Fatores intervenientes na gerência do cuidado de enfermagem à criança hospitalizada com cardiopatia reumática / Intervening factors in the management of nursing care for children hospitalized with rheumatic heart disease / Factores que intervienen en la gestión del cuidado de enfermería al niño hospitalizado con cardiopatía reumática

Objetivo: analisar os fatores intervenientes na gerência do cuidado de enfermagem à criança hospitalizada com cardiopatia reumática. Método: estudo descritivo-exploratório com abordagem qualitativa, que utilizou a Teoria Fundamentada em Dados e o Interacionismo Simbólico, respectivamente, como referencial metodológico e teórico. A coleta de dados foi realizada em uma instituição especializada em atendimento cardiológico, no munícipio do Rio de Janeiro. Foram entrevistados 19 profissionais de enfermagem através de um roteiro semiestruturado. Resultado: emergiram os seguintes fatores intervenientes na prática da gerência do cuidado: condição socioeconômica da família, comportamento da criança, condições de trabalho, comunicação ineficaz, educação permanente, trabalho em equipe e experiência profissional. Conclusão: os resultados apontam para a necessidade de proposição de estratégias de ação e interação que facilitem a prática gerencial de cuidado à criança com cardiopatia reumática e sua família face aos fatores intervenientes identificados.

Objective: to analyze the factors involved in the management of nursing care for children hospitalized with rheumatic heart disease. Method: this is a descriptive-exploratory study with a qualitative approach, which used Data-Based Theory and Symbolic Interactionism, respectively, as methodological, and theoretical references. Data was collected in an institution specializing in cardiac care in the city of Rio de Janeiro. Nineteen nursing professionals were interviewed using a semi-structured script. Result: the following intervening factors in the practice of care management emerged: the family's socioeconomic status, the child's behavior, working conditions, ineffective communication, continuing education, teamwork, and professional experience. Conclusion: the results point to the need to propose strategies for action and interaction that facilitate management practice in caring for children with rheumatic heart disease and their families, given the intervening factors identified.

Objetivo: analizar los factores que intervienen en la gestión del cuidado de enfermería al niño hospitalizado con cardiopatía reumática. Método: estudio descriptivo-exploratorio con enfoque cualitativo, cuyos marcos metodológico y teórico fueron la Teoría Fundamentada y el Interaccionismo Simbólico, respectivamente. La recolección de datos se realizó en una institución especializada en atención cardiológica, en la ciudad de Río de Janeiro. Fueron entrevistados 19 profesionales de enfermería mediante un cuestionario semiestructurado. Resultado: surgieron los siguientes factores intervinientes en la práctica de la gestión del cuidado: condición socioeconómica de la familia, comportamiento del niño, condiciones de trabajo, comunicación ineficaz, educación continua, trabajo en equipo y experiencia profesional. Conclusión: los resultados indican que es necesario proponer estrategias de acción e interacción que faciliten la práctica de la gestión del cuidado al niño con cardiopatía reumática y a sus familiares, con respecto a los factores intervinientes identificados.

Clinical outcome of patients with rheumatic tricuspid valve disease: matched cohort study.

Rheumatic heart disease (RHD) is still a major health problem, especially in low- to mid-income countries, leading premature deaths owing to valvular disease. Although left-sided valvular involvement is most commonly seen in RHD, the tricuspid valve can also be affected. However, there is a lack of information about the prognostic value of primary tricuspid valve (TV) disease in RHD. This study aimed to determine the impact of TV disease on clinical outcome in RHD. This prospective study enrolled patients with rheumatic mitral valve disease (MVD) referred to a tertiary center for management of heart valve disease. Primary rheumatic TV disease was defined by echocardiographic features including thickening of leaflets associated with some degree of restricted mobility. Patients with rheumatic TV disease were matched to patients with MVD using 1:1 genetic matching algorithm that maximized balance of baseline covariates prior to exploring outcome differences. The main outcome was either need for MV replacement or death. Among 694 patients eligible for the study, age of 47 ± 13 years, 84% female, 39 patients (5.6%) had rheumatic TV disease. After excluding patients with incomplete data, 33 patients with TV disease were matched to 33 controls based on age, right-sided heart failure, atrial fibrillation, and MV area. During a mean follow-up of 42 months (median 28, IQR 8 to 71 months), 32 patients (48.5%) experienced adverse events, including 6 cardiovascular deaths and 26 patients who underwent surgery for mitral valve replacement. The adjusted analysis demonstrated a significant association between TV disease and the outcome, with a hazard ratio (HR) of 3.386 (95% CI 1.559-7.353; P = 0.002) in the genetic matched cohort with balance on baseline covariates of interest. The model exhibited good discriminative ability, as indicated by a C-statistic of 0.837. In patients with rheumatic mitral valve disease, rheumatic TV disease significantly increased risk of adverse events compared with matched controls. The involvement of TV may express overall disease severity that adversely affects clinical outcome.

