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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.

Resumo Fundamento Pacientes com anemia falciforme (AF) têm risco aumentado de complicações cardiovasculares. O teste ergométrico é usado como marcador de prognóstico em uma série de doenças cardiovasculares. Entretanto, há uma escassez de evidências sobre exercícios em pacientes com AF, especialmente em relação à sua segurança, viabilidade e possível função prognóstica. Objetivos Usamos o teste em esteira máximo para determinar a segurança e a viabilidade do teste ergométrico em pacientes com AF. Além disso, os fatores associados à duração do exercício, bem como o impacto das alterações causadas pelo exercício em resultados clínicos, também foram avaliados. Métodos 113 pacientes com AF que passaram pelo teste ergométrico e por uma avaliação cardiovascular abrangente incluindo um ecocardiograma e os níveis do peptídeo natriurético do tipo B (BNP). O desfecho de longo prazo foi uma combinação de eventos incluindo morte, crises álgicas graves, síndrome torácica aguda ou internações hospitalares por outras complicações associadas â doença falciforme. A análise de regressão de Cox foi realizada para identificar as variáveis associadas ao resultado. Um p valor

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.

IntroductionMitral 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.MethodsConsecutive 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.ResultsThe 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).ConclusionsIn 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.

Mitral regurgitation (MR) is a major complication of the percutaneous mitral valvuloplasty (PMV). Despite high technical expertise and cumulative experience with the procedure, the incidence rate of severe MR has not decreased. Although some of MR can be anticipated by echocardiographic analysis; leaflet tearing, which leads to the most dreaded type of MR, remains unpredictable. Irregular valvular collagen remodeling is likely to compromise tissue architecture and increase the tearing risk during PMV balloon inflation. In this study, we evaluated histological and molecular characteristics of excised mitral valves from patients with rheumatic mitral stenosis (MS) who underwent emergency surgery after PMV due to severe MR caused by leaflet tear. Those findings were compared with patients who underwent elective mitral valve replacement surgery owing to severe MS, in whom PMV was not indicated. In vitro assay using peripheral blood mononuclear cells was performed to better understand the impact of the cellular and molecular alterations identified in leaflet tear mitral valve specimens. Our analysis showed that focal infiltration of inflammatory cells contributes to accumulation of MMP-1 and IFN-γ in valve leaflets. Moreover, we showed that IFN-γ increase the expression of MMP-1 in CD14+ cells (monocytes) in vitro. Thus, inflammatory cells contribute to unevenly remodel collagen resulting in variable thickening causing abnormalities in leaflet architecture making them more susceptible to laceration.

Background The natural history of latent rheumatic heart disease (RHD) detected by echocardiography remains unclear. We aimed to assess the accuracy of a simplified score based on the 2012 World Heart Federation criteria in predicting mid‐term RHD echocardiography outcomes in children from 4 different countries. Methods and Results Patient‐level baseline and follow‐up data of children with latent RHD from 4 countries (Australia, n=62; Brazil, n=197; Malawi, n=40; New Zealand, n=94) were combined. A simplified echocardiographic scoring system previously developed from Brazilian and Ugandan cohorts, consisting of 5 point‐based variables with respective weights, was applied: mitral valveanterior leaflet thickening (weight=3), excessive leaflet tip motion (3), regurgitation jet length ≥2 cm (6), aortic valve focal thickening (4), and any regurgitation (5). Unfavorable outcome was defined as worsening diagnostic category, persistent definite RHD or development/worsening of valve regurgitation/stenosis. The score model was updated using methods for recalibration. 393 patients (314 borderline, 79 definite RHD) with median follow‐up of 36 (interquartile range, 25–48) months were included. Median age was 14 (interquartile range, 11–16) years and secondary prophylaxis was prescribed to 16%. The echocardiographic score model applied to this external population showed significant association with unfavorable outcome (hazard ratio, 1.10; 95% CI, 1.04–1.16; P=0.001). Unfavorable outcome rates in low (≤5 points), intermediate (6–9), and high‐risk (≥10) children at 3‐year follow‐up were 14.3%, 20.8%, and 38.5% respectively (P

Mitral stenosis (MS) is a consequence of rheumatic heart disease that leads to heart failure requiring mechanical intervention. Percutaneous mitral commissurotomy (PMC) is the treatment of choice for the intervention, and currently there are no soluble markers associated with hemodynamic improvement after PMC. This study aims to determine the changes in cytokine/chemokine plasma levels, as well as T cell activation after PMC, and to investigate their association with immediate hemodynamic improvement and clinical outcomes. Plasma samples from eighteen patients with well-defined MS who underwent PMC and 12 healthy controls were analyzed using BioPlex immunoassay. We observed that 16 out of the 27 (60%) molecules assessed were altered in patients' plasma pre-PMC as compared to control group. Of those, IL-1β, IL-12, IL-6, IL-4, PDGF, and CCL11 showed significant decrease after PMC. Stratifying the patients according to adverse outcome after a 28-month median follow up, we detected a significant reduction of IL-1β, IL-12, IL-6, IL-4, IFN-γ, CXCL-10, VEGF, FGF and PDGF post-PMC in patients without events, but not in those who presented adverse events during the follow-up. Patients with adverse outcomes had lower IL-10 pre-PMC, as compared to the ones without adverse events. In addition, the frequency of CD8+ activated memory cells was increased after PMC, while the frequency of CD4+ activated memory cells did not change. Our results show an association between the decrease of specific cytokines and changes in T cell activation with hemodynamic improvement post-PMC, as well as with long-term outcomes, suggesting their possible use as soluble markers for hemodynamic recovery after MS intervention.

