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As the burden of rheumatic heart disease (RHD) increases in Ethiopia, there is a growing need for low-cost interventions to mitigate its impact. This study aimed to assess the effectiveness of chronic disease self-management education (CDSME) on clinical outcomes, depression, and anxiety among patients with RHD. A quasi-experimental study was deployed among 166 patients with RHD at Jimma Medical Center between April and July 2024. A multi-component CDSME was endured for four consecutive months. Data were analyzed using SPSS version 25.0. Systolic blood pressure decreased from 131.31 mmHg (SD±15.68) at pre-education to 113.77 mmHg (SD±20.98) at post-education (t- 12.251, p<0.001). Diastolic blood pressure decreased from 94.87 mmHg (SD±19.41) pre-education to 79.28 mmHg (SD±9.33) post-education (t-12.306, p<0.001). Hemoglobin level changed from 11.97 g/dl (SD±1.99) to 13.84 g/dl (SD±1.37) after the intervention (t -11.96, p < 0.001). Similarly, hospital depression subscale decreased from 11.93 (SD±3.43) to 9.48 (SD±3.67) (t - 8.37, p < 0.001) post intervention. Regarding the hospital anxiety subscale, the mean score before education program was 8.59 (SD±2.91) and decreased to 7.30 (SD±1.97) (t -6.44, p < 0.001) after education. This intervention is simple, cost-effective, and has the potential to be scaled up and implemented in the current healthcare system in Ethiopia., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interest of personal relationships that could have appeared to influence the work reported to influence the work reported in this paper, (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose of Review: To summarize the latest developments in rheumatic fever and rheumatic heart disease (RHD) prevention, (early) diagnosis, and treatment., Recent Findings: The revised Jones criteria have demonstrated increased sensitivity and specificity for rheumatic fever diagnosis in high-risk populations. The management of rheumatic fever remains symptom-based, with no treatment options proven to alter the disease course or prevent chronic RHD. The revised World Heart Federation (WHF) guidelines for the echocardiographic diagnosis of RHD encourage task-shifting of RHD screening, extending reach in endemic regions. These guidelines also present an A-D classification of RHD and provide recommendations for the management of early disease. Integrated models for RHD screening within existing health structures are emerging and automated intelligence is showing potential to support RHD screening and diagnosis. Innovative strategies to foster adherence and equitable access to secondary prophylaxis, such as re-examination of the efficacy of oral penicillin, trials of longer acting penicillin formulations and implants are underway. There is renewed interest and investment in a well tolerated and effective GAS vaccine., Summary: We are living in a time of possibility with global acceleration to address the prevailing burden of RHD. Together, we can ensure that RHD does not once again fall off the global health agenda, until equitable elimination has been achieved., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: This review will synthesize studies on costs, the impact of these costs, and the cost-effectiveness of treatments for rheumatic heart disease (RHD) in low- and middle-income countries., Introduction: RHD incurs high costs owing to its clinical complexity, surgical treatments, and prolonged hospital stays. Thus, the disease has a substantial economic impact on the health system, patients, and their families. No systematic review on economic evidence of treatments for RHD has been published to date., Inclusion Criteria: This review will consider all cost and cost-effectiveness studies on RHD treatments for children and young adults (5─30 years) residing in low- and middle-income countries., Methods: The review will follow the JBI methodology for systematic reviews of economic evaluation evidence. The search strategy will locate published and unpublished studies in English. Systematic searches will be conducted in MEDLINE (PubMed), MEDLINE (Ovid), Embase (Ovid), Scopus, CINAHL (EBSCOhost), National Health Service Economic Evaluation Databases, Pediatric Economic Database Evaluation, and Cost-Effectiveness Analysis Registry. Two independent reviewers will screen titles and abstracts, followed by a full-text review based on the inclusion criteria. Data will be extracted using a modified JBI data extraction form for economic evaluations. JBI's Dominance Ranking Matrix for economic evaluations will be used to summarize and compare the results of cost and cost-effectiveness studies. The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach will be used to assess the certainty of economic evidence for outcomes related to resource use., Review Registration: PROSPERO CRD42023425850., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 JBI.)

Background: It is uncertain which percutaneous balloon mitral valvuloplasty (PBMV) success definitions should be used because there are no studies comparing the effects of these definitions on subsequent outcomes. We evaluated the association between 3 success definitions and long-term clinical outcomes in patients with rheumatic mitral stenosis who underwent PBMV., Methods and Results: This multicenter retrospective study included patients with severe rheumatic mitral stenosis who underwent PBMV. Three definitions were used as follows: (A) post-PBMV mitral valve area (MVA) ≥1.5 cm 2 or ≥50% increase in MVA with MR <3+; (B) post-PBMV MVA ≥1.5 cm 2 and MR ≤2+; and (C) post-PBMV MVA ≥1.5 cm 2 or ≥50% increase in MVA, with no more than 1-grade increment in MR. Multivariable Cox regression analyses were performed to evaluate the associations between PBMV success and the composite of all-cause mortality, mitral surgery, and repeat PBMV. Successful PBMV, according to definitions A, B, and C was associated with a lower risk of the composite outcomes (definition A-hazard ratio [HR], 0.55 [95% CI, 0.43-0.69], definition B-HR, 0.55 [95% CI, 0.43-0.69], definition C-HR, 0.55 [95% CI, 0.44-0.69]). Patients meeting all 3 success definitions had the lowest risk compared with those who did not meet any definition or met 1 or 2 definitions., Conclusions: All 3 success definitions had a prognostic impact on outcomes. Patients who achieved post-PBMV MVA ≥1.5 cm 2 , irrespective of the percentage increase in MVA, and MR ≤grade 2, with no more than a 1-grade increment in MR, had the most favorable outcomes.

Introduction: Rheumatic heart disease with persistent atrial fibrillation (RHD-AF) is associated with increased morbidity. However, there is no standardized approach for the maintenance of sinus rhythm (SR) in them. We aimed to determine the utility of a stepwise approach to achieve SR in RHD-AF., Methods: Consecutive patients with RHD-AF from July 2021 to August 2023 formed the study cohort. The stepwise approach included pharmacological rhythm control and/or electrical cardioversion (Central illustration). In patients with recurrence, additional options included AF ablation or pace and ablate strategy with conduction system pacing or biventricular pacing. Clinical improvement, NT-proBNP, 6-Minute Walk Test (6MWT), heart failure (HF) hospitalizations, and thromboembolic complications were documented during follow-up., Results: Eighty-three patients with RHD-AF (mean age 56.13 ± 9.51 years, women 72.28%) were included. Utilizing this approach, 43 (51.81%) achieved and maintained SR during the study period of 11.04 ± 7.14 months. These patients had improved functional class, lower NT-proBNP, better distance covered for 6MWT, and reduced HF hospitalizations. The duration of AF was shorter in patients who achieved SR, compared to those who remained in AF (3.15 ± 1.29 vs 6.93 ± 5.23, p = 0.041). Thirty-five percent (29) maintained SR after a single cardioversion over the study period. Only one underwent AF ablation. Of the 24 who underwent pace and ablate strategy, atrial lead was implanted in 22 (hybrid approach), and 50% of these achieved and maintained SR. Among these 24, none had HF hospitalizations, but patients who maintained SR had further improvement in clinical and functional parameters., Conclusions: RHD-AF patients who could achieve SR with a stepwise approach, had better clinical outcomes and lower HF hospitalizations., (© 2024 Wiley Periodicals LLC.)

