurrence and mortality. Patients without a previous history of RF and/or mitral stenosis (MS) and/or aortic stenosis (AS) were defined as first-episode patients, and patients with previous history of RF and/or MS and/or AS, were defined as recurrent RF patients based on the Jones criteria. RESULTS: Of 51 patients in total, 26 had first-episode RF and 25 had recurrent RF. Arthritis occurred in a significantly higher number of first-episode patients (P = 0.047) whereas shortness of breath (SOB; P = 0.003), palpitation (P = 0.034), and aortic regurgitation (AR; P = 0.001) occurred in a significantly higher number of recurrent RF patients. Audible murmur of corresponding echocardiographic regurgitation was present in all recurrent RF patients whereas audible murmur was present in 61.5% and echocardiographic regurgitation in 81% in first-episode patients (P = 0.007). Palpitation, SOB, audible murmur, thrill, age and AR on admission were independent predictors of recurrence. Palpitation, age and AS on admission were independent predictors of mortality. CONCLUSIONS: Subclinical carditis occurred only in the first-episode patients, which requires further evaluation for clinical significance. Because all deaths occurred in recurrent RF group (P = 0.02), secondary prophylaxis and management of sore throat need re-emphasis.

Comparison of mechanical and biological prostheses when used to replace heart valves in children and adolescents with rheumatic fever.

OBJECTIVE: To assess the outcomes in children and adolescents with rheumatic fever of the implantation of mechanical as opposed to biological heart valves. METHODS: We assessed 73 patients with rheumatic heart disease under the age of 18 years, who underwent replacement of heart valves between January, 1996, and December, 2005, at the National Institute of Cardiology in Rio de Janeiro, Brazil. Of the group, 71 patients survived, and were divided into a group of 52 receiving mechanical prostheses, and 19 with biological prostheses. We compared endpoints between the groups in terms of mortality, reoperation, haemorrhage, and stroke. Survival curves were estimated using the Kaplan-Meier method and were compared by the Mantel (log-rank) test. RESULTS: Overall mortality was 8.2%. In those receiving mechanical prostheses, 2 (3.8%) patients died, 5 (9.6%) underwent reoperation, 2 (3.8%) suffered severe haemorrhage, and 3 (5.8%) had strokes. In those receiving biological valves, 2 (10.5%) patients died, and 4 (21%) underwent reoperation. After 2, 4, and 8 years, overall survival was 96%, 93% and 86%, respectively, with a borderline difference between the groups (p = 0.06). The probabilities of remaining free from reoperation (p = 0.13), and from combined endpoints, showed no statistically significant difference between the groups (p = 0.28). CONCLUSIONS: Patients with mechanical prostheses had lower mortality and required fewer reoperations, but when all combined endpoints were considered, the groups did not differ. The biological prosthesis proved to be a good option for cardiac surgery in children and adolescents with difficulties or risks of anticoagulation.

Prevention and control of rheumatic fever and rheumatic heart disease: the Cuban experience (1986-1996-2002).

BACKGROUND: Rheumatic fever (RF) and rheumatic heart disease (RHD) are still major medical and public health problems mainly in developing countries. Pilot studies conducted during the last five decades in developed and developing countries indicated that the prevention and control of RF/RHD is possible. During the 1970s and 1980s, epidemiological studies were carried out in selected areas of Cuba in order to determine the prevalence and characteristics of RF/RHD, and to test several long-term strategies for prevention of the diseases. METHODS: Between 1986 and 1996 we carried out a comprehensive 10-year prevention programme in the Cuban province of Pinar del Rio and evaluated its efficacy five years later. The project included primary and secondary prevention of RF/RHD, training of personnel, health education, dissemination of information, community involvement and epidemiological surveillance. Permanent local and provincial RF/RHD registers were established at all hospitals, policlinics and family physicians in the province. Educational activities and training workshops were organised at provincial, local and health facility level. Thousands of pamphlets and hundreds of posters were distributed, and special programmes were broadcast on the public media to advertise the project. RESULTS: There was a progressive decline in the occurrence and severity of acute RF and RHD, with a marked decrease in the prevalence of RHD in school children from 2.27 patients per 1,000 children in 1986 to 0.24 per 1,000 in 1996. A marked and progressive decline was also seen in the incidence and severity of acute RF in five- to 25-year-olds, from 18.6 patients per 100,000 in 1986 to 2.5 per 100,000 in 1996. There was an even more marked reduction in recurrent attacks of RF from 6.4 to 0.4 patients per 100,000, as well as in the number and severity of patients requiring hospitalisation and surgical care. Regular compliance with secondary prophylaxis increased progressively and the direct costs related to treatment of RF/RHD decreased with time. The implementation of the programme did not incur much additional cost for healthcare. Five years after the project ended, most of the measures initiated at the start of the programme were still in place and occurrence of RF/RHD was low.

