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.

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.

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.

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.

Tendencia en la mortalidad por fiebre reumática aguda y cardiopatía reumática crónica en Venezuela, 1955-1994 / Mortality trends for acute rheumatic fever and chronic rheumatic heart disease in Venezuela, 1955-1994

Incluye defunciones por fiebre reumática aguda (FRA) y cardiopatia reumática crônica (CRC) registradas en Venezuela de 1955 a 1994, según edad y sexo. Se calcularon tasas de mortalidad por 100.000 habitantes, ajustadas según método directo a la población mundial estándar de la OMS. Se produjo un descenso en la tasa ajustada de mortalidad (TAM) por FR para ambos sexos de 7,68 a 1,08 (Variación Porcentual [VP] -85,9 por cento); sexo masculino de 7,53 a 0,84 (VP -88,8 por cento) y sexo femenino de 7,83 a 1,33 (VP -83,0 por cento). Las TAM fueron mayores en el sexo femenino. La proporción de muertes por FR sobre las producidas por enfermedades circulatorias (CIE-6 330-334,400-447; CIE-7 330-334,400-447; CIE-8 390-438; CIE-9 390-438) descendió en 88,5 por cento. Se observó, para ambos sexos, un descenso en las TAM por FRA (VP -93,5 por cento) y CRC (VP -85,1 por cento). El porcentaje de defunciones por FRA y CRC ocurridas en menores de 45 años mostró una tendencia ascendente. La reducción en las TAM por FR puede ser atribuible a mejoras socioeconómicas, así como a prevención con penicilina. La estabilización en las TAM a partir de 1980 podría explicarse por el deterioro socioeconómico experimentado en Venezuela y por la aparición de cepas más virulentas del EBHA.

Accidente vascular cerebral en paciente joven / Cerebral vascular accident in young person

Mujer de 25 años de edad de área rural quien ingresó con cuadro de accidente vascular cerebral referida del Hospital de Upala. Se detectó por TAC infarto cerebral en lóbulo pariental izquierdo y se encontraron datos en corazón correspondiente a valvulopatía mitral con estenosis que se atribuyó en la autopsia a fiebre reumática. Presentó émbolo a circulación sistémica que fue causante del infarto cerebral. Falleció con signos histológicos francos de insuficiencia cardiaca global. Se atribuye en la literatura inadecuada prevención de la fiebre reumática en los países en vías de desarrollo y se anotan respuestas inmunes anormales a la faringitis estreptocóccica.

Demanda hospitalaria, manifestaciones clínicas y prevención de fiebre reumática y cardiopatía en el Instituto de Salud del Niño 1989-1993 / Hospital demand, clinical sign and prevention of rheumatic fever and rheumatic cardiopathy in the Instituto de Salud del Niño 1989-1993

Se estudiaron retrospectivamente 66 pacientes hospitalizados en el Servicio de Cardiología del Instituto de Salud del Niño-entre 1989 y 1993- con el diagnóstico de fiebre reumática y cardiopatía reumática. Se encontró un aumento significativo en los 3 últimos años. El promedio en días de hospitalización se duplicó en 1993 respecto a 1989. El grupo etáreo más afectado fue entre los 11-15 años, con 39 pacientes (59 por ciento). El 74.5 por ciento tuvieron clase funcional III o IV, el 98 por ciento compromiso cardíaco; la lesión valvular más encontrada fue de insuficiencia aórtica (41 por ciento); la complicación más frecuente fue endocarditis infecciosa en 15 pacientes (23 por ciento). Fueron operados 8 pacientes sin mortalidad quirúrgica, 4 pacientes (6 por ciento) fallecieron de complicaciones clínicas. El severo compromiso cardiovascular mostrado en nuestra casuística se debe a una recurrencia elevada de carditis reumática por una ineficaz prevención. Ello hace imprescindible la implementación de un programa ampliado de prevención que considere la administración de fármacos y las condiciones sociales del enfermo para mejorar sus condiciones de vida.