Background: Rheumatic mitral stenosis (MS) remains a common and concerning health problem in Asia. Percutaneous balloon mitral valvuloplasty (PBMV) is the standard treatment for patients with symptomatic severe MS and favorable valve morphology. However, studies on the incidence and predictors of adverse cardiac outcomes following PBMV in Asia have been limited. This study aims to evaluate the incidence and predictors of adverse outcomes in patients with rheumatic MS following PBMV., Methods: A retrospective cohort study was conducted on patients with symptomatic severe MS who underwent successful PBMV between 2002 and 2020 at a tertiary academic institute in Thailand. Patients were followed up to assess adverse outcomes, defined as a composite of cardiac death, heart failure hospitalization, repeat PBMV, or mitral valve surgery. Univariable and multivariable analyses were performed to identify predictors of adverse outcomes. A p-value of < 0.05 was considered statistically significant., Results: A total of 379 patients were included in the study (mean age 43 ± 11 years, 80% female). During a median follow-up of 5.9 years (IQR 1.7-11.7), 74 patients (19.5%) experienced adverse outcomes, with an annualized event rate of 2.7%. Multivariable analysis showed that age (hazard ratio [HR] 1.03, 95% confidence interval [CI] 1.008-1.05, p = 0.006), significant tricuspid regurgitation (HR 2.17, 95% CI 1.33-3.56, p = 0.002), immediate post-PBMV mitral valve area (HR 0.39, 95% CI 0.25-0.64, p = 0.01), and immediate post-PBMV mitral regurgitation (HR 1.91, 95% CI 1.18-3.07, p = 0.008) were independent predictors of adverse outcomes., Conclusions: In patients with symptomatic severe rheumatic MS, the incidence of adverse outcomes following PBMV was 2.7% per year. Age, significant tricuspid regurgitation, immediate post-PBMV mitral valve area, and immediate post-PBMV mitral regurgitation were identified as independent predictors of these adverse outcomes., (© 2024. The Author(s).)

Background: Percutaneous balloon mitral valvuloplasty (PBMV) is the ACC/AHA class I recommendation for treating symptomatic rheumatic mitral stenosis with suitable valve morphology, less than moderate MR and absence of left atrium clot. The mitral valve restenosis and significant mitral regurgitation (MR) are known adverse outcomes of PBMV. This study aimed to evaluate the outcomes of PBMV in patients with severe mitral stenosis and the effect of Commissural Calcification (CC) on the outcomes., Methods: In this single-center retrospective cohort study, 876 patients who underwent PBMV were categorized into three groups based on their Wilkins score (Group I: score ≤ 8, Group II: score 9-10, and Group III: score 11-12). Patients were evaluated before, early after PBMV and at 6- and 24-month follow-ups. Main clinical outcomes were defined as significant restenosis and or symptomatic significant MR (moderate to severe and severe MR) or candidate for mitral valve replacement (MVR). The outcomes were compared between patients with and without CC., Results: A total of 876 patients with mean age 46.4 ± 12.3 years (81.0% females) were categorized based on Wilkins score. 333 (38.0%) were in Group I, 501 (57.2%) were in Group II, and 42 (4.8%) were in Group III. CC was present in 175 (20.0%) of the patients, among whom 95 (54.3%) had calcification of the anterolateral commissure, 64 (36.6%) had calcification of the posteromedial commissure, and in 16 (9.1%) patients both commissures were calcified. There was a significant difference in Wilkins score between patients with and without CC (P < 0.001). CC was associated with higher odds of significant symptomatic MR at early and mid-term follow up (OR: 1.69, 95%CI 1.19-2.41, P = 0.003; and OR: 3.90, 95%CI 2.61-5.83, P < 0.001, respectively), but not with restenosis (P = 0.128). Wilkins Groups II and III did not show higher odds of significant symptomatic MR compared to Group I at early (II: P = 0.784; III: P = 0.098) and mid-term follow up (II: P = 0.216; III: P = 0.227). Patients in Wilkins Group II had higher odds of restenosis compared to Group I (OR: 2.96,95%CI: 1.35-6.27, P = 0.007)., Conclusion: Commissural calcification (CC) is an independent predictor of the significant symptomatic MR (an important determinant of adverse outcome) following PBMV in the early and mid-term follow-up. Mitral valve restenosis occurs more in patients with higher Wilkins score compared to group I with score ≤ 8. Combined Wilkins score and CC should be considered for patient suitability for PBMV., (© 2024. The Author(s).)

The transcatheter edge-to-edge mitral valve repair (TEER) has been recommended as a reliable treatment option for selected patients with severe degenerative and functional mitral regurgitation (MR). Although MR patients with rheumatic etiology were excluded from two significant trials (EVEREST II and COAPT) that established a role for the TEER in degenerative and functional MR. However, it has been reported that the TEER procedure could be safely and effectively performed in carefully selected rheumatic MR patients. Therefore, we share a case report of successfully treating severe rheumatic MR using a novel-designed TEER system (JensClip TM )., (© 2024 Wiley Periodicals LLC.)

Abstract: This review summarizes the evaluation for underlying rheumatic conditions in patients presenting with acute pericarditis, treatment considerations for specific rheumatic conditions, and the role of imaging in diagnosis and monitoring. Pericarditis may be one of the initial presentations of a rheumatic disease or identified in a patient with known rheumatic disease. There is also growing evidence for using anti-inflammatory and immunosuppressive agents for treating recurrent pericarditis, which can overlap with the treatment of rheumatic diseases., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Rheumatic heart disease remains the most common cardiovascular disease in children and young adults. The outcome of interventional versus medical therapy on the long term is not fully elucidated yet. This study provides contemporary data on the clinical profile, treatment and follow up of patients with rheumatic mitral stenosis (MS) in Tanzania., Methods: Patients' medical information, investigations and treatment data were recorded in this prospective cohort study. They were followed up for 6-24 months to determine the long-term outcome. Interventional therapy was defined as a combination of surgery and percutaneous balloon mitral valvuloplasty. Kaplan-Meier curves and Cox proportional hazards model were used in analyses. p-Value < 0.05 was considered statistically significant., Results: We enrolled 290 consecutive patients. Interventions were done in half of the patients. Median follow up was 23.5 months. Mortality was higher in the medical than interventional treatment (10.4% vs. 4%, log-rank p = 0.001). Median age was 36 years, females (68.3%) and low income (55.5%). Multivalvular disease was found in 116 (40%) patients, atrial fibrillation (31.4%), stroke/transient ischaemic attack (18.9%) and heart failure class III-IV (44.1%). Median (IQR) duration of disease was 3 (4) years, secondary prophylaxis (27.7%) and oral anticoagulants use (62.3%). In multivariable analysis, the risk of death among patients on medical was 3.07 times higher than those on interventional treatment (crude HR 3.07, 95% CI 1.43-6.56, p = 0.004), 2.44 times higher among patients with arrhythmias versus without arrhythmias (crude HR 2.44, 95% CI 1.19-4.49, p = 0.015) and 2.13 times higher among patients with multivalvular than single valve disease (crude HR 2.13, 95% CI 1.09-4.16, p = 0.026)., Conclusions: Intervention is carrying low mortality compared to medical treatment. Arrhythmias and multivalvular disease are associated with a high mortality. Rheumatic MS is more prevalent in young people, females and individuals with low income. There is a late hospital presentation and a low use of both secondary prophylactic antibiotics and anticoagulants., (© 2023 The Authors. European Journal of Clinical Investigation published by John Wiley & Sons Ltd on behalf of Stichting European Society for Clinical Investigation Journal Foundation.)