Doença reumática: negligenciada, mas ainda presente e mortal / Rheumatic disease: nelected but still present and deadly

A Febre Reumática é uma doença freqüente e que inflige sérias conseqüências a um numeroso grupo de pacientes. É causa freqüente de cirurgia cardíaca em nosso meio. Sua fisiopatologia é complexa e as primeiras manifestações são extremamente variáveis, fazendo com que seja de difícil diagnóstico. O artigo faz um resumo da fisiopatologia atual, quadro clínico e tratamentos mais indicados para pacientes com Febre Reumática. Deve ser enfatizada a profilaxia, especialmente a profilaxia primária, que impede o desenvolvimento da doença.

Rheumatic fever is a frequent disease in Brazil, and leads to serious long-termconsequences, such as chronic rheumatic heart disease. Cardiac surgery due to rheumatic sequelae is quite common, having tragic consequences for the young population. The complex physiopathology and variable onset symptoms make the diagnosis of rheumatic fever a trickyone. The article summarizes the current physiopathology, diagnostic and treatment for the manifestations of rheumatic fever, acute and chronic. Special emphasis on primary prophylaxisis advised, as it prevents the development of the disease.

[Rheumatic fever and rheumatic heart disease]. / La malattia reumatica: focus sulla cardite reumatica cronica.

Rheumatic heart disease, the sequel of acute rheumatic fever, is a very common cause of cardiovascular mortality and morbidity all over the world, and is the predominant indication for cardiac surgery in the industrialized countries. Diagnosis of rheumatic chronic carditis may sometimes be difficult because valvular regurgitation may not always be detected by routine clinical auscultation. A recent report from the World Health Organization Expert Committee recognizes the usefulness of echocardiography Doppler in providing supporting evidence for diagnosis of rheumatic carditis in the presence of equivocally pathological murmur, and recommends that patients with subclinical carditis should be managed as rheumatic heart disease until proven otherwise, because the disease still represents a major health problem. The aim of this review is to give an update on the disease by underlining changes made by the World Health Organization on disease diagnosis and patient management.

Acute severe mitral regurgitation during first attacks of rheumatic fever: clinical spectrum, mechanisms and prognostic factors.

BACKGROUND AND AIM OF THE STUDY: The study aim was to describe the clinical spectrum and mechanism of acute severe mitral regurgitation (MR) observed during first episodes of rheumatic fever (RF), and to identify prognostic factors related to the short-term outcome. METHODS: Since 1990, 44 patients (mean age 9.2 +/- 0.1 years; range: 4-17 years) have been admitted to the authors' institution with severe MR related to a first episode of RF, fulfilling revised Jones' criteria. Twenty-three patients admitted between 1995 and 2002 were included prospectively, and 21 admitted before 1994 were studied retrospectively. RESULTS: Left ventricular end-diastolic and end-systolic dimensions were 51 +/- 2 mm (46 +/- 3 mm/m2 BSA) and 32 +/- 2 mm (28 +/- 2 mm/m2 BSA), respectively; mean fractional shortening of the left ventricle was 39.0 +/- 1.0% (range: 31-52%); Doppler-derived pulmonary arterial systolic pressure (PAPS) was 51 +/- 6 mm (range: 27-90 mm). The mitral valve annulus was enlarged in all patients (mean diameter 31 +/- 2 mm; 27 +/- 4 mm/m2 BSA). MR resulted from prolapse of the anterior mitral valve leaflet (P of AMVL) in 16 patients (36%), and from prolapse of the posterior mitral valve leaflet (P of PMVL) in nine (20%); the other 19 patients (43%) had restrictive motion of the PMVL, with normal motion of the AMVL, resulting in a 'false prolapse' of the AMVL (FP of AMVL). During the six-month interval following the RF episode, mitral valve surgery was required in 11 patients (25%); three patients (7%) died from cardiogenic shock before they could undergo surgery, while the other 30 patients were stabilized under medical treatment. Using univariate analysis, death or mitral valve surgery was associated with PAPS > 50 mm (OR = 1.7, p = 0.04), male gender (OR = 1.88, p = 0.008), clinical signs of congestive heart failure at admission (OR = 2.7, p < 10(-4)), and prolapse of the PMVL (OR = 5.2, p = 0.01). Death occurred, or mitral valve surgery was necessary, in eight patients with P of PMVL (89%), in four with P of AMVL (25%), and in two with FP of AMVL (11%) (p < 0.001). Despite limitations due to co-linearities and small sample size, multivariate analysis identified P of PMVL as the most potent predictor of adverse outcome. The long-term follow up (mean 6.3 years) of patients without P of PMVL, alive and not operated on during the first six-month interval after an RF episode, demonstrated a sharp decrease in the mean severity of MR (from grade 4 to 1.7; range: 1-3). CONCLUSION: In contrast to previous reports of chronic rheumatic MR, acute severe MR due to RF is more frequently related to P of AMVL or P of PMVL, than to FP of AMVL. Patients with P of AMVL or FP of AMVL tend to improve with medical treatment; however, those with P of PMVL carry a poor medical prognosis, and most often require early mitral valve surgery.