Agreement between Handheld and Standard Echocardiography for Diagnosis of Latent Rheumatic Heart Disease in Brazilian Schoolchildren from High-Prevalence Settings (Agreement between Screening and Standard Echo for RHD).

Introduction: Handheld echocardiography (echo) is the tool of choice for rheumatic heart disease (RHD) screening. We aimed to assess the agreement between screening and standard echo for latent RHD diagnosis in schoolchildren from an endemic setting. Methods: Over 14 months, 3 nonphysicians used handheld machines and the 2012 WHF Criteria to determine RHD prevalence in consented schoolchildren from Brazilian low-income public schools. Studies were interpreted by telemedicine by 3 experts (Brazil, US). RHD-positive children (borderline/definite) and those with congenital heart disease (CHD) were referred for standard echo, acquired and interpreted by a cardiologist. Agreement between screening and standard echo, by WHF subgroups, was assessed. Results: 1390 students were screened in 6 schools, with 110 (7.9%, 95% CI 6.5-9.5) being screen positive (14 ± 2 years, 72% women). Among 16 cases initially diagnosed as definite RHD, 11 (69%) were confirmed, 4 (25%) reclassified to borderline, and 1 to normal. Among 79 cases flagged as borderline RHD, 19 (24%) were confirmed, 50 (63%) reclassified to normal, 8 (10%) reclassified as definite RHD, and 2 had mild CHD. Considering the 4 diagnostic categories, kappa was 0.18. In patients with borderline RHD reclassified to non-RHD, the most frequent WHF criterion was B (isolated mitral regurgitation, 64%), followed by A (2 mitral valve morphological features, 31%). In 1 patient with definite RHD reclassified to normal, the WHF criterion was D (borderline RHD in aortic and mitral valves). After standard echo, RHD prevalence was 3.2% (95% CI 2.3-4.2). Conclusions: Although practical, RHD screening with handheld devices tends to overestimate prevalence.

Temporal Trends of Age-Adjusted Mortality Rates for Rheumatic Heart Disease in Brazil From 2000 to 2021.

Background Rheumatic heart disease (RHD) is a chronic cardiovascular condition stemming from an infectious origin, posing a substantial health burden, particularly in economically disadvantaged regions. It starts with acute rheumatic fever (ARF), a complication following group A Streptococcus infection, leading to heart valve damage and, over time, structural heart abnormalities. RHD contributes to premature deaths, especially in low-middle-income countries. Although the incidence and prevalence have generally reduced globally due to antibiotics and improved healthcare, it remains a significant public health concern in Brazil, echoing its prevalence in many developing nations around the world. RHD stands as a poignant testament to the intersection of socio-economic disparities and healthcare challenges within Brazil's diverse population. In Brazil, despite advancements in healthcare, RHD continues to impact communities, highlighting the urgent need for enhanced prevention strategies, access to quality healthcare services, and heightened awareness to combat this preventable, yet persistent, cardiac condition. Understanding the epidemiological landscape and socio-cultural factors influencing RHD in Brazil is crucial for developing targeted interventions aimed at mitigating its burden on individuals, families, and the healthcare system at large. Thus, our study focuses on analyzing age-related mortality rates linked to ARF and chronic RHD (ARHD) in Brazil from 2000 to 2021, particularly examining gender disparities. Materials and methods This retrospective cohort study employed a descriptive time-series approach, utilizing comprehensive nationwide data from Brazil spanning from 2000 to 2021 to assess trends in diverse age groups, among both sexes, enabling a detailed analysis of temporal patterns. Mortality data, extracted and categorized meticulously, were subjected to Joinpoint statistical analyses enabling comparative assessments, with average annual percent change (AAPC) and annual percent change (APC) serving as key metrics to quantify and interpret trends over the analyzed period. Results The acute RHD (ARHD)-related mortality declined over the analyzed years supported by AAPC, with higher mortality reduction in females. The age-adjusted mortality rate for "males and females" decreased from 78 to 67 deaths/100,000 from 2000 to 2021. Female mortality dropped from 85 to 69/100,000, and male mortality decreased from 73 to 63/100,000 over the same period. For ARHD, male age groups (20-29, 60-69, 70-79, 80+) showed declining mortality, while the 30-59 age group exhibited an upward. Females AAMR for chronic RHD (CRHD) decreased across all age groups, with significant reductions in the 80 years and above age group from 2000-2002 (APC: -11.94*) and steadily from 2002 onwards (APC: -1.33). Conclusions Our study revealed an overall decline in mortality rates for both acute and CRHD across both sexes. Females consistently exhibited higher mortality rates and a more pronounced reduction compared to males in both acute and CRHD. In ARHD, males experience the highest mortality in the 50-59 age group, while females have a peak in the 40-49 age group. The 60-69 age group had the highest mortality in CRHD for both sexes. Conversely, the 20-29 age group displayed the lowest mortality in CRHD, and the 80-89 age group had the lowest mortality in ARHD.