Background: For rheumatic mitral stenosis (MS), a multidisciplinary evaluation is mandatory to determine the optimal treatment: medical, percutaneous balloon mitral valvuloplasty (PBMV) or valve surgery. Clinical and imaging evaluations are essential for procedural risk assessment and outcomes. PBMV interventions are increasingly available in Africa and are feasible options for selected candidates. Enhancing PBMV training/skills transfer across most of African countries is possible., Objectives: The aim of this study was to provide insight into the clinical practice of patients with rheumatic MS evaluated for PBMV in a Tanzanian teaching hospital and to define the role of imaging, and evaluate the heart team and training/skills transfer in PBMV interventions., Methods: From August 2019 to May 2022, 290 patients with rheumatic MS were recruited consecutively in the Tanzania Mitral Stenosis study. In total, 43 (14.8%) patients were initially evaluated for eligibility for PBMV by a heart team. We carried out the clinical assessment, laboratory investigations, transthoracic/oesophageal echocardiography (TTE/TEE) and electrocardiography., Results: The median age was 31 years (range 11-68), and two-thirds of the patients were female (four diagnosed during pregnancy). Two patients had symptomatic MS at six and eight years. Nine patients had atrial fibrillation with left atrial thrombus in three, and two were detected by TEE. Nine patients in normal sinus rhythm had spontaneous echo contrast. The mean Wilkins score was 8.6 (range 8-12). With re-evaluation by the local and visiting team, 17 patients were found to have unfavourable characteristics: Bi-commissural calcification (four), ≥ grade 2/4 mitral regurgitation (six), high scores and left atrial thrombus (three), left atrial thrombus (two), and severe pulmonary hypertension (two). Three patients died before the planned PBMV. Eleven patients were on a waiting list. We performed PBMV in 12 patients, with success in 10 of these, and good short-term outcomes [mean pre-PBMV (16.03 ± 5.52 mmHg) and post-PBMV gradients (3.08 ± 0.44 mmHg, p < 0.001)]. There were no complications., Conclusions: PBMV had good outcomes for selected candidates. TEE is mandatory in pre-PBMV screening and for procedural guidance. In our cohort, patients with Wilkins score of up to 11 underwent successful PBMV. We encourage PBMV skills expansion in low- and middle-income countries, concentrating on expertise centres.

Echocardiography is an important diagnostic imaging modality in recognizing rheumatic heart disease, a chronic sequelae of acute rheumatic fever. Left-sided heart valves, especially the mitral valve is typically affected, with stenosis or regurgitation as a consequence. Although assessment of valve area by 2D planimetry is the reference method for mitral stenosis severity, 3D planimetry provides more accurate measurement and diagnostic value. Careful selection of patients in terms of echocardiographic criteria is essential to ensure safety and success of the intervention and better long-term outcomes. Several echocardiographic scores based upon mitral valve mobility, thickening, calcification, and subvalvular thickening are developed to assess mitral valve anatomy and the feasibility of percutaneous mitral commissurotomy. 3D transesophageal echocardiography (TEE) provides detailed information of the mitral anatomy (commissural fusions, and subvalvular apparatus) before intervention. In addition, 3D TEE planimetry provides a more accurate measurement of the valve area compared with 2D echocardiography. Generally, huge annular calcification and lack of commissural fusion are unfavorable echocardiographic markers that increase the risk of complications and preclude the feasibility of percutaneous balloon mitral valvuloplasty. More contemporary prospective echocardiography research studies on patients with RHD from low- and middle-income countries are needed., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Aim: To co-design a rheumatic fever service model which enables young people with acute rheumatic fever/rheumatic heart disease (ARF/RHD) and their families to access the health and wellbeing services they need., Method: Co-design, a collaborative and participatory approach, was used to gather experiences and ideas from 21 consumers and 30 health professionals. Thematic analysis was undertaken., Results: Māori and Pacific patients and their whānau/aiga identified the importance of whānau/aiga support and involvement throughout their ARF/RHD journey. They described that the way care was delivered was often frustrating, fragmented and lacked effective communication. Participants expressed the need for information to improve their understanding of ARF/RHD. Health professionals identified the need for better continuity of care and felt that they were currently working siloed from other professionals with little visibility of other roles or opportunity for collaboration. The ideas for improvement were grouped into themes and resulted in development and prototyping of peer support groups, patient and staff education resources, clinical dashboard and pathway development, and an enhanced model of care for delivery to patients receiving penicillin prophylaxis., Conclusion: The co-design process enabled consumers and staff of ARF/RHD services to share experiences, identify ideas for improvement, co-design prototypes and test initiatives to better support the needs of those delivering and receiving ARF/RHD services., Competing Interests: Nil., (© PMA.)

Competing Interests: Disclosures None.

Objective: Most rheumatic heart disease (RHD) registries are static and centralized, collecting epidemiological and clinical data without providing tools to improve care. We developed a dynamic cloud-based RHD case management application with the goal of improving care for patients with RHD in Uganda., Methods: The Active Community Case Management Tool (ACT) was designed to improve community-based case management for chronic disease, with RHD as the first test case. Global and local partner consultation informed selection of critical data fields and prioritization of application functionality. Multiple stages of review and revision culminated in user testing of the application at the Uganda Heart Institute., Results: Global and local partners provided feedback of the application via survey and interview. The application was well received, and top considerations included avenues to import existing patient data, considering a minimum data entry form, and performing a situation assessment to tailor ACT to the health system setup for each new country. Test users completed a postuse survey. Responses were favorable regarding ease of use, desire to use the application in regular practice, and ability of the application to improve RHD care in Uganda. Concerns included appropriate technical skills and supports and potential disruption of workflow., Conclusion: Creating the ACT application was a dynamic process, incorporating iterative feedback from local and global partners. Results of the user testing will help refine and optimize the application. The ACT application showed potential for utility and integration into existing care models in Uganda., Competing Interests: None declared., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Background: Rheumatic Heart Disease (RHD) causes high morbidity and mortality rates among children and young adults, impacting negatively on their health-related quality of life (HRQoL). This study aimed to evaluate the HRQoL and healthcare consultations of adult patients with RHD in Namibia., Methods: From June 2019 to March 2020, a questionnaire was administered to 83 RHD patients during routine follow-ups. The EQ-5D-5L instrument was used to assess the health-related quality of life before diagnosis and at the time of the survey. The Ethiopian value set for EQ-5D-5L was used to calculate Quality-Adjusted Life Years (QALY)., Results: Most respondents were women (77%), young adults below the age of 30 years (42%), and individuals who grew up in rural areas (87%). The mean QALY statistically significantly improved from 0.773 pre-diagnosis to 0.942 in the last 12 months (p < 0.001). Sixty-six patients who had surgery reported a better QALY. Healthcare visits statistically significantly increased from on average 1.6 pre-diagnosis to 2.7 days in the last 12 months (p < 0.001). The mean distance to the nearest facility was 55 km, mean cost of transport was N$65, and mean time spent at the clinic was 3.6 h. The median time from diagnosis to the survey was 7 years (quartiles 4 and 14 years)., Conclusion: Treatment and surgery can improve HRQoL substantially among RHD patients. Being diagnosed with RHD affects patients living in socioeconomically disadvantaged rural areas through cost and time for healthcare visits. It would be valuable with further research to understand differences between disease severities., (© 2023. BioMed Central Ltd., part of Springer Nature.)

