Echocardiography Assessment of Rheumatic Heart Disease: Implications for Percutaneous Balloon Mitral Valvuloplasty.

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.

Burden of disease and barriers to comprehensive care for rheumatic heart disease in South Africa: an updated systematic review protocol.

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.

Global, Regional, and National Advances Toward the Management of Rheumatic Heart Disease Based on the Global Burden of Disease Study 2019.

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.

Health system costs of rheumatic heart disease care in South Africa.

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.

Rheumatic heart disease anno 2020: Impacts of gender and migration on epidemiology and management.

BACKGROUND: The epidemiology and management of diseases can be influenced by social demographic factors. Gender and migration are among these factors. METHODS: We aimed at reviewing the impacts of gender and migration on rheumatic heart disease (RHD) epidemiology and management by a nonsystematic literature review of published studies on RHD worldwide. Our PubMed search terms included RHD pathophysiology, diagnosis, complications, management or prevention, combined with words 'rheumatic mitral stenosis (MS)', 'outcomes after percutaneous balloon mitral valvuloplasty (PBMV)', 'gender or sex difference' and 'migration'. The reporting of this study conforms to SANRA (the Scale for Assessment of Narrative Review Articles) guidelines. RESULTS: We retrieved eight studies about the impact of sex on outcomes after PBMV. All of these studies showed a female predominance for RHD. Two studies showed that there is no impact, three studies showed female sex as a predictor of poor outcomes, and the other three showed male sex a predictor of poor outcomes. Although RHD is reported to be eradicated in the developed countries, 2.1% of refugees recently screened for RHD in Italy were found to have subclinical RHD. This prevalence is similar to those found in India (2.0%), Cambodia (2.2%) and Mozambique (3%). CONCLUSIONS: There are contradicting results for outcomes after PBMV between males and females. It is not clear whether sex difference plays a role in pathophysiology, diagnosis, management and prognosis of MS. Migration has impacts on epidemiology and management of RHD. Further studies are required in these two fields to explore their relationship to RHD.

Generating Global Priority for Addressing Rheumatic Heart Disease: A Qualitative Policy Analysis.

Background Rheumatic heart disease (RHD) poses a high burden in low-income countries, as well as among indigenous and other socioeconomically disadvantaged populations in high-income countries. Despite its severity and preventability, RHD receives insufficient global attention and resources. We conducted a qualitative policy analysis to investigate the reasons for recent growth but ongoing inadequacy in global priority for addressing RHD. Methods and Results Drawing on social science scholarship, we conducted a thematic analysis, triangulating among peer-reviewed literature, organizational documents, and 20 semistructured interviews with individuals involved in RHD research, clinical practice, and advocacy. The analysis indicates that RHD proponents face 3 linked challenges, all shaped by the nature of the issue. With respect to leadership and governance, the fact that RHD affects mostly poor populations in dispersed regions complicates efforts to coordinate activities among RHD proponents and to engage international organizations and donors. With respect to solution definition, the dearth of data on aspects of clinical management in low-income settings, difficulties preventing and addressing the disease, and the fact that RHD intersects with several disease specialties have fueled proponent disagreements about how best to address the disease. With respect to positioning, a perception that RHD is largely a problem for low-income countries and the ambiguity on its status as a noncommunicable disease have complicated efforts to convince policy makers to act. Conclusions To augment RHD global priority, proponents will need to establish more effective governance mechanisms to facilitate collective action, manage differences surrounding solutions, and identify positionings that resonate with policy makers and funders.

Task Sharing in the Diagnosis, Prevention, and Management of Rheumatic Heart Disease: A Systematic Review.

BACKGROUND: Globally, rheumatic heart disease (RHD) is a major contributor to the burden of cardiovascular disease. Major gaps in RHD prevention and treatment have been documented at all levels of health systems in low- and middle-income countries. Task sharing is an approach that could prove effective in remediating bottlenecks in RHD-related care. OBJECTIVES: This study conducted a systematic review to assess the state of the evidence for the use of task sharing in the diagnosis, prevention, and management of RHD. METHODS: Guided by a previously published protocol, we searched various databases using a systematic search strategy including MeSH and free-text terms for (1) group A streptococcus, acute rheumatic fever, and RHD and (2) strategies of task sharing in limited-resource settings. Two investigators independently screened the search outputs, selected the studies, extracted the data, and assessed the risk of bias, resolving discrepancies by discussion and consensus. RESULTS: The publications search yielded 212 records, of which 18 articles were deemed as potentially eligible for inclusion. None of the studies, however, met with the inclusion criteria. CONCLUSIONS: There is a lack of evidence for the use of task-sharing approaches in scaling up RHD prevention and treatment services in limited-resource settings. Considering the persistent burden of group A streptococcus, acute rheumatic fever, and RHD in low- and middle-income countries, this work highlights the urgent need to develop and test models of RHD-related care utilizing an evidence-based approach to task sharing. [Task Sharing in the Diagnosis, Prevention, and Management of Rheumatic Heart Disease: A Systematic Review; CRD42017072989].