Fatal Myocarditis following COVID-19 mRNA Immunization: A Case Report and Differential Diagnosis Review.

Carditis in childhood is a rare disease with several etiologies. We report a case of infant death due to pericarditis and myocarditis after the mRNA vaccine against COVID-19 (COVIDmRNAV). A 7-year-old male child received the first dose of the COVIDmRNAV and presented with monoarthritis and a fever non-responsive to oral antibiotics. The laboratory investigation showed signs of infection (leukocytosis, high levels of c-reactive protein). His condition rapidly deteriorated, and the patient died. The autopsy identified pericardial fibrin deposits, hemorrhagic areas in the myocardium, and normal valves. A diffuse intermyocardial inflammatory infiltrate composed of T CD8+ lymphocytes and histiocytes was identified. An antistreptolysin O (ASO) dosage showed high titers. The presence of arthritis, elevated ASO, and carditis fulfills the criteria for rheumatic fever. However, valve disease and Aschoff's nodules, present in 90% of rheumatic carditis cases, were absent in this case. The temporal correlation with mRNA vaccination prompted its inclusion as one of the etiologies. In cases of myocardial damage related to COVID-19mRNAV, it appears to be related to the expression of exosomes and lipid nanoparticles, leading to a cytokine storm. The potential effects of the COVID-19mRNAV must be considered in the pathogenesis of this disease, whether as an etiology or a contributing factor to a previously initiated myocardial injury.

Outcomes of latent rheumatic heart disease: External validation of a simplified score in patients with and without secondary prophylaxis.

BACKGROUND: Secondary antibiotic prophylaxis reduces progression of latent rheumatic heart disease (RHD) but not all children benefit. Improved risk stratification could refine recommendations following positive screening. We aimed to evaluate the performance of a previously developed echocardiographic risk score to predict mid-term outcomes among children with latent RHD. METHODS: We included children who completed the GOAL, a randomized trial of secondary antibiotic prophylaxis among children with latent RHD in Uganda. Outcomes were determined by a 4-member adjudication panel. We applied the point-based score, consisting of 5 variables (mitral valve (MV) anterior leaflet thickening (3 points), MV excessive leaflet tip motion (3 points), MV regurgitation jet length ≥ 2 cm (6 points), aortic valve focal thickening (4 points) and any aortic regurgitation (5 points)), to panel results. Unfavorable outcome was defined as progression of diagnostic category (borderline to definite, mild definite to moderate/severe definite), worsening valve involvement or remaining with mild definite RHD. RESULTS: 799 patients (625 borderline and 174 definite RHD) were included, with median follow-up of 24 months. At total 116 patients (14.5%) had unfavorable outcome per study criteria, 57.8% not under prophylaxis. The score was strongly associated with unfavorable outcome (HR = 1.26, 95% CI 1.16-1.37, p < 0.001). Unfavorable outcome rates in low (≤6 points), intermediate (7-9 points) and high-risk (≥10 points) children at follow-up were 11.8%, 30.4%, and 42.2%, (p < 0.001) respectively (C-statistic = 0.64 (95% CI 0.59-0.69)). CONCLUSIONS: The simple risk score provided an accurate prediction of RHD status at 2-years, showing a good performance in a population with milder RHD phenotypes.