Background Population growth, aging, and major alterations in epidemiologic trends inadvertently modulate the status of rheumatic heart disease (RHD) epidemiology. This investigation predicted RHD burden pattern and temporal trends to provide epidemiologic evidence. Methods and Results Prevalence, mortality, and disability-adjusted life-years data for RHD were obtained from the GBD (Global Burden of Disease) study. We performed decomposition analysis and frontier analysis to assess variations and burden in RHD from 1990 to 2019. In 2019, there were >40.50 million RHD cases worldwide, along with nearly 0.31 million RHD-related deaths and 10.67 million years of healthy life lost to RHD. The RHD burden was commonly concentrated within lower sociodemographic index regions and countries. RHD primarily affects women (22.52 million cases in 2019), and the largest age-specific prevalence rate was at 25 to 29 years in women and 20 to 24 years in men. Multiple reports demonstrated prominent downregulation of RHD-related mortality and disability-adjusted life-years at the global, regional, and national levels. Decomposition analysis revealed that the observed improvements in RHD burden were primarily due to epidemiological alteration; however, it was negatively affected by population growth and aging. Frontier analysis revealed that the age-standardized prevalence rates were negatively linked to sociodemographic index, whereas Somalia and Burkina Faso, with lower sociodemographic index, showed the lowest overall difference from the frontier boundaries of mortality and disability-adjusted life-years. Conclusions RHD remains a major global public health issue. Countries such as Somalia and Burkina Faso are particularly successful in managing adverse outcomes from RHD and may serve as a template for other countries.

Introduction: Rheumatic heart disease (RHD) is responsible for a significant burden of cardiovascular morbidity and mortality, and remains the most common cause of acquired heart disease among children and young adults in low-income and middle-income countries. Additionally, the global COVID-19 pandemic has forced the emergency restructuring of many health systems, which has had a broad impact on health in general, including cardiovascular disease. Despite significant cost to the health system and estimates from 2015 indicating both high incidence and prevalence of RHD in South Africa, no cohesive national strategy exists. An updated review of national burden of disease estimates, as well as literature on barriers to care for patients with RHD, will provide crucial information to assist in the development of a national RHD programme., Methods and Analysis: Using predefined search terms that capture relevant disease processes from Group A Streptococcal (GAS) infection through to the sequelae of RHD, a search of PubMed, Scopus, ISI Web of Science, Sabinet African Journals, SA Heart and Current and Completed Research databases will be performed. All eligible studies on RHD, acute rheumatic fever and GAS infection published from April 2014 to December 2022 will be included. Vital registration data for the same period from Statistics South Africa will also be collected. A standardised data extraction form will be used to capture results for both quantitative and qualitative analyses. All studies included in burden of disease estimates will undergo quality assessment using standardised tools. Updated estimates on mortality and morbidity as well as a synthesis of work on primary, secondary and tertiary prevention of RHD will be reported., Ethics and Dissemination: No ethics clearance is required for this study. Findings will be disseminated in a peer-reviewed journal and submitted to national stakeholders in RHD., Prospero Registration Number: CRD42023392782., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Rheumatic heart disease (RHD) remains a significant public health problem in countries with limited health resources. People living with RHD face numerous social challenges and have difficulty navigating ill-equipped health systems. This study sought to understand the impact of RHD on PLWRHD and their households and families in Uganda., Methods: In this qualitative study, we conducted in-depth interviews with 36 people living with RHD sampled purposively from Uganda's national RHD research registry, stratifying the sample by geography and severity of disease. Our interview guides and data analysis used a combination of inductive and deductive methods, with the latter informed by the socio-ecological model. We ran thematic content analysis to identify codes that were then collapsed into themes. Coding was done independently by three analysts, who compared their results and iteratively updated the codebook., Results: The inductive portion of our analysis, which focused on the patient experience, revealed a significant impact of RHD on work and school. Participants often lived in fear of the future, faced limited childbirth choices, experienced domestic conflict, and suffered stigmatization and low self-esteem. The deductive portion of our analysis focused on barriers and enablers to care. Major barriers included the high out-of-pocket cost of medicines and travel to health facilities, as well as poor access to RHD diagnostics and medications. Major enablers included family and social support, financial support within the community, and good relationships with health workers, though this varied considerably by location., Conclusion: Despite several personal and community factors that support resilience, PLWRHD in Uganda experience a range of negative physical, emotional, and social consequences from their condition. Greater investment is needed in primary healthcare systems to support decentralized, patient-centered care for RHD. Implementing evidence-based interventions that prevent RHD at district level could greatly reduce the scale of human suffering. There is need to increase investment in primary prevention and tackling social determinants, to reduce the incidence of RHD in communities where the condition remains endemic., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2023 The Author(s).)

It is a sad reality that although eminently preventable, and despite possessing such knowledge for >70 years, rheumatic heart disease (RHD) remains the most common cause of cardiovascular morbidity and early mortality in young people worldwide. A disease of the poor, RHD is one of the most neglected diseases. Several challenges are unique to the acute rheumatic fever/RHD continuum and contribute to its persistence, including its sequestration among the poorest, its protracted natural history, the erratic availability of penicillin, and the lack of a concerted effort in endemic regions. However, there is cause for optimism following a resurgence in scientific interest over the last 15 years. This review presents the latest advancements in epidemiology, diagnosis, and management. It also discusses pressing research questions on disease pathophysiology, the barriers to implementation of effective management strategies, and pragmatic policy solutions required for translation of current knowledge into meaningful action., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Purpose: To create a cohort with high specificity for moderate and severe rheumatic heart disease (RHD) in New Zealand, not reliant on International Classification of Diseases discharge coding. To describe the demography and cardiac profile of this historical and contemporary cohort., Design and Participants: Retrospective identification of moderate or severe RHD with disease onset by 2019. Case identification from the following data sources: cardiac surgical databases, RHD case series, percutaneous balloon valvuloplasty databases, echocardiography databases, regional rheumatic fever registers and RHD clinic lists. The setting for this study was a high-income country with continued incidence of acute rheumatic fever (ARF)., Findings to Date: A Registry cohort of 4959 patients was established. The initial presentation was RHD without recognised prior ARF in 41%, and ARF in 59%. Ethnicity breakdown: Māori 38%, Pacific 33.5%, European 21.9%, other 6.7%. Ethnic disparities have changed significantly over time. Prior to 1960, RHD cases were 64.3% European, 25.3% Māori and 6.7% Pacific. However, in contrast, from 2010 to 2019, RHD cases were 10.7% European, 37.4% Māori and 47.2% Pacific.Follow-up showed 32% had changed region of residence within New Zealand from their initial presentation. At least one cardiac intervention (cardiac surgery, transcatheter balloon valvuloplasty) was undertaken in 64% of the cohort at a mean age of 40 years. 19.8% of the cohort had multiple cardiac interventions. At latest follow-up, 26.9% of the cohort died. Of those alive, the mean follow-up is 20.5+19.4 years. Māori and Pacific led governance groups have been established to provide data governance and oversight for the registry., Future Plans: Detailed mortality and morbidity of the registry cases will be defined by linkage to New Zealand national health data collections. The contemporary cohort of the registry will be available for future studies to improve clinical management and outcomes for the 3450 individuals living with chronic RHD., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Percutaneous balloon mitral valvuloplasty (PBMV) is primarily performed for rheumatic mitral stenosis (MS). Therefore, limited data exist on PBMV in countries with a low incidence of rheumatic disease. Using the Nationwide Readmission Database, we examined trends in in-hospital mortality and 30-day readmission among patients who received PBMV for rheumatic and non-rheumatic MS. We also examined the change in 90-day hospitalization rate before vs after PBMV. Between 2016 and 2019, there were 1109 hospitalizations in which patients received PBMV for rheumatic (n = 955, 86.1%) vs non-rheumatic MS (n = 154, 13.9%). The all-cause in-hospital mortality for rheumatic and non-rheumatic MS did not change over time (0.9% → 2.0%, P = 0.94, and 5.9% → 9.5%, P = 0.09 respectively). Similarly, the 30-day readmission for patients with rheumatic and non-rheumatic MS did not change over time (12.4% → 9.9%, P = 0.26, and 4.4% → 10.5%, P = 0.30, respectively). The 90-day all-cause hospitalization rate remained the same before vs after PBMV for rheumatic and non-rheumatic MS (25.5% → 21.8%; P = 0.14, and 24.0% → 33.7%; P = 0.19, respectively). Although no statistically significant change was noted over time for trends in in-hospital mortality, 30-day readmission, or even in the change in 90-day all-cause hospitalizations before and after PBMV for both types of MS, among those with non-rheumatic MS, there was a signal of an increase in the in-hospital mortality, and 30-day readmission, even more, there was 29% relative increase in 90-day hospitalizations after PBMV. Future studies are needed to examine the role of PBMV in patients with non-rheumatic MS., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Acute Rheumatic Fever (ARF) and Rheumatic Heart Disease (RHD) are autoimmune sequelae of Group A Streptococcus infection with significant global disease burden. The pathogenesis of these diseases is poorly understood, and no immune modulating therapies are available to stop progression from ARF to RHD. Cytokines and chemokines are immune signaling molecules critical to the development of autoimmune diseases. An increasing number of studies point to a central role for pro-inflammatory cytokines and chemokines in ARF and RHD pathogenesis, in particular IL-6, IL-8/CXCL8, and TNFα, which are elevated in circulation in both ARF and RHD patients. Histological studies of RHD valve tissue implicates Th1 and Th17 associated pro-inflammatory cytokines, chemokine CXCL9, and the fibrosis-associated cytokine TGF-β in progressive cycles of inflammatory damage and fibrotic repair. Taken together, this suggests immune molecules contribute to both the acute inflammatory disease stage of ARF, as well as cardiac remodeling and valve dysfunction in RHD. Monoclonal antibody blockade of pro-inflammatory cytokines IL-6 and TNFα are approved therapies for many autoimmune diseases and the most successful immunomodulating therapies for rheumatoid arthritis. Current evidence suggests possible benefit for ARF patients from IL-6 and TNFα blockade, in particular to interrupt progression to RHD, and warrants immediate investigation., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Background: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) disproportionately affect Aboriginal and Torres Strait Islander people in Australia, with devastating impacts on morbidity, mortality and community wellbeing. Research suggests that general practitioners and primary care staff perceive insurmountable barriers to improving clinical outcomes, including the need for systemic change outside their scope of practice., Objective: The aim of this article is to identify constructive, micro-level strategies that primary healthcare clinicians can consider, adopt and sustainably use to improve care for people with ARF and RHD in their routine clinical practice., Discussion: Through skilled clinical care, reflection and culturally safe practices, individual primary healthcare clinicians have substantial capacity to improve care experiences and outcomes for Aboriginal and Torres Strait Islander people and communities affected by ARF and RHD.