Rheumatic heart valve disease (RHVD) is a post-infectious sequel of acute rheumatic fever resulting from an abnormal immune response to a streptococcal pharyngitis that triggers valvular damage. RHVD is the leading cause of cardiovascular death in children and young adults, mainly in women from low and middle-income countries. It is known that long-term inflammation and high degree of fibrosis leads to valve dysfunction due to anatomic disruption of the valve apparatus. However, since public and private investments in RHVD studies are practically inexistent the number of publications is scarce. This disease shows different natural history and clinical presentations as compared to other degenerative heart valve diseases. Although more than five decades passed after the pioneering studies on the pathogenesis of RHVD, it is still unclear how self-tolerance mechanisms fail in this disease, and how humoral and cellular inflammatory responses are interconnected. Despite that pathological mechanisms have been already proposed for RHVD, none of them are able to explain the preferential involvement of the mitral valve. This review focuses on pathophysiology and underlying mechanisms of RHVD.

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.

Rheumatic Heart Disease Educational Strategies Introduction: The burden of rheumatic heart disease (RHD) is still high in Brazil. Lack of population awareness about the disease limits the efficacy of prevention programs. We aimed to evaluate the effectiveness of education on RHD in schools, comparing the conventional expository teaching method with tablet-based worked examples. Method: A prospective, cluster randomized trial was conducted over eight months in six randomly selected low-income Brazilian public schools. Each class was considered a cluster (total: 90), being randomized 1:1 to receive one of the educational methods. Pre-test evaluated students’ prior knowledge on RHD. Post-tests, 10 days, and three months later, evaluated retention of knowledge. Results: At total 1,301 students (52% female) completed the study, being 63% from high school. Baseline knowledge about RHD was universally low (average score expository classes [G1] 33.9% vs. worked examples [G2] 32.5%, p = 0.23). A significant but similar improvement was observed in both groups in the immediate post-test (pre- vs. post: p < 0.001): G1 57.5% vs. G2 56.7%, p = 0.69. In the late post-test, a significant 20% worsening was observed in both groups and the final scores were again similar: G1 45.0% vs. G2 45.9%, p = 0.87. Highschool students had higher scores (p < 0.001), and girls had better overall performances than boys (p < 0.001). Conclusion: The novel technology of tablet-based worked examples had similar results compared with expository classes for RHD education in schools. Both educational processes resulted in modest gains in knowledge, with low retention. More studies are needed to determine if increased knowledge leads to behavioral changes that could reduce RHD burden. Highlights: In a cluster-randomized trial, two different educational strategies about Rheumatic Heart Disease (RHD) for children – standard expository classes and worked examples based on interactive modules in tablet computers – were compared in public schools of underserved Brazilian neighborhoods. Baseline knowledge was low, and the novel tablet-based technology had similar results compared with traditional teaching for RHD education in schools, with no differences in acquisition and mid-term retention of knowledge. Both educational interventions resulted in similar 71% improvement in the immediate post-test, although with suboptimal retention, with over 20% worsening in three months in both groups. Our data suggests that the optimal strategy for RHD education is yet to be determined, and future studies should be warranted to determine if increased knowledge leads to behavioral changes that could reduce disease burden in endemic areas.

Background Risk stratification of Chagas disease patients in the limited‐resource setting would be helpful in crafting management strategies. We developed a score to predict 2‐year mortality in patients with Chagas cardiomyopathy from remote endemic areas. Methods and Results This study enrolled 1551 patients with Chagas cardiomyopathy from Minas Gerais State, Brazil, from the SaMi‐Trop cohort (The São Paulo‐Minas Gerais Tropical Medicine Research Center). Clinical evaluation, ECG, and NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) were performed. A Cox proportional hazards model was used to develop a prediction model based on the key predictors. The end point was all‐cause mortality. The patients were classified into 3 risk categories at baseline (low,

Background: Stroke is one of the leading causes of morbidity and mortality with a significant percentage classified as cryptogenic. Left atrial (LA) remodelling, a substrate for atrial fibrillation (AF) and stroke development, may play a role in identification of the aetiology of cryptogenic stroke. We aimed to examine LA function to gain mechanistic insights into the pathophysiology of cryptogenic stroke in young patients otherwise at low risk for cardiovascular disease. Methods: Patients aged