Neutrophil-Lymphocyte Ratio and Red Blood Cell Distribution Width in Patients with Atrial Fibrillation and Rheumatic Valve Disease.

The lifetime risk of developing atrial fibrillation (AF) is 1 in 3 adults, resulting in a prevalence of 2-4%. Rheumatic heart disease (RHD) is a frequent aetiology of valvular heart disease in lowand middle-income countries. Between 21% and 80% of patients with mitral valve disease, especially with stenosis, may have AF. Both these conditions, AF and RHD, present a state of persistent inflammation. In turn, inflammation is a frequent cause of anisocytosis, which can be evidenced through the parameter RDW (red bold cell distribution width). Factors associated with increased RDW are also known as risk factors associated with a higher incidence of AF. RDW may have an independent role in the pathogenesis of AF and the increased propensity of both thromboembolic and bleeding events. Another marker involved in the incidence of AF is the neutrophil-lymphocyte ratio. This is also a marker of oxidative stress and inflammation and is associated with a higher rate of AF recurrence. This review will evaluate these biomarkers and their association with cardiovascular events in patients with AF and RHD. The hypotheses and current debates about the relationship of biomarkers with the severity of chronic valve dysfunction, with acute rheumatic carditis in the paediatric population, and with the presence of thrombus in the left atrium will be discussed.

Population health and sociodemographic variables as predictors of access to cardiac medicine and surgery in Haiti.

BACKGROUND: In Haiti, cardiovascular disease is a leading cause of morbidity and mortality, with congenital and rheumatic heart disease comprising a large portion of disease burden. However, domestic disparities in cardiac care access and their impact on clinical outcomes remain poorly understood. We analyzed population-level sociodemographic variables to predict cardiac care outcomes across the 10 Haitian administrative departments. METHODS: This cross-sectional study combined data from a 2016-17 Haitian national survey with aggregate outcomes from the Haiti Cardiac Alliance (HCA) database (n = 1817 patients). Using univariate and multivariable regression analyses, the proportion of HCA patients belonging to each of three clinical categories (active treatment, lost to follow-up, deceased preoperatively) was modeled in relation to six population-level variables selected from national survey data at the level of the administrative department. RESULTS: In univariate analysis, higher department rates of childhood growth retardation were associated with a lower proportion of patients in active care (OR = 0.979 [0.969, 0.989], p = 0.002) and a higher proportion of patients lost to follow-up (OR = 1.016 [1.006, 1.026], p = 0.009). In multivariable analysis, the proportion of department patients in active care was inversely associated with qualified prenatal care (OR = 0.980 [0.971, 0.989], p = 0.005), and child growth retardation (OR = 0.977 [0.972, 0.983]), p = 0.00019). Similar multivariable results were obtained for department rates of loss to follow-up (child growth retardation: OR = 1.018 [1.011, 1.025], p = 0.002; time to nearest healthcare facility in an emergency: OR = 1.004 [1.000, 1.008, p = 0.065) and for preoperative mortality (prenatal care: OR = 0.989 [0.981, 0.997], p = 0.037; economic index: OR = 0.996 [0.995, 0.998], p = 0.007; time to nearest healthcare facility in an emergency: OR = 0.992 [0.988, 0.996], p = 0.0046). CONCLUSIONS: Population-level survey data on multiple variables predicted domestic disparities in HCA clinical outcomes by region. These findings may help to identify underserved areas in Haiti, where increased cardiac care resources are required to improve health equity. This approach to analyzing clinical outcomes through the lens of population-level survey data may inform future health policies and interventions designed to increase cardiac care access in Haiti and other low-income countries.

A new surgical technique for left atrial reduction in giant left atrium.