In South Africa, maternal mortality from cardiovascular disease remains high. The recent Saving Mothers report 2017-2019 from the Confidential Enquiries into Maternal Deaths revealed that indirect maternal death from medical and surgical disorders is the fourth commonest cause of maternal death, accounting for 16.9% of deaths, with cardiac disease accounting for one-third of this. The burden of rheumatic heart disease (RHD) is a significant contributor to maternal morbidity and mortality. The true burden is unknown due to limited data. The natural history of RHD confers additional risk as many cases may remain undiagnosed, with first presentation occurring during pregnancy. This undiagnosed subset of women may be the result of poor accessibility to healthcare facilities and primary healthcare interventions for acute rheumatic fever. RHD causes progressive damage to the heart valves, especially the left-sided valves, which eventually require surgical correction with mechanical prosthetic valves.

Introduction: Globally, rheumatic heart disease (RHD) is an important cause of acquired heart disease in children and adolescents. Clinical trials on RHD treatment with Traditional Chinese Medicine (TCM) or integrated medicine are gradually increasing in China. However, because the outcomes of clinical trials are subject to heterogeneity and selective reporting, similar studies cannot be merged and compared, complicating assessing the effectiveness and safety of TCM, and diminishing the value of clinical trials. Therefore, there is an urgent need to design a TCM or integrated medicine core outcome set (COS) for RHD., Methods and Analysis: The development of this study will take place in four stages under the direction of a multidisciplinary advisory board. (1) Establishing a comprehensive outcomes checklist through a systematic review of previously published research, retrieval of clinical trial registration centres, patient's semistructured interviews, and clinician's questionnaire surveys; (2) Screen stakeholder groups from various fields to participate in the Delphi survey; (3) Two e-Delphi surveys will be conducted to determine the outcomes of various concerned stakeholder groups; (4) Hold a face-to-face consensus meeting to develop the COS-TCM-RHD., Ethics and Dissemination: The ethical approval for this study has been obtained from the Tianjin University of Traditional Chinese Medicine Ethics Committee (TJUTCM-EC20210008). The findings will be disseminated in peer-reviewed journals and meetings., Trial Registration Number: This study has been registered at the Core Outcome Measures in Effectiveness Trials (COMET) database (Registration #1743)., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are rare in high-income countries; however, in Aotearoa New Zealand ARF and RHD disproportionately affect Indigenous Māori and Pacific Peoples. This narrative review explores the evidence regarding non-surgical management of patients with clinically significant valve disease or heart failure due to RHD., Methods: Medline, EMBASE and Scopus databases were searched, and additional publications were identified through cross-referencing. Included were 28 publications from 1980 onwards., Results: Of the available interventions, improved anticoagulation management and a national RHD register could improve RHD outcomes in New Zealand. Where community pharmacy anticoagulant management services (CPAMS) are available good anticoagulation control can be achieved with a time in the therapeutic range (TTR) of more than 70%, which is above the internationally recommended level of 60%. The use of pharmacists in anticoagulation control is cost-effective, acceptable to patients, pharmacists, and primary care practitioners. There is a lack of local data available to fully assess other interventions; including optimal therapy for heart failure, equitable access to specialist RHD care, prevention, and management of endocarditis., Conclusion: As RHD continues to disproportionately affect Indigenous and minority groups, pro-equity tertiary prevention interventions should be fully evaluated to ensure they are reducing disease burden and improving outcomes in patients with RHD., (Copyright © 2022 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Aims: The histopathology of mitral valve (MV) tissues have been reported in necropsy and retrospective studies. We prospectively studied the histopathological changes in rheumatic mitral stenosis using advanced techniques and corroborated these with clinical presentation, pathogenesis, and management., Methods: From January 2020 to February 2021, surgically excised rheumatic stenotic MV from 54 Tanzanian patients were studied. These were examined using hematoxylin-eosin, von Kossa staining, and immunohistochemistry., Results: The median (range) age of patients was 39 (14-57) years with 34 (63%) females. Secondary prophylaxis was given to 7 (13%) patients and 2 (3.7%) had evidence of rheumatic fever (RF). With hematoxylin-eosin, 37 (68.5%) specimens showed fibrinoid degeneration (FD), 44 (81.5%) leucocytic infiltrates, 6 (11.1%) Aschoff nodules, 30 (55.6%) calcification, and 39 (72.2%) fibrosis. Thirty-five (64.8%) specimens were positive to von Kossa. The proportion of specimens positive for CD3, CD20, CD68, and CD8 were 46 (85.2%), 35 (64.8%), 39 (72.2%), and 8 (14.8%) respectively. Valvular calcium was high among older patients, males and with a higher trans-MV gradient. The degree of inflammatory cellular infiltration was associated with valvular calcification, FD with ARF, leucocytic infiltrates with disease duration of <10 years, and fibrosis with the absence of atrial fibrillation. C-reactive protein and anti-streptolysin titres were high in CD20 and CD8 staining cells., Conclusion: This study confirms that high MV calcium are found in patients who are old, male, and with severe mitral stenosis. The association between clinical parameters with histopathological-immunohistochemical studies observed in our study provides new insight to disease presentation. We found a low rate of secondary prophylaxis and two patients with ARF. Our findings are comparable with those from other countries suggesting similar pathogenesis and thus intervention modalities. This is the first study on mitral valve histopathology to be reported from Africa., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Rheumatic heart disease (RHD) is the most commonly acquired heart disease in children and young people in low and middle-income settings. Fragile health systems and scarcity of data persist to limit the understanding of the relative burden of this disease. The aims of this study were to estimate the prevalence of RHD and to assess the RHD-related health care systems in Namibia., Methods: Data was retrieved from outpatient and inpatient registers for all patients diagnosed and treated for RHD between January 2010 to December 2020. We used descriptive statistics to estimate the prevalence of RHD. Key observations and engagement with local cardiac clinicians and patients helped to identify key areas of improvement in the systems., Results: The outpatient register covered 0.032% of the adult Namibian population and combined with the cumulative incidence from the inpatient register we predict the prevalence of clinically diagnosed RHD to be between 0.05% and 0.10% in Namibia. Young people (< 18 years old) are most affected (72%), and most cases are from the north-eastern regions. Mitral heart valve impairment (58%) was the most common among patients. We identified weaknesses in care systems i.e., lack of patient unique identifiers, missing data, and clinic-based prevention activities., Conclusion: The prevalence of RHD is expected to be lower than previously reported. It will be valuable to investigate latent RHD and patient follow-ups for better estimates of the true burden of disease. Surveillance systems needs improvements to enhance data quality. Plans for expansions of the clinic-based interventions must adopt the "Awareness Surveillance Advocacy Prevention" framework supported by relevant resolutions by the WHO., (© 2022. The Author(s).)