Introduction: In recent years, new technologies – noticeably ultra-portable echocardiographic machines – have emerged, allowing for Rheumatic Heart Disease (RHD) early diagnosis. We aimed to perform a cost-utility analysis to assess the cost-effectiveness of RHD screening with handheld devices in the Brazilian context. Methods: A Markov model was created to assess the cost-effectiveness of one-time screening for RHD in a hypothetical cohort of 11-year-old socioeconomically disadvantaged children, comparing the intervention to standard care using a public perspective and a 30-year time horizon. The model consisted of 13 states: No RHD, Undiagnosed Asymptomatic Borderline RHD, Diagnosed Asymptomatic Borderline RHD, Untreated Asymptomatic Definite RHD, Treated Asymptomatic Definite RHD, Untreated Mild Clinical RHD, Treated Mild Clinical RHD, Untreated Severe Clinical RHD, Treated Severe Clinical RHD, Surgery, Post-Surgery and Death. The initial distribution of the population over the different states was derived from primary echo screening data. Costs of the different states were derived from the Brazilian public health system database. Transition probabilities and utilities were derived from published studies. A discount rate of 3%/year was used. A cost-effectiveness threshold of $25,949.85 per Disability Adjusted Life Year (DALY) averted is used in concordance with the 3x GDP per capita threshold in 2015. Results: RHD echo screening is cost-effective with an Incremental Cost-Effectiveness Ratio of $10,148.38 per DALY averted. Probabilistic modelling shows that the intervention could be considered cost-effective in 70% of the iterations. Conclusion: Screening for RHD with hand held echocardiographic machines in 11-year-old children in the target population is cost-effective in the Brazilian context. Highlights: A cost-effectiveness analysis showed that Rheumatic Heart Disease (RHD) echocardiographic screening utilizing handheld devices, performed by non-physicians with remote interpretation by telemedicine is cost-effective in a 30-year time horizon in Brazil. The model included primary data from the first large-scale RHD screening program in Brazilian underserved populations and costs from the Unified Health System (SUS), and suggests that the Incremental Cost-Effectiveness Ratio of the intervention is considerably below the acceptable threshold for Brazil, even after a detailed sensitivity analysis. Considering the high prevalence of subclinical RHD in Brazil, and the significant economic burden posed by advanced disease, these data are important for the formulation of public policies and surveillance approaches. Cost-saving strategies first implemented in Brazil by the PROVAR study, such as taskshifting to non-physicians, computer-based training, routine use of affordable devices and telemedicine for remote diagnosis may help planning RHD control programs in endemic areas worldwide.

BackgroundConsidering the limited accuracy of clinical examination for early diagnosis of rheumatic heart disease (RHD), echocardiography has emerged as an important epidemiological tool. The ideal setting for screening is yet to be defined. We aimed to evaluate the prevalence and pattern of latent RHD in schoolchildren (aged 5–18 years) and to compare effectiveness of screening between public schools, private schools, and primary care centers in Minas Gerais, Brazil. Methods and ResultsThe PROVAR (Rheumatic Valve Disease Screening Program) study uses nonexperts and portable and handheld devices for RHD echocardiographic screening, with remote interpretation by telemedicine, according to the 2012 World Heart Federation criteria. Compliance with study consent and prevalence were compared between different screening settings, and variables associated with RHD were analyzed. In 26 months, 12 048 students were screened in 52 public schools (n=10 901), 2 private schools (n=589), and 3 primary care centers (n=558). Median age was 12.9 years, and 55.4% were girls. Overall RHD prevalence was 4.0% borderline (n=486) and 0.5% definite (n=63), with statistically similar rates between public schools (4.6%), private schools (3.5%), and primary care centers (4.8%) (P=0.24). The percentage of informed consents signed was higher in primary care centers (84.4%) and private schools (66.9%) compared with public schools (38.7%) (P

Background: Chagas Disease (CD) can cause Chagas cardiomyopathy. The new coronavirus disease (COVID-19) also affects the cardiovascular system and may worsen Chagas cardiomyopathy. However, the cardiac evolution of patients with CD infected by COVID-19 is not known. Thus, the objective of this study is to assess, within one year, whether there was cardiac progression after COVID-19 in CD. Methods: Longitudinal study with CD patients. The outcome was cardiac progression, defined as the appearance of new major changes in the current ECG compared to the previous ECG considered from the comparison of electrocardiograms (ECGs) performed with an interval of one year. Positive Anti-SARS-CoV2 Serology was the independent variable of interest. For each analysis, a final multiple model was constructed, adjusted for sociodemographic, clinical, and pandemic-related characteristics. Results: Of the 404 individuals included, 22.8 % had positive serology for COVID-19 and 10.9 % had cardiac progression. In the final model, positive serology for COVID-19 was the only factor associated with cardiac progression in the group as a whole (OR = 2.65; 95 % CI = 1.27–5.53) and for new-onset cardiomyopathy in the group with normal previous ECG (OR = 3.50; 95 % CI = 1.21–10.13). Conclusion: Our study shows an association between COVID-19 and progression of Chagas cardiomyopathy, evaluated by repeated ECGs, suggesting that COVID-19 accelerated the natural history of CD.