Fiebre reumática: incidencia, prevalencia y aspectos clinicoepidemiológicos / Rheumatic fever: incidence, prevalence and clinico-epidemiological aspects

Se estudia la mortalidad, incidencia, prevalencia y algunas características clínicas y epidemiológicas de la fiebre reumática (FR), y la enfermedad reumática (ERC) en 3 provincias del pais (Ciudad de La Habana, Santiago de Cuba y Pinar del Rio). Se utilizó em método epidemiológico, basado en el estudio de estadísticas vitales, aplicación de registros permanente de la enfermedad y encuesta a la población escolar en las provincias escogidas. Se utilizaron los criterios de Jones revisados y modificados. La mortalidad por ERC y FR en todas las edades en Cuba disminuyó desde 6,00 por cada 100 000 en 1963 a 1965, hasta tasas alrededor de 2,0 en los años 80. La mortalidad por FR aguda está por debajo de 0,2. La incidencia de FR aguda primer ataque es de 12,0 por 100 000 más alta en las provincias de Pinar del Río y Santiago de Cuba. Es mayor en las edades de 5 a 9 años y similar en uno y otro sexo. La enfermedad se presenta con más severidad en las recurrencias y en las provincias de Santiago de Cuba y Pinar del Río. La prevalencia total fue de 5.8 por 1 000 y fue más alta en las provincias de Santiago de Cuba y Pinar del Río (6.5 y 8,5). Correspondieron a estas provincias las frecuencias más elevadas de cardiopatía reumática, así como un mayor número de pacientes que ignoraban que padecían la enfermedad

Ethnic differences in mortality from acute rheumatic fever and chronic rheumatic heart disease in New Mexico, 1958-1982.

To examine time trends and differences in mortality rates from acute rheumatic fever and chronic rheumatic heart disease in New Mexico's Hispanic, American Indian, and non-Hispanic white populations, we analyzed vital records data for 1958 through 1982. Age-adjusted mortality rates for acute rheumatic fever were low and showed no consistent temporal trends among the three ethnic groups over the study period. Age-adjusted and age-specific mortality rates for chronic rheumatic heart disease in Hispanic and non-Hispanic whites decreased over the 25-year period, although rates were higher among Hispanics than among non-Hispanics during most of the time period. In American Indians, age-adjusted mortality rates for chronic rheumatic heart disease increased between 1968 and 1977 to twice the non-Indian mortality rates during the same period. Despite this increase in mortality from chronic rheumatic heart disease among New Mexico's American Indians from 1968 to 1977, the New Mexico data generally reflect national trends of decreasing mortality from chronic rheumatic heart disease.

Severe acute rheumatic fever: recent trends in the severity of the disease - abstract

Acute rheumatic fever remains a common disease in Jamaica. Between August, 1973 and July, 1978, there was only one death recorded among children under 12 years admitted to the University Hospital of the West Indies with this disease. There were 38 - 48 annual admissions for the disease during this period. Between August, 1978 and July 1979, there were 26 admissions for rheumatic fever. Five of these children presented with an unusually severe form of acute rheumatic fever which led to a fatal outcome in four cases. Only one of these children had previous admissions for the disease and he had received no penicillin prophylaxis for the preceding four months. The only surviving child developed frank pulmonary oedema within a few hours of admission and required ventilation for 5 days. Post-mortem findings in 4 cases showed rheumatic pancarditis, oedema and congestion of the lung and congestion of the liver. Decline in the incidence and severity of acute rheumatic fever has been reported from several centres since the 1920s. These recent cases of severe acute rheumatic fever may represent an incidental clustering of cases or may indicate that the severity of the disease is increasing in this population (AU)

Acute rheumatic fever in Jamaican children

Forty-four cases of acute rheumatic fever in children admitted to the University College Hospital over four years are reviewed. The disease is not uncommon in Jamaica and its symptomatology in general is similar to that found in temperate climates. Chorea was rare in the series reported while nodules and erythema marginatum were not seen. The disease affects slightly younger age groups than in temperate climates and is severe with a high mortality and high incidence of cardiac complications.(Summary)