Objectives: Rheumatic fever (RF) and rheumatic heart disease (RHD) remain among the major heart problems among children in Nepal. Although these conditions are preventable and treatable, the lack of proper knowledge and resources to diagnose and manage these conditions in rural health centres is a key concern. This study assessed the impact of educational sessions to improve the knowledge of healthcare workers in the early recognition, diagnosis, and management of RF and RHD in rural far-western Nepal., Design, Setting and Participants: This study used a pretest and post-test interventional design and was conducted among 64 healthcare workers in two primary healthcare centres and a peripheral district-level hospital in Achham district in the far-western region of Nepal. A self-administered questionnaire was used before and after the educational sessions. Data were analysed using SPSS V.21., Results: The overall test scores increased from 10 (SD=2.4) pre-intervention to 13.8 (SD=1.9) post-intervention (p<0.001). Similarly, participant confidence (graded 1-5) in differentiating bacterial from viral sore throat rose from 3.6 (SD=1.08) pre-intervention to 3.98 (SD=1.09) post-intervention (p<0.05). Confidence in managing RF increased from 3.9 (SD=0.88) pre-intervention to 4.30 (SD=0.8) post-intervention (p<0.001)., Conclusion: The findings suggest that the investigated educational sessions are promising with respect to improving the knowledge and confidence of healthcare workers in the early recognition, diagnosis, and management of RF and RHD at the primary healthcare level. Further studies with a larger sample size and conducted in different parts of the country are warranted to assess the effectiveness and impact of scaling up such educational interventions in Nepal., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To evaluate the long-term clinical outcomes of children with rheumatic heart disease (RHD) in Uganda, and determine characteristics that predict adverse outcomes., Methods: This retrospective cohort study evaluated the risk of death in Ugandan children with clinical RHD from 2010 to 2018; enrolling children aged 5-18 years old from an existing registry. Demographic data and clinical data (baseline complications, RHD severity, cardiac interventions) were collected. The primary outcome was survival. Univariable and multivariable hazard ratios (HR) were obtained from Cox proportional hazards regression. Survival probabilities were developed using Kaplan-Meier curves; log-rank tests compared survival based on cardiac interventions, disease severity and time of enrolment., Results: 612 cases met inclusion criteria; median age 12.8 years (IQR 5.3), 37% were male. Thirty-one per cent (187 of 612) died during the study period; median time to death 7.8 months (IQR 18.3). In univariable analysis, older age (HR 1.26, 95% CI=1.0 to 1.58), presence of baseline complications (HR 2.06, 95% CI=1.53 to 2.78) and severe RHD (HR 5.21, 95% CI=2.15 to 12.65) were associated with mortality. Cardiac intervention was associated with a lower risk of mortality (HR 0.06, 95% CI=0.02 to 0.24). In multivariable models, baseline complications (HR 1.78, 95% CI=1.31 to 2.41), severe RHD (HR 4.58, 95% CI=1.87 to 11.23) and having an intervention (HR 0.05, 95% CI=0.01 to 0.21) remained statistically significant. Kaplan-Meier survival curves demonstrated >25% mortality in the first 30 months, with significant differences in mortality based on intervention status and severity of disease., Conclusions: The mortality rate of children with clinical RHD in Uganda exceeds 30%, over an 8-year time frame, despite in-country access to cardiac interventions. Children at highest risk were those with cardiac complications at baseline and severe RHD., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Rheumatic Fever (RF)/ Rheumatic Heart Disease (RHD) is the result of autoimmune response triggered by group A Beta-haemolytic streptococcal pharyngitis leading to immune-inflammatory injury to cardiac valves. It is practically disappeared in developed countries. However, it continues to be a major cause of disease burden among children, adolescents, and young adults in low-income countries and even in high-income countries with socioeconomic inequalities. For decades, many cases of Acute Rheumatic Fever (ARF) and RHD were missed and were denied the secondary prophylaxis, as a result these patients used to end up with complications and untimely death. Advanced understanding of the echocardiography can prevent both under diagnosis and over diagnosis and thus help in management strategy. Another new advancement in recent past is the mitral valve repair, which is technically demanding, and the results are acceptable in experienced cardiac surgical units. Whenever feasible, valve repair should be preferred over valve replacement since it precludes the need for anticoagulation and future risks of prosthesis dysfunction., (© Journal of the Association of Physicians of India 2011.)

Background: Rheumatic heart disease (RHD) persists in low-middle-income countries and in high-income countries where there are health inequities. RHD in pregnancy (RHD-P) is associated with poorer maternal and perinatal outcomes. Our study examines models of care for women with RHD-P from the perspectives of health care providers., Methods: A descriptive qualitative study exploring Australian health professionals' perspectives of care pathways for women with RHD-P. Thematic analysis of semi-structured interviews with nineteen participants from maternal health and other clinical and non-clinical domains related to RHD-P., Results: A constellation of factors challenged the provision of integrated women-centred care, related to health systems, workforces and culture. Themes that impacted on the provision of quality woman-centred care included conduits of care - helping to break down silos of information, processes and access; 'layers on layers' - reflecting the complexity of care issues; and shared understandings - factors that contributed to improved understandings of disease and informed decision-making., Conclusions: Pregnancy for women with RHD provides an opportunity to strengthen health system responses, improve care pathways and address whole-of-life health. To respond effectively, structural and cultural changes are required including enhanced investment in education and capacity building - particularly in maternal health - to support a better informed and skilled workforce. Aboriginal Mothers and Babies programs provide useful exemplars to guide respectful effective models of care for women with RHD, with relevance for non-Indigenous women in high-risk RHD communities.For key goals to be met in the context of RHD, maternal health must be better integrated into RHD strategies and RHD better addressed in maternal health., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2021 The Author(s).)