Aims: To assess whether contractile reserve during dobutamine stress echocardiography (DSE) can predict left ventricular functional recovery in patients with peripartum cardiomyopathy and to assess myocardial fibrosis by magnetic resonance imaging (MRI) in these patients. Methods: Nine patients with peripartum cardiomyopathy were enrolled. All patients underwent DSE and were followed for six months, when a rest Doppler echocardiogram was repeated. MRI was also performed at the beginning of follow-up to identify myocardial fibrosis. Results: Mean age was 29 ± 7.9 years and mean left ventricular ejection fraction at baseline was 39.4 ± 8.6% (range 24–49%). Eight of the nine patients showed left ventricular functional recovery with mean ejection fraction at follow-up of 57.1 ± 13.8%. The ejection fraction response to DSE did not predict recovery at follow-up. On the other hand, left ventricular ejection fraction at baseline correlated with ejection fraction at follow-up. Mild fibrosis was detected in only one patient. Conclusion: Left ventricular ejection fraction at baseline was a predictor of left ventricular functional recovery in patients with peripartum cardiomyopathy. Dobutamine stress echocardiography at presentation of the disease did not predict recovery at follow-up. Myocardial fibrosis appeared to be uncommon in this cardiomyopathy. Resumo: Objetivos: Avaliar se a reserva contrátil durante o ecocardiograma de estresse com dobutamina (EED) pode predizer a recuperação funcional do ventrículo esquerdo em pacientes com miocardiopatia periparto e também acessar a fibrose miocárdica através da ressonância nuclear magnética (RNM) nestas pacientes. Métodos: Nove pacientes com miocardiopatia periparto foram incluídas. Todas as pacientes foram submetidas ao EED e acompanhadas por 6 meses, quando um novo ecocardiograma de repouso foi realizado. A RNM também foi realizada no início do seguimento para identificar fibrose miocárdica. Resultados: A idade média das pacientes foi de 29 ± 7,9 anos e a fração de ejeção basal média do ventrículo esquerdo foi de 39,4 ± 8,6% (variando de 24 a 49%). Oito das nove pacientes tiveram recuperação funcional do ventrículo esquerdo, com fração de ejeção média no seguimento de 57,1 ± 13,8%. A resposta da fração de ejeção ao EED não foi um preditor de recuperação no seguimento. Por outro lado, a fração de ejeção basal teve correlação com a fração de ejeção no seguimento. Fibrose discreta foi detectada em apenas uma paciente. Conclusão: A fração de ejeção basal do ventrículo esquerdo foi um preditor de recuperação funcional ventricular em pacientes com miocardiopatia periparto. O EED na apresentação da doença não foi um preditor de recuperação no seguimento. Fibrose miocárdica pareceu ser incomum nesta miocardiopatia. Keywords: Dobutamine stress echocardiography, Peripartum cardiomyopathy, Myocardial fibrosis, Pregnancy, Palavras-chave: Ecocardiograma de estresse com dobutamina, Miocardiopatia periparto, Fibrose miocárdica, Gravidez

Resumo Fundamentos: Os mecanismos subjacentes pelos quais a doença cardíaca reumática (DCR) levam à disfunção valvar grave não são totalmente compreendidos. Objetivo: O presente estudo avaliou as alterações histopatológicas nas valvas mitrais (VM) buscando uma associação entre o padrão de disfunção valvar predominante e os achados histopatológicos. Métodos: Em 40 pacientes submetidos à troca da VM devido a DCR e em 20 controles submetidos a transplante cardíaco, foram analisados os aspectos histológicos da VM excisada. Dados clínicos e ecocardiográficos também foram coletados. As análises histológicas foram realizadas usando coloração com hematoxilina-eosina. Determinou-se inflamação, fibrose, neoangiogênese, calcificação e metaplasia adiposa. Valores de p