Objective: The study objective was to evaluate the safety and clinical and echocardiographic outcomes of a new surgical technique in adult patients diagnosed with a giant left atrium. Methods: We analyzed a cohort of patients who underwent left atrium reduction surgery between January 2016 and June 2020 performed by a specialized surgical team in 2 national reference centers in Lima, Peru. We assessed the major adverse valvular-related events and the New York Heart Association functional class as primary clinical outcomes. Also, our primary echocardiographic endings were the diameter, area, and volume of the left atrium. We assessed these variables at 3 time periods: baseline (t0), perioperative period (t1), and extended follow-up (t2: 12 ± 3.4 months). We carried out descriptive and bivariate exploratory statistical analysis for dependent measures. Results: We included 17 patients, 70.6% of whom were women. Rheumatic mitral valve disease (76.5%) was the main etiology. We performed 14 (82.4%) mitral valve replacements and 3 repairs. Major adverse valvular-related events occurred in 1 patient (5.9%) (hemorrhagic stroke) at t1. A significant reduction in the size of the left atrium was observed: diameter (77 mm vs 48 mm, P < .001), area (75 cm2 vs 31 cm2, P < .001), and volume (332 cm3 vs 90 cm3, P < .001). Compared with t0 and t1, these echocardiographic findings remained without significant changes during t2. Conclusions: Our surgical left atrium reduction technique was associated with improved clinical functionality and reduced left atrium measures in patients with a giant left atrium undergoing mitral valve surgery.

Focused cardiac ultrasound in pregnancy.

Cardiac disease in pregnancy is an important cause of maternal morbidity and mortality. In many high-income countries, acquired cardiac disease is now the largest cause of maternal mortality. Given its prevalence in low- and middle-income countries (LMICs), rheumatic heart disease is the most common cause of cardiac disease in pregnancy worldwide and is associated with poor maternal outcome. The diagnosis of cardiac disease in pregnancy is often delayed resulting in excess maternal morbidity and mortality. Maternal mortality review committees have suggested that prompt recognition and treatment of heart disease in pregnancy may improve maternal outcome. Given the similarities between symptoms of normal pregnancy and those of cardiac disease, the clinical diagnosis of heart disease in pregnancy is challenging with echocardiography being the primary diagnostic modality. Focused cardiac ultrasound (FOCUS) at the point of care provides supplemental data to the history and physical examination and has been demonstrated to permit early diagnosis and improvement in the management of cardiac disease in emergency medicine, intensive care, and anesthesia. It has also been demonstrated to be useful in surveillance for rheumatic heart disease in LMICs. The use of FOCUS may allow earlier and more accurate diagnosis of cardiac disease in pregnancy with the potential to decrease morbidity and mortality in both developed and developing countries.

The role of echocardiographic screening in reducing the burden of rheumatic heart disease in Latin America / El papel del tamizaje ecocardiográfico en la reducción de la carga de la cardiopatía reumática en América Latina / O papel da triagem ecocardiográfica na redução da carga de doença cardíaca reumática na América Latina

ABSTRACT The objectives of this article are to reflect on the rationale behind the use of echocardiographic screening for rheumatic heart disease and to provide key recommendations about steps needed to implement and improve echocardiographic screening programs in Latin America. Rheumatic heart disease remains a public health problem affecting mainly low-income and lower-middle-income countries and populations. Latin America is an area with economic inequalities, and the epidemiology of rheumatic heart disease remains largely unknown. Echocardiographic screening is useful for updating the epidemiology and providing early diagnosis of the disease. We discuss different approaches used in successful echocardiographic screening programs worldwide and in Latin America. We then identify the key elements needed to establish successful echocardiographic screening programs in Latin America, including increased awareness and involvement from multiple sectors (e.g. the community, health care professionals, scientific organizations and public health entities), identification of areas in need, development of a plan and structure that include different screening approaches, and how to ensure appropriate follow up for those who screen positive.