Workforce Cost Model for Expanding Congenital and Rheumatic Heart Disease Services in Kenya.

BACKGROUND: Cardiovascular disease is the number one global killer, with over three quarters of these deaths arising from the populations of low- and middle-income countries (LMICs). Addressing the burden of cardiovascular disease in LMICs must include medical and surgical services for these patients. In this article, we model the needs and costs to scale up the cardiac provider workforce in Kenya, which can be adapted to other LMICs based on country-specific workforce hours and workforce salaries. METHODS: Using published epidemiological reports from sub-Saharan Africa, we structured the model based on the expected disease burden of congenital and rheumatic disease in a simulated 1,000-person population. Services modeled include clinic visits, echocardiograms, diagnostic cardiac catheterizations, interventional catheterizations, and heart surgery. Costs were modeled based on Kenyan public sector salaries. After scaling the model, we created a sensitivity analysis of change in service duration and salaries. RESULTS: Based on a 1,000-person Kenyan population, we estimate that 2.5 heart surgeries will be needed every year, with a corresponding annual workforce cost of US$526. Including accompanying services of clinic visits, echocardiograms, and both diagnostic and interventional cardiac catheterizations, the total annual workforce cost is US$899. Based on estimated productive hours for public sector workforce, 196 full-time equivalent cardiac surgeons will be needed for the entire population of Kenya (2017 figure). CONCLUSIONS: We present a model for appropriate cardiovascular service staffing based on disease burden and workforce costs. This model can be scaled up as needed to plan for local capacity building.

[CURRENT TRENDS IN THE PREVENTION, DIAGNOSIS AND TREATMENT OF RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE (REVIEW)].

The purpose of the work is to analyze the evolution of the clinical course, diagnosis, treatment and prevention of acute rheumatic fever (ARF) and chronic rheumatic heart disease (RHD), antibiotic therapy of streptococcal infection, using modern analysis of modern literature and our own observations. Diagnostic criteria for the disease were revised. Improving the capabilities of echocardiography can improve the prognosis of the disease through the early use of treatment and prevention. The level of ORL / RHD correlates with the economic development of countries. Mutation of the etiological factor of rheumatism - ß-HSA, the formation of antibiotic resistance, increased migration, tourist activity in countries with high morbidity due to ß-HSA can lead to new outbreaks of this infection. Prevention ARF/RHD is multistage. Thus, using the experience of healthcare in different countries, new approaches to prevention can improve disease control.

Task sharing to improve the prevention, diagnosis and management of rheumatic heart disease: a systematic review protocol.

INTRODUCTION: Rheumatic heart disease (RHD) remains an important cause of cardiovascular morbidity and mortality globally due to weak health systems in many countries. RHD can be effectively prevented and managed; however, RHD-related interventions have not been widely adopted in countries with severe human resource constraints. Task sharing is a recognised approach to personnel shortages that could prove effective for RHD and has, anecdotally, been attempted in a few settings. We propose a systematic review and meta-analysis protocol to assess models that use non-physician workers to expand access to, and quality of, RHD-related healthcare. METHODS: We will include randomised controlled trials (RCT), cluster RCTs, quasi-experimental and controlled before and after studies providing information on the effectiveness of non-physician workers in providing care for streptoccocal pharyngitis, rheumatic fever and RHD. We will search relevant electronic databases and grey literature using medical subject headings. Standardised data extraction forms will be used to collect effect sizes that will be pooled in random-effects models. We will also conduct subgroup analyses and note other important quantitative findings, such as cost reduction, and qualitative findings, such as patient satisfaction. We will also assess study quality and risk of bias and metabias. ETHICS AND DISSEMINATION: Ethics approval is not required for this systematic review of previously published literature. The results of the systematic review will be broadly disseminated via conference presentations, multidisciplinary workshops and peer-reviewed publications. PROSPERO REGISTRATION NUMBER: CRD42017072989.