Resumo Fundamento: As ferramentas de telecardiologia são estratégias valiosas para melhorar a estratificação de risco. Objetivo: Objetivamos avaliar a acurácia da tele-eletrocardiografia (ECG) para predizer anormalidades no ecocardiograma de rastreamento na atenção primária. Métodos: Em 17 meses, 6 profissionais de saúde em 16 unidades de atenção primária foram treinados em protocolos simplificados de ecocardiografia portátil. Tele-ECGs foram registrados para diagnóstico final por um cardiologista. Pacientes consentidos com anormalidades maiores no ECG pelo código de Minnesota e uma amostra 1:5 de indivíduos normais foram submetidos a um questionário clínico e ecocardiograma de rastreamento interpretado remotamente. A doença cardíaca grave foi definida como doença valvular moderada/grave, disfunção/hipertrofia ventricular, derrame pericárdico ou anormalidade da motilidade. A associação entre alterações maiores do ECG e anormalidades ecocardiográficas foi avaliada por regressão logística da seguinte forma: 1) modelo não ajustado; 2) modelo 1 ajustado por idade/sexo; 3) modelo 2 mais fatores de risco (hipertensão/diabetes); 4) modelo 3 mais história de doença cardiovascular (Chagas/cardiopatia reumática/cardiopatia isquêmica/AVC/insuficiência cardíaca). Foram considerados significativos valores de p < 0,05. Resultados: No total, 1.411 pacientes realizaram ecocardiograma, sendo 1.149 (81%) com anormalidades maiores no ECG. A idade mediana foi de 67 anos (intervalo interquartil de 60 a 74) e 51,4% eram do sexo masculino. As anormalidades maiores no ECG se associaram a uma chance 2,4 vezes maior de doença cardíaca grave no ecocardiograma de rastreamento na análise bivariada (OR = 2,42 [IC 95% 1,76 a 3,39]) e permaneceram significativas (p < 0,001) após ajustes no modelo 2 (OR = 2,57 [IC 95% 1,84 a 3,65]), modelo 3 (OR = 2,52 [IC 95% 1,80 a 3,58]) e modelo 4 (OR = 2,23 [IC 95% 1,59 a 3,19]). Idade, sexo masculino, insuficiência cardíaca e doença cardíaca isquêmica também foram preditores independentes de doença cardíaca grave no ecocardiograma. Conclusões: As anormalidades do tele-ECG aumentaram a probabilidade de doença cardíaca grave no ecocardiograma de rastreamento, mesmo após ajustes para variáveis demográficas e clínicas.

Background American Indians face significant barriers to diagnosis and management of cardiovascular disease. We sought to develop a real‐world implementation model for improving access to echocardiography within the Indian Health Service, the American Indian Structural Heart Disease Partnership. Methods and Results The American Indian Structural Heart Disease Partnership was implemented and evaluated via a 4‐step process of characterizing the system where it would be instituted, building point‐of‐care echocardiography capacity, deploying active case finding for structural heart disease, and evaluating the approach from the perspective of the clinician and patient. Data were collected and analyzed using a parallel convergent mixed methods approach. Twelve health care providers successfully completed training in point‐of‐care echocardiography. While there was perceived usefulness of echocardiography, providers found it difficult to integrate screening point‐of‐care echocardiography into their workday given competing demands. By the end of 12 months, 6 providers continued to actively utilize point‐of‐care echocardiography. Patients who participated in the study felt it was an acceptable and effective approach. They also identified access to transportation as a notable challenge to accessing echocardiograms. Over the 12‐month period, a total of 639 patients were screened, of which 36 (5.6%) had a new clinically significant abnormal finding. Conclusions The American Indian Structural Heart Disease Partnership model exhibited several promising strategies to improve access to screening echocardiography for American Indian populations. However, competing priorities for Indian Health Service providers' time limited the amount of integration of screening echocardiography into outpatient practice. Future endeavors should explore community‐based solutions to develop a more sustainable model with greater impact on case detection, disease management, and improved outcomes.

Neurological complications are frequent during the active course of infective endocarditis (IE), and they are associated with high in-hospital mortality rates. However, limited data exist on the prognostic value of these complications for late outcomes. This study aimed to assess the long-term impact of neurological complications in patients surviving an IE episode. A total of 263 consecutive IE patients admitted to a tertiary care center between 2007 and 2022 were prospectively included. Neurological complications at admission included transient ischemic attack (TIA), ischemic stroke, hemorrhagic stroke, intracerebral abscess, and meningitis. The primary outcome was a composite of overall mortality or heart valve surgery. Of the patients, 34.2% died in the hospital, leaving 173 survivors for long-term follow-up. Over a median of 3.5 years, 29 patients died, and 13 (9%) underwent cardiac surgery, resulting in an overall adverse event rate of 30%. Neurological complications independently predicted long-term adverse outcomes (hazard ratio (HR) 2.237; 95% CI 1.006–4.976), after adjusting for age, chronic kidney disease (CKD), and heart failure (HF) development. In an IE patient cohort, neurological complications at admission, which is a complication directly related to the IE process, were independent predictors of long-term outcomes.