RHD in pregnancy (RHD-P) is associated with an increased burden of maternal and perinatal morbidity and mortality. A sequellae of rheumatic fever resulting in heart valve damage if untreated, RHD is twice as common in women. In providing an historical overview, this commentary provides context for prevention and treatment in the 21 st century. Four underlying themes inform much of the literature on RHD-P: its association with inequities; often-complex care requirements; demands for integrated care models, and a life-course approach. While there have been some gains particularly in awareness, strengthened policies and funding strategies are required to sustain improvements in the RHD landscape and consequently improve outcomes. As the principal heart disease seen in pregnant women in endemic regions, it is unlikely that the Sustainable Development Goal 3 target of reduced global maternal mortality ratio can be met by 2030 if RHD is not better addressed for women and girls., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2021 The Author(s).)

Background: Subjective wellbeing (SWB) is a self-reported positive life judgment and good feeling. RHD, rheumatic heart disease, is a long-term sequel of single or recurrent acute rheumatic fever. There are no studies that assessed SWB in RHD patients. We aimed to assess SWB among RHD subjects enrolled in chronic care at Tikur Anbessa Specialized Hospital (TASH), Ethiopia., Methods: This observational cross-sectional study employed a validated Amharic WHO-5 wellbeing index to assess SWB. Sociodemographic and clinical data were collected using structured questionnaire. RHD subjects aged 9 years and above were included. Factors associated with SWB were assessed using logistic regression models., Results: The study included 384 subjects, females 68.2% (262). Children, < 18 years, constituted one third of study subjects, 32.8% (126). Moderate and severe echocardiographic RHD dominated, 85.9% (330) with no associated comorbidity, 84.4% (324). Only 17.2% (66) had surgical or device intervention. Poor SWB was documented in 9.6% of study subjects (95% CI: 6.88-13.04). On multivariable regression, those with younger age RHD diagnosis, < 20 years, had almost three times higher odds of poor SWB, adjusted odds ratio (aOR) 2.69(95% CI: 1.30-5.58, P 0.008). Those with monthly family income of < 1000 Ethiopian Birr had three times higher odds of poor SWB, aOR 2.97(95% CI: 1.24-7.1, P 0.014). Study subjects who had good medication adherence had reduced odds of poor SWB, aOR 0.37(95% CI: 0.18-0.77, P 0.028). Those who received psychologic support from their families also had reduced odds of poor SWB, aOR 0.26(95% CI: 0.11-0.64, P 0.003)., Conclusion: Poor SWB was documented in one-tenth of RHD patients. Family income, younger age at RHD diagnosis, medication adherence and psychological support predicted poor SWB. Poor SWB has to be considered and assessed among RHD patients particularly among those with younger age at RHD diagnosis and poor family income. Further mixed studies are recommended to assess how medication adherence and psychological supports associate with positive SWB among RHD patients., (© 2021. The Author(s).)

Rheumatic heart disease (RHD) remains a leading cause of mortality and morbidity in pregnant patients in low- to middle-income countries. Apart from the clinical challenges, these areas face poor infrastructure and resources to allow for early detection, with many women presenting to medical services for the first time when they deteriorate clinically during the pregnancy. The opportunity for preconception counselling and planning may thus be lost. It is ideal for all women to be seen before conception and risk-stratified according to their clinical state and pathology. The role of the cardio-obstetrics team has emerged over the past decade with the aim of a seamless transition to and from the appropriate levels of care during pregnancy. Severe symptomatic mitral and aortic valve stenoses portend the greatest risk to both mother and fetus. In mitral stenosis, beta-blockers are the cornerstone of therapy and only a small number of patients require balloon valvuloplasty. Regurgitant lesions mostly require diuretics alone for the treatment of heart failure. The mode of delivery is usually vaginal; caesarean section is performed in those with obstetrical indications or in cases with severe stenosis and a poor clinical state. The postpartum period presents a second high-risk period for maternal adverse events, with heart failure and arrhythmias being the most frequent. This review aims to provide a practical evidence-based multi-disciplinary approach to the management of women with RHD in pregnancy., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: The World Symposium of Pulmonary Hypertension in 2018, updated the definition of pulmonary hypertension (PH) as mean pulmonary artery pressures (PAP) > 20 mmHg. Pulmonary venous hypertension secondary to left-heart disease, constitutes the most common cause of PH, and the determination of a co-existent pre-capillary (primary) PH becomes paramount, particularly at the moment of evaluating and managing patients with heart failure. Pulmonary artery pressures above the systemic pressures define supra-systemic PH and generally leads to frank right ventricular failure and high mortality., Case Presentation: We present the perioperative management of a patient with rheumatic mitral valve disease, initially found to have severe PH due to pulmonary venous hypertension, who underwent percutaneous mitral balloon valvuloplasty complicated with mitral chordae rupture, severe mitral regurgitation and supra-systemic PH. Multiple medical therapies and an intra-aortic balloon pump were used as means of non-surgical management of this complication., Conclusions: This case report illustrates the perioperative implications of combined pre- and post-capillary PH and supra-systemic PH, as this has not been widely discussed in previous literature. A thorough literature review of the clinical characteristics of PH, methods to determine co-existent pre- and post-capillary PH components, as well as concomitant right ventricular failure is presented. Severe PH has known detrimental effects on the hemodynamic status of patients, which can ultimately lead to a decrease in effective cardiac output and poor tissue perfusion., (© 2021. The Author(s).)

Background: The strategy of isolated tricuspid valve surgery has undergone innovations in recent years. This study aimed to summarize our experience using an endoscopic approach to repeat isolated tricuspid valve surgery (RITS) after left-sided valve replacement (LSVR)., Methods: From June 2013 to May 2019, 79 patients underwent endoscopic RITS after LSVR at our institution. Patients were divided into the tricuspid valvuloplasty (TVP) group (N.=49) and the tricuspid valve replacement (TVR) group (N.=30); perioperative outcomes and follow-up results were compared., Results: There were seven postoperative deaths (8.9%). In-hospital mortality was higher in the TVR group than in the TVP group, although this difference was not statistically significant (13.3% vs. 6.1%, P=0.417). More patients experienced residual moderate-to-severe tricuspid regurgitation (TR) at discharge in the TVP group than in the TVR group (26.7% vs. 0%, P=0.003). Five patients died from heart, and multiorgan failure during follow-up; the overall 3- and 5-year survival rates were 93.8% [95% confidence interval (CI): (87.1-99.9%)] and 85.3% (95% CI: 73.3-99.2%), respectively, and no significant differences were found between the two groups (P=0.103). The overall rates of the 3- and 5-year freedom from severe recurrent TR were 93.2% (95% CI: 85.9-99.9%) and 89.0% (78.7-99.9%), respectively, and no significant difference was found between groups (P=0.176)., Conclusions: Repeat isolated tricuspid valve surgery after left-sided valve replacement is associated with adverse perioperative outcomes. Endoscopic access offers an alternative approach for RITS after LSVR with acceptable results. TVP results in lower surgical mortality than TVR while carrying a higher risk of residual moderate-to-severe TR.