Asymptomatic rheumatic heart disease in South African schoolchildren: Implications for addressing chronic health conditions through a school health service.

When new evidence comes to light, it compels us to contemplate the implications of such evidence for health policy and practice. This article examines recent research evidence on the prevalence of asymptomatic rheumatic heart disease (RHD) in South Africa and considers the implications for the Integrated School Health Programme (ISHP). RHD is still a major burden of disease in developing countries, and elimination of this preventable condition ranks high among World Heart Federation goals. If left untreated, it becomes a chronic health condition that individuals have to cope with into their adult lives. The ISHP regards the health needs of children with chronic health conditions, which include conditions such as RHD, as a key service component. However, the chronic health component of the ISHP is still poorly developed and can benefit from good evidence to guide implementation. A recent study to ascertain the prevalence of RHD in asymptomatic schoolchildren through mass screening affords an opportunity to reflect on whether, and how, asymptomatic chronic health conditions in schoolchildren could be addressed, and what the implications would be if this were done through a school-based programme such as the ISHP.

Congenital heart disease and rheumatic heart disease in Africa: recent advances and current priorities.

Africa has one of the highest prevalence of heart diseases in children and young adults, including congenital heart disease (CHD) and rheumatic heart disease (RHD). We present here an extensive review of recent data from the African continent highlighting key studies and information regarding progress in CHD and RHD since 2005. Main findings include evidence that the CHD burden is underestimated mainly due to the poor outcome of African children with CHD. The interest in primary prevention for RHD has been recently re-emphasised, and new data are available regarding echocardiographic screening for subclinical RHD and initiation of secondary prevention. There is an urgent need for comprehensive service frameworks to improve access and level of care and services for patients, educational programmes to reinforce the importance of prevention and early diagnosis and a relevant research agenda focusing on the African context.

Variability in disease burden and management of rheumatic fever and rheumatic heart disease in two regions of tropical Australia.

BACKGROUND: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) contribute to Aboriginal Australian and Torres Strait Islander health disadvantage. At the time of this study, specialist ARF/RHD care in the Kimberley region of Western Australia was delivered by a broad range of providers. In contrast, in Far North Queensland (FNQ), a single-provider model was used as part of a coordinated RHD control programme. AIMS: To review ARF/RHD management in the Kimberley and FNQ to ascertain whether differing models of service delivery are associated with different disease burden and patient care. METHODS: An audit of ARF/RHD management. Classification and clinical management data were abstracted from health records, specialist letters, echocardiograms and regional registers using a standardised data collection tool. RESULTS: Four hundred and seven patients were identified, with 99% being Aboriginal and/or Torres Strait Islanders. ARF without RHD was seen in 0.4% of Aboriginal and/or Torres Strait Islander residents and RHD in 1.1%. The prevalence of RHD was similar in both regions but with more severe disease in the Kimberley. More FNQ RHD patients had specialist review within recommended time frames (67% vs 45%, χ(2) , P < 0.001). Of patients recommended benzathine penicillin secondary prophylaxis, 17.7% received ≥80% of scheduled doses in the preceding 12 months. Prescription and delivery of secondary prophylaxis was greater in FNQ. CONCLUSIONS: FNQ's single-provider model of specialist care and centralised RHD control programme were associated with improved patient care and may partly account for the fewer cases of severe disease and reduced surgical procedures and other interventions observed in this region.

Acute rheumatic fever and rheumatic heart disease--priorities in prevention, diagnosis and management. A report of the CSANZ Indigenous Cardiovascular Health Conference, Alice Springs 2011.

Three priority areas in the prevention, diagnosis and management of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) were identified and discussed in detail: 1. Echocardiography and screening/diagnosis of RHD ­ Given the existing uncertainty it remains premature to advocate for or to incorporate echocardiographic screening for RHD into Australian clinical practice. Further research is currently being undertaken to evaluate the potential for echocardiography screening. 2. Secondary prophylaxis ­ Secondary prophylaxis (long acting benzathine penicillin injections) must be seen as a priority. Systems-based approaches are necessary with a focus on the development and evaluation of primary health care-based or led strategies incorporating effective health information management systems. Better/novel systems of delivery of prophylactic medications should be investigated. 3. Management of advanced RHD ­ National centres of excellence for the diagnosis, assessment and surgical management of RHD are required. Early referral for surgical input is necessary with multidisciplinary care and team-based decision making that includes patient, family, and local health providers. There is a need for a national RHD surgical register and research strategy for the assessment, intervention and long-term outcome of surgery and other interventions for RHD.