Objective A substantial proportion of patients with rheumatic heart disease (RHD) have tricuspid regurgitation (TR). This study aimed to identify the impact of functional TR on clinical outcomes and predictors of progression in a large population of patients with RHD.Methods A total of 645 patients with RHD were enrolled, mean age of 47±12 years, 85% female. Functional TR was graded as absent, mild, moderate or severe. TR progression was defined either as worsening of TR degree from baseline to the last follow-up echocardiogram or severe TR at baseline that required surgery or died. Incidence of TR progression was estimated accounting for competing risks.Results Functional TR was absent in 3.4%, mild in 83.7%, moderate in 8.5% and severe in 4.3%. Moderate and severe functional TR was associated with adverse outcome (HR 1.91 (95% CI 1.15 to 3.2) for moderate, and 2.30 (95% CI 1.28 to 4.13) for severe TR, after adjustment for other prognostic variables. Event-free survival rate at 3-year follow-up was 91%, 72% and 62% in patients with no or mild, moderate and severe TR, respectively. During mean follow-up of 4.1 years, TR progression occurred in 83 patients (13%) with an overall incidence of 3.7 events (95% CI 2.9 to 4.5) per 100 patient-years. In the Cox model, age (HR 1.71, 95% CI 1.34 to 2.17), New York Heart Association functional class III/IV (HR 2.57, 95% CI 1.54 to 4.30), right atrial area (HR 1.52, 95% CI 1.10 to 2.10) and right ventricular (RV) dysfunction (HR 2.02, 95% CI 1.07 to 3.84) were predictors of TR progression. By considering competing risk, the effect of RV dysfunction on TR progression risk was attenuated.Conclusions In patients with RHD, functional TR was frequent and associated with adverse outcomes. TR may progress over time, mainly related to right-sided cardiac chambers remodelling.

ABSTRACT Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment. Herein, we present our experience in the management of three children with severe pulmonary arterial hypertension refractory to clinical treatment who underwent Potts surgery in addition to clinical treatment.

Resumo Fundamento: A doença de Chagas leva à redução da capacidade funcional. Entretanto, o estágio em que o comprometimento funcional é detectável permanece obscuro. Objetivos: O presente estudo teve como objetivo comparar a capacidade funcional de pacientes em diferentes estágios da doença de Chagas e de indivíduos saudáveis e verificar os determinantes do consumo de oxigênio de pico (VO2pico). Métodos: Em um estudo transversal, foram selecionados 160 indivíduos, 35 saudáveis e 125 com doença de Chagas. No grupo chagásico, 61 (49%) estavam na forma indeterminada da doença, 45 (36%) com cardiomiopatia chagásica (CC) e função cardíaca preservada e 19 (15%) com disfunção cardíaca e CC dilatada. Os dados foram analisados por meio de análise de regressão univariada e multivariada. A significância estatística foi fixada em 5%. Resultados: Pacientes na forma indeterminada da doença apresentaram capacidade funcional semelhante a indivíduos saudáveis (p> 0,05). Pacientes com ChC e função cardíaca preservada apresentaram VO2pico menor que os pacientes na forma indeterminada (p

Background Screening programmes using echocardiography offer opportunity for intervention through identification and treatment of early (latent) rheumatic heart disease (RHD). We aimed to compare two methods for classifying progression or regression of latent RHD: serial review method and blinded, side-by-side review.Methods A four-member expert panel reviewed 799 enrolment (in 2018) and completion (in 2020) echocardiograms from the GOAL Trial of latent RHD in Uganda to make consensus determination of normal, borderline RHD or definite RHD. Serial interpretations (enrolment and completion echocardiograms read at two different time points, 2 years apart, not beside one another) were compared with blinded side-by-side comparisons (enrolment and completion echocardiograms displayed beside one another in random order on same screen) to determine outcomes according to prespecified definitions of disease progression (worsening), regression (improving) or no change. We calculated inter-rater agreement using Cohen’s kappa.Results There were 799 pairs of echocardiogram assessments included. A higher number, 54 vs 38 (6.8% vs 4.5%), were deemed as progression by serial interpretation compared with side-by-side comparison. There was good inter-rater agreement between the serial interpretation and side-by-side comparison methods (kappa 0.89). Disagreement was most often a result of the difference in classification between borderline RHD and mild definite RHD. Most discrepancies between interpretation methods (46 of 47, 98%) resulted from differences in valvular morphological evaluation, with valves judged to be morphologically similar between enrolment and final echocardiograms when compared side by side but classified differently on serial interpretation.Conclusions There was good agreement between the methods of serial and side-by-side interpretation of echocardiograms for change over time, using the World Heart Federation criteria. Side-by-side interpretation has higher specificity for change, with fewer differences in the interpretation of valvular morphology, as compared with serial interpretation.