RESUMEN Los objetivos de este artículo son reflexionar sobre los fundamentos que justifican el uso del tamizaje ecocardiográfico para detectar la cardiopatía reumática y ofrecer algunas recomendaciones importantes sobre los pasos que habría que dar para poner en marcha programas de tamizaje ecocardiográfico y mejorar los existentes en América Latina. La cardiopatía reumática sigue siendo un problema de salud pública que afecta principalmente a países y grupos poblacionales de ingresos bajos y medianos bajos. América Latina es una región de grandes desigualdades económicas y las características epidemiológicas de la cardiopatía reumática siguen siendo desconocidas en gran medida. El tamizaje ecocardiográfico resulta útil para actualizar los datos epidemiológicos y posibilitar un diagnóstico temprano de la enfermedad. En este artículo se analizan los diferentes enfoques empleados en algunos programas de tamizaje ecocardiográfico eficaces de distintas partes del mundo, incluida América Latina. A continuación se determinan los elementos clave necesarios para establecer programas eficaces de tamizaje ecocardiográfico en América Latina, incluida una mayor concientización y participación de diversos sectores (p. ej., la comunidad, los profesionales de salud, las organizaciones científicas y las entidades de salud pública), la identificación de las zonas más necesitadas, la elaboración de un plan y una estructura que incluyan diferentes abordajes del tamizaje, y el modo de garantizar un seguimiento adecuado de aquellas personas con un resultado positivo en el tamizaje.

RESUMO Os objetivos deste artigo são oferecer observações sobre a fundamentação do uso da triagem ecocardiográfica para doença cardíaca reumática e fornecer recomendações importantes sobre as etapas necessárias para implementar e melhorar os programas de triagem ecocardiográfica na América Latina. A doença cardíaca reumática continua sendo um problema de saúde pública que afeta principalmente países e populações de renda baixa e média-baixa. A América Latina é uma área com desigualdades econômicas, e a epidemiologia da doença cardíaca reumática continua amplamente desconhecida. A triagem ecocardiográfica serve para atualizar a epidemiologia e proporcionar o diagnóstico precoce da doença. Examinamos as diferentes abordagens usadas em programas de triagem ecocardiográfica bem-sucedidos em todo o mundo e na América Latina. Em seguida, identificamos os principais elementos necessários para estabelecer programas de triagem ecocardiográfica com sucesso na América Latina. Tais programas incluiriam maior conscientização e envolvimento de vários setores (por exemplo, a comunidade, profissionais de saúde, organizações científicas e entidades de saúde pública), identificação de áreas carentes, desenvolvimento de um plano e estrutura abrangendo diferentes abordagens de triagem e formas de garantir o seguimento adequado de pessoas com resultado positivo na triagem.

Neurological and Psychiatric Disorders in Patients with Rheumatic Heart Disease: Unveiling what is Beyond Cardiac Manifestations.

Background: Rheumatic heart disease (RHD) is the most serious manifestation of rheumatic fever, which may also affect the brain. The current study assessed the prevalence of neuropsychiatric manifestations in patients with RHD, including clinical features associated with basal ganglia motor dysfunction (BGMD). Methods: We conducted neurologic and psychiatric assessments in consecutive patients with RHD referred to a tertiary center for heart valve diseases. Echocardiography was performed to assess the pattern of valvular involvement and RHD severity. Validated questionnaires for the evaluation of cognition, depression, anxiety, and obsessive-compulsive symptoms (OCS) were applied. BGMD was clinically defined by the presence of hyperkinetic movement disorders. Results: Fifty patients with age of 43.2 ± 10.8 years, 84% female, were included. Mitral valve was affected in 47 patients (94%), and 21 of them (42%) also had aortic valve involvement. Chorea (22%), chronic tics (18%), OCS (48%), major depression (34%), generalized anxiety disorder (54%), cognitive complaints (66%), migraine (52%) and seizures (18%) were frequently reported. The factors associated with BGMD were age (p = 0.018), major depression (p = 0.013), and Yale-Brown Obsessive Compulsive (Y-BOCS) score (p = 0.011). The severity of heart disease was not associated with BGMD. Conclusions: Neuropsychiatric manifestations are frequent in RHD patients, which may persist up to three decades after acute rheumatic fever. Age, major depression and severity of OCS were independently associated with BGMD. These manifestations deserve a close attention of clinicians and researchers dealing with adult patients with RHD.

Current Situation of Acute Rheumatic Fever and Rheumatic Heart Disease in Latin America and the Caribbean: A Systematic Review.