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 <0.001). The updated score model showed good performance in predicting unfavorable outcome. Conclusions The echocardiographic score model for predicting RHD outcome was updated and validated for different latent RHD populations. It has potential utility in the clinical and screening setting for risk stratification of latent RHD.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract-based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non-White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08-1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline-based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Background: Rheumatic heart disease (RHD) is still a concerning issue in developing countries. Among delayed RHD presentations, rheumatic mitral valve stenosis (MS) remains a prevalent finding. Percutaneous transvenous mitral commissurotomy (PTMC) is the intervention of choice for severe mitral stenosis (MS). We aimed to assess the mid-term outcome of PTMC in patients with immediate success., Methods: In this retrospective cohort study, out of 220 patients who had undergone successful PTMC between 2006 and 2018, the clinical course of 186 patients could be successfully followed. Cardiac-related death, undergoing a second PTMC or mitral valve replacement (MVR) were considered adverse cardiac events for the purpose of this study. In order to find significant factors related to adverse cardiac outcomes, peri-procedural data for the studied patients were collected.The patients were also contacted to find out their current clinical status and whether they had continued secondary antibiotic prophylaxis regimen or not. Those who had not suffered from the adverse cardiac events were additionally asked to undergo echocardiographic imaging, in order to assess the prevalence of mitral valve restenosis, defined as mitral valve area (MVA) < 1.5 cm 2 and loss of ≥ 50% of initial area gain., Results: During the mean follow-up time of 5.69 ± 3.24 years, 31 patients (16.6% of patients) had suffered from adverse cardiac events. Atrial fibrillation rhythm (p = 0.003, HR = 3.659), Wilkins echocardiographic score > 8 (p = 0.028, HR = 2.320) and higher pre-procedural systolic pulmonary arterial pressure (p = 0.021, HR = 1.031) were three independent predictors of adverse events and immediate post-PTMC mitral valve area (IMVA) ≥ 2 cm 2 (p < 0.001, HR = 0.06) was the significant predictor of event-free outcome. Additionally, follow-up echocardiographic imaging detected mitral restenosis in 44 patients (23.6% of all patients). The only statistically significant protective factor against restenosis was again IMVA ≥ 2 cm 2 (p = 0.001, OR = 0.240)., Conclusion: The mid-term results of PTMC are multifactorial and may be influenced by heterogeneous peri-procedural determinants. IMVA had a great impact on the long-term success of this procedure. Continuing secondary antibiotic prophylaxis was not a protective factor against adverse cardiac events in this study. (clinicaltrial.gov registration: NCT04112108)., (© 2021. The Author(s).)

Background: Rheumatic Heart Disease (RHD) is a disease of poverty that is neglected in developing countries, including South Africa. Lack of adequate evidence regarding the cost of RHD care has hindered national and international actions to prevent RHD related deaths. The objective of this study was to estimate the cost of RHD-related health services in a tertiary hospital in the Western Cape, South Africa., Methods: The primary data on service utilisation were collected from a randomly selected sample of 100 patient medical records from the Global Rheumatic Heart Disease Registry (the REMEDY study) - a registry of individuals living with RHD. Patient-level clinical data, including, prices and quantities of medications and laboratory tests, were collected from the main tertiary hospital providing RHD care. All annual costs from a health system perspective were estimated in 2017 (base year) in South African Rand (ZAR) using a combination of ingredients and step-down costing approaches and later converted to United States dollars (USD). Step-down costing was used to estimate provider time costs and all other facility costs such as overheads. A 3% discount rate was also employed in order to allow depreciation and opportunity cost. We aggregated data to estimate the total annual costs and the average annual per-patient cost of RHD and conducted a one-way sensitivity analysis., Results: The estimated total cost of RHD care at the tertiary hospital was USD 2 million (in 2017 USD) for the year 2017, with surgery costs accounting for 65%. Per-patient, average annual costs were USD 3900. For the subset of costs estimated using the ingredients approach, outpatient medications, and consumables related to cardiac catheterisation and heart valve surgery were the main cost drivers., Conclusions: RHD-related healthcare consumes significant tertiary hospital resources in South Africa, with annual per-patient costs higher than many other non-communicable and infectious diseases. This analysis supports the scaling up of primary and secondary prevention programmes at primary health centers in order to reduce future tertiary care costs. The study could also inform resource allocation efforts and provide cost estimates for future studies of intervention cost-effectiveness.

Mitral stenosis (MS) is associated with left atrial (LA) functional and morphological changes as a result of chronic increase in LA pressure. Relieving the mitral obstruction via balloon mitral valvuloplasty (BMV) might be associated with LA structural and functional remodeling. To study alterations of LA volume and functions 1 year following successful BMV in patients with isolated rheumatic severe mitral stenosis. Thirty patients (median age 33 years, 22 women) with severe rheumatic MS were included in the study. Using biplane method, trans-thoracic 2D echocardiography was used to estimate LA volume indexed to body surface area (BSA). Maximal, minimal and pre-A left atrial volumes were measured and indexed to BSA. LA volumetric functions were then assessed and the measurements were repeated 6 months and 1 year after successful valvuloplasty. At baseline, median mitral valve area (MVA) was 0.9 (0.6-1.3) cm 2 measured by planimetry with a mean pressure gradient of 12.5 (8-24) mmHg. Following BMV, a significant regression of left atrial volume index was noticed at 6 months compared to baseline (51 vs. 60 ml/m 2 , p = 0.001) with a further decrease at 1 year (48 vs. 51 ml/m 2 , p = 0.03). At 6 months, volumetric assessment of left atrial functions showed a significant improvement in LA total emptying fraction (42% vs 30%, p = 0.001) as well as in LA passive emptying fraction (26% vs 14%, p = 0.033) and LA active emptying fraction (20% vs. 18%, p = 0.016). All these indices showed further improvement at 1 year [47% (P = 0.02), 29% (p = 0.03) and 31% (p = 0.001) respectively]. In patients with isolated rheumatic MS, mitral valvuloplasty was associated with a significant decline of LA volume accompanied by a significant improvement of its volumetric functions.

During the 1920s, acute rheumatic fever (ARF) was the leading cause of mortality in children in the United States. By the 1980s, many felt ARF had all but disappeared from the US. However, although ARF and rheumatic heart disease (RHD) rates remain low in the US today, disease burden is unequal and tracks along other disparities of cardiovascular health. It is estimated that 1% to 3% of patients with untreated group A streptococcus (GAS) infection, most typically GAS pharyngitis, will develop ARF, and of these, up to 60% of cases will result in chronic RHD. This article reviews the epidemiology, pathogenesis, diagnosis, and management of ARF/RHD to increase awareness of ARF/RHD for clinicians based in the US. [ Pediatr Ann. 2021;50(3):e98-e104.] .

Introduction: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) cause significant morbidity and premature mortality among Australian Aboriginal and Torres Strait Islander peoples. RHDAustralia has produced a fully updated clinical guideline in response to new knowledge gained since the 2012 edition. The guideline aligns with major international ARF and RHD practice guidelines from the American Heart Association and World Heart Federation to ensure best practice. The GRADE system was used to assess the quality and strength of evidence where appropriate., Main Recommendations: The 2020 Australian guideline details best practice care for people with or at risk of ARF and RHD. It provides up-to-date guidance on primordial, primary and secondary prevention, diagnosis and management, preconception and perinatal management of women with RHD, culturally safe practice, provision of a trained and supported Aboriginal and Torres Strait Islander workforce, disease burden, RHD screening, control programs and new technologies., Changes in Management as a Result of the Guideline: Key changes include updating of ARF and RHD diagnostic criteria; change in secondary prophylaxis duration; improved pain management for intramuscular injections; and changes to antibiotic regimens for primary prevention. Other changes include an emphasis on provision of culturally appropriate care; updated burden of disease data using linked register and hospitalisations data; primordial prevention strategies to reduce streptococcal infection addressing household overcrowding and personal hygiene; recommendations for population-based echocardiographic screening for RHD in select populations; expanded management guidance for women with RHD or ARF to cover contraception, antenatal, delivery and postnatal care, and to stratify pregnancy risks according to RHD severity; and a priority classification system for presence and severity of RHD to align with appropriate timing of follow-up., (© 2020 AMPCo Pty Ltd.)