ABSTRACT Despite substantial progress toward its control, Chagas disease continues to be a major public health problem in Latin America and has become a global health concern. The disease affects approximately 6 million people, of whom 20-40% will develop cardiomyopathy over the years after the initial Trypanosoma cruzi infection. Chagas cardiomyopathy is the most serious and frequent manifestation of Chagas disease. Clinical manifestations vary widely according to the severity of myocardial dysfunction, ranging from asymptomatic to severe forms, including dilated cardiomyopathy with heart failure, arrhythmias, thromboembolism events, and sudden death. Chagas disease is a risk factor for stroke regardless of the severity of cardiomyopathy, which is a leading cause of chronic disability. Classically, stroke etiology in patients with Chagas disease is thought to be cardioembolic and related to apical aneurysm, mural thrombus, and atrial arrhythmias. Although most strokes are thromboembolic, other etiologies have been observed. Small vessel disease, atherosclerosis, and cryptogenic diseases have been reported in patients with Chagas disease and stroke. The potential mechanisms involved in non-embolic strokes include the presence of associated risk factors, pro-inflammatory and prothrombotic disease states, and endothelial dysfunction. However, the contribution of each mechanism to stroke in Chagas disease remains unclear. The review aims to provide an overview of stroke in Chagas disease, highlighting the main pathophysiological mechanisms, clinical presentation, approaches for prevention, and unanswered questions regarding treatment strategies.

Resumo Fundamento A ecocardiografia tridimensional (ECO 3D) permite a geração de uma curva volume-tempo representativa das alterações no volume ventricular esquerdo (VE) ao longo de todo o ciclo cardíaco. Objetivo O presente estudo tem como objetivo demonstrar as adaptações hemodinâmicas presentes na cardiomiopatia chagásica (CC) por meio das medidas de volume e fluxo obtidas pela curva volume-tempo por ECO 3D. Métodos Vinte pacientes com CC e 15 indivíduos saudáveis foram incluídos prospectivamente em um estudo de desenho transversal. Realizou-se ECO 3D em todos os indivíduos e as curvas volume-tempo do VE foram geradas. O fluxo foi obtido pela primeira derivada da curva volume-tempo por meio do software MATLAB. A significância estatística foi definida com p

Abstract INTRODUCTION: Cardiac involvement seems to impact prognosis of COVID-19, being more frequent in critically ill patients. We aimed to assess the prognostic value of right ventricular (RV) and left ventricular (LV) dysfunction, evaluated by bedside echocardiography (echo), in patients hospitalized with COVID-19. METHODS: Patients admitted in 2 reference hospitals in Brazil from Jul to Sept/2020 with confirmed COVID-19 and moderate/severe presentations underwent clinical and laboratory evaluation, and focused bedside echo (GE Vivid-IQ), at the earliest convenience, with remote interpretation. The association between demographics, clinical comorbidities and echo variables with all-cause hospital mortality was assessed, and factors significant at p

Resumo Introdução No Brasil, a única droga disponível para o tratamento específico do Trypanosoma cruzi, causador da Doença de Chagas (DC) é o Benzonidazol (BZN), cujas reações adversas podem propiciar a interrupção do tratamento. Objetivo O objetivo desse trabalho é quantificar a proporção de ocorrência de efeitos adversos, descrevê-los e identificar os fatores de risco associados à essa ocorrência. Método Revisão sistemática conduzida de acordo com os padrões estabelecidos pelo Preferred Reporting Items is Systematic Reviews and Metanalyses (PRISMA). Incluíram-se somente ensaios clínicos randomizados controlados. Resultados Dos 134 estudos identificados, cinco foram incluídos. O tamanho da amostra variou de 77 a 2854 pessoas, e a faixa etária dos participantes, variou de zero a 75 anos. Todos os estudos incluídos apontaram reações adversas ao uso do BZN. A frequência de reação adversa chegou a 38% e a mais comum foi o rash cutâneo. O abandono do tratamento devido à reação foi citado em três estudos, variando de 6,4% a 13,4%. Conclusão As reações adversas ao uso do BZN variaram de acordo com a idade do paciente e esquema terapêutico de dose e tempo adotado, sendo o conhecimento médico acerca dessas reações imprescindível para seu uso seguro e adesão ao tratamento.

Background Chagas disease remains a major cause of cardiovascular death in endemic areas. Focused echocardiography (FoCUS) is a point-of-care means of assessing cardiac function which can be useful for the diagnosis of cardiac involvement. Objective This study aims evaluating the characteristics of validity and reliability of FoCUS applied on Chagas disease patients. Methods Patients with Chagas disease coming from an endemic area were selected from a large cohort (SaMi-Trop). A simplified echocardiogram with only three images was extracted from the conventional echocardiogram performed in this cohort. The images were evaluated by an observer who was blinded to the clinical and echocardiographic data, to determine the accuracy and reliability of FoCUS for cardiac assessment. The analysis constituted of 5 prespecified variables, dichotomized in absence or presence: left ventricular (LV) size and systolic function, right ventricular (RV) size and systolic function, and LV aneurysm. Results We included 725 patients with a mean age of 63.4 ± 12.3 years, 483 (67%) female. Abnormal electrocardiogram was observed in 81.5% of the patients. Left and right ventricular dysfunctions were found in 103 (14%) and 49 (7%) of the patients, respectively. Sensitivity, specificity, positive predictive value and negative predictive value were 84%, 94%, 70% and 97% for LV enlargement and 81%, 93%, 68% and 97% for LV systolic dysfunction, respectively, and 46%, 99%, 60% and 98% for RV dilatation, and 37%, 100%, 100% and 96% for RV dysfunction, respectively. Inter and intraobserver agreement were 61% and 87% for LV enlargement and 63% and 92% for LV dysfunction, respectively, and 50% and 49% for RV size and 46% and 79% for RV dysfunction, respectively. LV apical aneurysm was found in 45 patients (6.2%) with the lowest sensitivity of FoCUS study (11%; 95% CI 2–28%). Conclusions FoCUS showed satisfactory values of validity and reliability for assessment of cardiac chambers in patients with Chagas disease, except for apical aneurysm. This tool can identify heart disease with potential impact on patient management in the limited-resource setting.