Background: Rheumatic heart disease (RHD) disproportionately affects low-income and middle-income countries. Latin America and the Caribbean (LAC) have been less represented in scientific literature. We aimed to describe the epidemiology, burden and implemented screening and prevention strategies of RHD in LAC. Methods: We systematically searched PubMed, Embase, LILACS, and SciELO from 1990 to April 2021. Observational and experimental studies that described data on the epidemiology, burden, or prevention/screening strategies of RHD, regardless of age or language, were included. The risk of bias was assessed by previously published tools depending on their study design. Pre-specified data were independently extracted and presented by each topic (epidemiology, burden, prevention/screening). PROSPERO registration number: CRD42021250043. Results: Forty-eight studies out of 1692 non-duplicate records met the eligibility criteria. They were mainly from Brazil, observational in design, and hospital-based. Data on the epidemiology of acute rheumatic fever (ARF) was not recent (most before 2000) with studies describing decreasing incidence through the years. The prevalence of RHD was described in six studies, ranging from 0.24 to 48 per 1,000 among studies evaluating schoolchildren. Nine studies described data based on admissions, ranging from 0.04% to 7.1% in single-center studies. Twenty-four studies assessed the burden of RHD with most of them reporting mortality rates/proportions and complications such as the need for intervention, atrial fibrillation, or embolism. Six preventive strategies were identified that included educational, register-based, and/or secondary prophylaxis strategies. Three well-established echocardiographic screening studies in Brazil and Peru were identified. Conclusions: Most ARF/RHD research in LAC comes from a single country, Brazil where preventive/screening efforts have been conducted. There was a paucity of data from several countries in the region, reflecting the need for epidemiological studies from more countries in LAC which will provide a better picture of the current situation of ARF/RHD and guide the implementation of preventive strategies.

Long-term results of the Ross operation in rheumatic valve disease.

AIMS: The aim of this study was to evaluate the impact of rheumatic heart disease (RHD) on the long-term outcomes of the Ross operation when compared to patients with bicuspid aortic valves (BAV). METHODS AND RESULTS: We analysed patients with rheumatic disease and BAV who underwent a Ross operation between 1995 and 2020. The primary outcomes were late survival and reoperations on the pulmonary autograft and the right ventricular allograft. We also performed a propensity score analysis to adjust for differences in baseline characteristics. The cumulative incidence of the events in the matched cohorts was compared using the Fine-Gray model. There were 147 patients with rheumatic disease and 314 with bicuspid valves. In the unmatched population, patients with rheumatic disease were younger, more symptomatic, had worse left ventricular function and were more likely to present a preoperative diagnosis of aortic insufficiency. Propensity matching resulted in 86 pairs with similar baseline characteristics. Compared with those with bicuspid valves, rheumatic patients had similar 20-year survival both in the unmatched (RHD = 67.8% vs BAV = 79.7%, P = 0.27] and matched cohorts (RHD = 72.5% vs BAV = 82.9%, P = 0.46). Cumulative incidence of reoperations on the pulmonary autograft after 20 years was also comparable between them in the unmatched (RHD 13.4% vs BAV 14.4%, P = 0.67) and matched patients (RHD 11.6% vs BAV 21.1%, P = 0.47). Pulmonary autograft dysfunction due to rheumatic involvement was detected in only three occasions. Cumulative incidence of reoperations on the right-sided allograft was higher in the unmatched cohort (RHD = 5.5% vs BAV = 16.7%, P = 0.035) but became nonsignificant after propensity score matching (RHD = 9.9% vs BAV = 32.3%, P = 0.051). The cumulative incidence of all Ross-related reoperations was similar in both groups. CONCLUSIONS: Among patients undergoing Ross operation, patients with RHD had similar long-term survival and rates of reoperations, when compared to patients with BAV.

Progression of Mitral Regurgitation in Rheumatic Valve Disease: Role of Left Atrial Remodeling.