Dynamic assessment of 'valvular reserve capacity' in patients with rheumatic mitral stenosis.

AIMS: Mitral stenosis (MS) may exhibit a dynamic valvular reserve. When resting gradients and systolic pulmonary pressure (sPAP) do not reflect the real severity of the disease, a dynamic evaluation becomes necessary. The aim of the study was to assess the clinical utility of exercise echocardiography in symptomatic patients with apparently subcritical MS. METHODS AND RESULTS: One hundred and thirty consecutive patients were referred for symptomatic MS. Patients with unimpressive resting MVA (>1-1.5 cm(2)) and mean PG (≥5-9 mmHg) underwent exercise echocardiography. Cardiac performance and mitral indices (MVA, peak/mean PG, sPAP) were measured. Exhaustion of valvular reserve capacity under exercise was defined as appearance of symptoms and sPAP > 60 mmHg. Forty-six patients (35%) (age: 53 ± 10 years; 74%, female) with resting MVA (1.2 ± 0.36 cm(2)), mean PG (6.8 ± 2.7 mmHg), and sPAP (38 ± 7 mmHg) inconsistent with symptoms underwent stress echocardiography. Exercise was stopped for dyspnoea (76%) or fatigue (24%). At peak workloads (57.2 ± 21.8 Watts), increased mean PG (17.2 ± 4.8 mmHg, P< 0.001) and sPAP (67.4 ± 11.4 mmHg; P< 0.0001) were observed, without change in MVA (1.25 ± 0.4 cm(2); P= n.s.). At univariate analysis, predictors of adaptation to exercise were age (-0.345; P = 0.024), mean PG (0.339; P= 0.023), and sPAP (0.354; P= 0.024); at multivariate analysis, best predictor was resting mean PG, although correlation was poor (-0.339; P= 0.015). CONCLUSION: In MS with limiting symptoms despite unimpressive findings at rest, valvular capacity exhaustion should be tested on a dynamic background, as no single resting index can predict potential haemodynamic adaptation to exercise. In such context, the contribution of exercise echocardiography remains extremely valuable.

Rheumatic heart disease in indigenous populations.

Rates of acute rheumatic fever and chronic rheumatic heart disease in Aboriginal people, Torres Strait Islanders and Maori continue to be unacceptably high. The impact of rheumatic heart disease is inequitable on these populations as compared with other Australians and New Zealanders. The associated cardiac morbidity, including the development of rheumatic valve disease, and cardiomyopathy, with possible sequelae of heart failure, development of atrial fibrillation, systemic embolism, transient ischaemic attacks, strokes, endocarditis, the need for interventions including cardiac surgery, and impaired quality of life, and shortened life expectancy, has major implications for the individual. The adverse health and social effects may significantly limit education and employment opportunities and increase dependency on welfare. Additionally there may be major adverse impacts on family and community life. The costs in financial terms and missed opportunities, including wasted young lives, are substantial. Prevention of acute rheumatic fever is dependent on the timely diagnosis and treatment of sore throats and skin infections in high-risk groups. Both Australia and New Zealand have registries for acute rheumatic fever but paradoxically neither includes all cases of chronic rheumatic heart disease many of whom would benefit from close surveillance and follow-up. In New Zealand and some Australian States there are programs to give secondary prophylaxis with penicillin, but these are not universal. Surgical outcomes for patients with rheumatic valvular disease are better for valve repair than for valve replacement. Special attention to the selection of the appropriate valve surgery and valve choice is required in pregnant women. It may be necessary to have designated surgical units managing Indigenous patients to ensure high rates of surgical repair rather than valve replacement. Surgical guidelines may be helpful. Long-term follow-up of the outcomes of surgery in Indigenous patients with rheumatic heart disease is required. Underpinning these strategies is the need to improve poverty, housing, education and employment. Cultural empathy with mutual trust and respect is essential. Involvement of Indigenous people in decision making, design, and implementation of primary and secondary prevention programs, is mandatory to reduce the unacceptably high rates of rheumatic heart disease.