Nearly a century after rheumatic fever (RF) and rheumatic heart disease (RHD) was eradicated from the developed world, the disease remains endemic in many low- and middle-income countries (LMICs), with grim health and socioeconomic impacts. The neglect of RHD which persisted for a semi-centennial was further driven by competing infectious diseases, particularly the human immunodeficiency virus (HIV) pandemic. However, over the last two-decades, slowly at first but with building momentum, there has been a resurgence of interest in RF/RHD. In this narrative review, we present the advances that have been made in the RF/RHD continuum over the past two decades since the re-awakening of interest, with a more concise focus on the last decade’s achievements. Such primary advances include understanding the genetic predisposition to RHD, group A Streptococcus (GAS) vaccine development, and improved diagnostic strategies for GAS pharyngitis. Echocardiographic screening for RHD has been a major advance which has unearthed the prevailing high burden of RHD and the recent demonstration of benefit of secondary antibiotic prophylaxis on halting progression of latent RHD is a major step forward. Multiple befitting advances in tertiary management of RHD have also been realized. Finally, we summarize the research gaps and provide illumination on profitable future directions towards global eradication of RHD.

Abstract Patients with Chagas cardiomyopathy (ChC) usually progress with fatigue and dyspnea. Exercise tests are valuable for the functional evaluation of these patients. However, information about the applicability of the exercise tests is scattered, and no studies have systematically reviewed the results. Thus, the present review explored the general aspects and prognostic value of exercise tests in patients with ChC. A literature search of the MEDLINE, Web of Science, CINAHL, Scopus, and LILACS databases was performed to identify relevant studies. There were no data restrictions, and articles that met the objective of the study were selected. Articles written in English, Portuguese, and Spanish were considered, and 25 articles were finally included. The peak oxygen uptake (VO2peak) was correlated with demographic and echocardiographic variables. Echocardiographic features of the left ventricular diastolic function and right ventricular systolic function appeared to be determinants of functional capacity, in addition to age and sex. VO2peak was associated with higher mortality, especially in patients with dilated ChC. The minute ventilation/carbon dioxide production slope (VE/VCO2 slope) was a strong predictor of survival; however, more studies are needed to verify this observation. Field tests showed moderate to strong correlation with VO2peak and thus may be inexpensive tools for the functional evaluation of patients with ChC. However, few studies have verified their prognostic significance. While exercise tests are useful tools for functional assessment, information is scarce regarding further considerations, and many of the criteria are based on guidelines for other heart diseases.

Objectives Echocardiographic (echo) screening is an important tool to estimate rheumatic heart disease (RHD) prevalence, but the natural history of screen-detected RHD remains unclear. The PROVAR+ (Programa de RastreamentO da VAlvopatia Reumática) study, which uses non-experts, telemedicine and portable echo, pioneered RHD screening in Brazil. We aimed to assess the mid-term evolution of Brazilian schoolchildren (5–18 years) with echocardiography-detected subclinical RHD and to assess the performance of a simplified score consisting of five components of the World Heart Federation criteria, as a predictor of unfavourable echo outcomes.Setting Public schools of underserved areas and private schools in Minas Gerais, southeast Brazil.Participants A total of 197 patients (170 borderline and 27 definite RHD) with follow-up of 29±9 months were included. Median age was 14 (12–16) years, and 130 (66%) were woman. Only four patients in the definite group were regularly receiving penicillin.Primary and secondary outcome measures Unfavourable outcome was based on the 2-year follow-up echo, defined as worsening diagnostic category, remaining with mild definite RHD or development/worsening of valve regurgitation/stenosis.Results Among patients with borderline RHD, 29 (17.1%) progressed to definite, 49 (28.8%) remained stable, 86 (50.6%) regressed to normal and 6 (3.5%) were reclassified as other heart diseases. Among those with definite RHD, 13 (48.1%) remained in the category, while 5 (18.5%) regressed to borderline, 5 (18.5%) regressed to normal and 4 (14.8%) were reclassified as other heart diseases. The simplified echo score was a significant predictor of RHD unfavourable outcome (HR 1.197, 95% CI 1.098 to 1.305, p