Long term follow up of rheumatic fever and predictors of severe rheumatic valvar disease in Brazilian children and adolescents.

OBJECTIVES: To investigate the progress of rheumatic fever (RF) and the predictors of severe chronic valvar disease. DESIGN: Patients prospectively followed up since their first attack of acute RF (ARF). SETTING: Universidade Federal de Minas Gerais, Brazil. PATIENTS: 258 children and adolescents who met the revised Jones criteria for RF. The follow up period ranged from 2-15 years. MAIN OUTCOME MEASURES: The presence and severity of mitral or aortic valvar disease were determined by both clinical and Doppler echocardiographic examinations. The variables associated with severe chronic valvar disease were initially identified by the Kaplan-Meier method and, later, by multivariate analysis. RESULTS: Doppler echocardiography of 258 patients studied showed that 186 (72.1%) developed chronic valvar disease and 41 (15.9%) progressed to severe chronic mitral or aortic lesions. Of 146 patients who developed carditis, 49 (33.6%) had a normal clinical examination in the chronic phase but only nine (6.2%) had normal Doppler echocardiographic findings--that is, 40 (27.4%) patients progressed to chronic subclinical valvar disease. Moderate or severe carditis, recurrences of ARF, and mother's low educational level were risk factors in predicting severe chronic valvar diseases. CONCLUSION: The increased risk of progressing to severe chronic valvar disease was associated with moderate or severe carditis, recurrences of ARF, and mother's low educational level. Hence, in a country such as Brazil, the options available for disease control are mainly primary and secondary prophylaxis.

[The causes of death of patients with rheumatic diseases in Moscow]. / Prichiny smerti bol'nykh s revmaticheskimi zabolevaniiami v Moskve.

The study was held in order to analyze the main causes of death in cases of rheumatic diseases (RD) in Moscow. The authors studied the pathology records of autopsies performed in 1999-2002 in two pathology departments of Moscow clinics. Cases with RD were selected. The study found 165 cases of RD, which constituted 2% of all autopsies performed in these departments. There were 99 cases (60%) of rheumatic heart disease (RHD), 4 cases (2.4%) of rheumatic fever (RF) relapse, 28 cases (17%) of rheumatoid arthritis (RA), 8 cases (4.8%) of systemic lupus erythematosus (SLE), 3 cases (1.8%) of scleroderma systematica (SS), 2 cases (1.2%) of ankylosing spondylitis (AS), 2 cases (1.2%) of systemic vasculitis (SPV), 11 cases (7.3%) of osteoarthrosis, 3 cases (1.8%) of gout, 1 case (0.6%) of polymyositis. The death of patients with RHD had been caused by hemodynamic decompensation (HD) in 54% of the cases, acute cardiovascular collapse (ACC) in 14% of the cases, 6% of the patients had died from thromboembolism (TE) and 26%--from other conditions (intoxication, uremia, brain and lung edema etc). The death of patients with RF was caused by TE in 2 cases, by HD in 1 case and by ACC in 1 case. Secondary amyloidosis resulting in chronic renal failure and uremia occurred in 5 out of 28 cases of RA, HD--in 3, ACC--in 7, TE--in 1, infectious complications--in 5, other complications--in 7 cases. Patients with SLE died from various conditions: uremia in 2 cases, acute adrenal failure in 1 case, infectious complications in 2, ACC--in 2, brain edema--in 1 case. The complications of SS were uremia and intoxication. ACC was the cause of death in cases of gout and SS. The majority of RD cases were patients with RHD. The main cause of death in RD was cardiovascular disorders.

Acute rheumatic fever: a chink in the chain that links the heart to the throat?

Acute rheumatic fever (ARF) remains a major problem in tropical regions, resource-poor countries, and minority indigenous communities. It has long been thought that group A streptococcal (GAS) pharyngitis alone was responsible for acute rheumatic fever; this belief has been supported by laboratory and epidemiological evidence gathered over more than 60 years, mainly in temperate climates where GAS skin infection is uncommon. GAS strains have been characterised as either rheumatogenic or nephritogenic based on phenotypic and genotypic properties. Primary prevention strategies and vaccine development have long been based on these concepts. The epidemiology of ARF in Aboriginal communities of central and northern Australia challenges this view with reported rates of ARF and rheumatic heart disease (RHD) that are among the highest in the world. GAS throat colonisation is uncommon, however, and symptomatic GAS pharyngitis is rare; pyoderma is the major manifestation of GAS infection. Typical rheumatogenic strains do not occur. Moreover, group C and G streptococci have been shown to exchange key virulence determinants with GAS and are more commonly isolated from the throats of Aboriginal children. We suggest that GAS pyoderma and/or non-GAS infections are driving forces behind ARF in these communities and other high-incidence settings. The question needs to be resolved as a matter of urgency because current approaches to controlling ARF/RHD in Aboriginal communities have clearly been ineffective. New understanding of the pathogenesis of ARF would have an immediate effect on primary prevention strategies and vaccine development.