Introduction: Mitral regurgitation (MR) is the most common valve abnormality in rheumatic heart disease (RHD) often associated with stenosis. Although the mechanism by which MR develops in RHD is primary, longstanding volume overload with left atrial (LA) remodeling may trigger the development of secondary MR, which can impact on the overall progression of MR. This study is aimed to assess the incidence and predictors of MR progression in patients with RHD. Methods: Consecutive RHD patients with non-severe MR associated with any degree of mitral stenosis were selected. The primary endpoint was a progression of MR, which was defined as an increase of one grade in MR severity from baseline to the last follow-up echocardiogram. The risk of MR progression was estimated accounting for competing risks. Results: The study included 539 patients, age of 46.2 ± 12 years and 83% were women. At a mean follow-up time of 4.2 years (interquartile range [IQR]: 1.2-6.9 years), 54 patients (10%) displayed MR progression with an overall incidence of 2.4 per 100 patient-years. Predictors of MR progression by the Cox model were age (adjusted hazard ratio [HR] 1.541, 95% CI 1.222-1.944), and LA volume (HR 1.137, 95% CI 1.054-1.226). By considering competing risk analysis, the direction of the association was similar for the rate (Cox model) and incidence (Fine-Gray model) of MR progression. In the model with LA volume, atrial fibrillation (AF) was no longer a predictor of MR progression. In the subgroup of patients in sinus rhythm, 59 had an onset of AF during follow-up, which was associated with progression of MR (HR 2.682; 95% CI 1.133-6.350). Conclusions: In RHD patients with a full spectrum of MR severity, progression of MR occurs over time is predicted by age and LA volume. LA enlargement may play a role in the link between primary MR and secondary MR in patients with RHD.

Anticoagulation in Atrial Fibrillation Associated with Mitral Stenosis.

Rheumatic valve disease is present in 0.4 % of the word population, mainly in lowincome countries. Rheumatic mitral stenosis affects more women and between 40 to 75 % of patients may have atrial fibrillation (AF), more frequently in upper-middle income countries. This rhythm disturbance is due to increased atrial pressure, chronic inflammation, fibrosis, and left atrial enlargement. There is also an increase in the prevalence of AF with age in patients with mitral stenosis. The risk of stroke is 4 % per year. Success rates for cardioversion, Cox-Maze procedure, and catheter ablation are low. Therefore, anticoagulation with vitamin K antagonist is mandatory for Evaluated Heart valves, Rheumatic or Artificial (EHRA) classification type 1. However, this anticoagulation is used by less than 80 % of those eligible and less than 30 % have the international normalized ratio in the therapeutic range. The safety and efficacy of using rivaroxaban, a direct factor Xa inhibitor anticoagulant, were demonstrated in the RIVER trial with a sample of 1005 patients with AF and bioprosthetic mitral valve. The indication for valve replacement, that is, if severe mitral stenosis or severe mitral regurgitation, was not specified. A randomized, open-label study (DAVID-MS) is underway to compare the effectiveness and safety of dabigatran and warfarin therapy for stroke prevention in patients with AF and moderate or severe mitral stenosis. Thus, the applicability of the use of direct anticoagulants in patients with AF and mitral stenosis and also in those undergoing mitral bioprostheses surgery will be the subject of further studies. The findings may explain if specific atrial changes of mitral stenosis even after the valve replacement will influence thromboembolic events with direct anticoagulants.

Investigation of the Familial Risk of Rheumatic Heart Disease with Systematic Echocardiographic Screening: Data from the PROVAR+ Family Study.

We aimed to use echocardiographic (echo) screening to evaluate the risk of Rheumatic Heart Disease (RHD) among the relatives of patients with advanced RHD, who were enrolled in the University Hospital's outpatient clinics from February 2020 to September 2021. Consenting first-degree relatives were invited for echo screening using handheld devices (GE VSCAN) by non-physicians, with remote interpretation. Matched controls (spouses, neighbors) living in the same household were enrolled in a 1:5 fashion. A standard echo (GE Vivid-IQ) was scheduled if abnormalities were observed. In 16 months, 226 relatives and 47 controls of 121 patients were screened, including 129 children, 77 siblings and 20 parents. The mean age was 40 ± 17 years, 67% of the patients were women, and 239 (88%) lived with the index case for >10 years. Echo findings suggestive of RHD were confirmed in zero controls and 14 (7.5%) relatives (p = 0.05): 11 patients had mild/moderate mitral regurgitation, and four were associated with mitral stenosis and abnormal morphology. Two patients had mild aortic regurgitation and abnormal morphology, which were associated with mild aortic and mitral stenosis, and two patients with advanced RHD had bioprostheses in the mitral (2) and aortic (1) positions. In conclusion, first-degree relatives of individuals with clinical RHD are at greater risk of having RHD, on top of socioeconomic conditions.