Progression Of Rheumatic Heart Disease Research Articles

Elevated Trans-mitral Pressure Gradient Predicts Surgery in Young People with Moderate-Severe Rheumatic Mitral Regurgitation.

Mitral regurgitation (MR) is the most common lesion in children with rheumatic heart disease (RHD). Progression of RHD results in the need for surgical intervention, the timing of which is dictated by left ventricular dilatation and the onset of heart failure symptoms. We sought to determine whether elevation in trans-mitral pressure gradient (TMPG) in those with moderate or severe rheumatic MR without significant mitral stenosis (MS) could predict the need for future surgical intervention. Echocardiographic studies were reviewed for 116 children and young people with moderate or severe rheumatic MR. Those with significant mitral stenosis or concurrent aortic valve disease were excluded. Trans-mitral pressure gradient was measured at baseline and details of mitral valve surgical intervention were retrieved from a registry database. Time to future surgery (up to six years) was compared between those with TMPG < 5mmHg and TMPG ≥ 5mmHg. Survival curves demonstrated an increased risk of surgery for those with TMPG ≥ 5mmHg with Cox proportional regression analysis providing a hazard ratio of 5.8. The proportion free from mitral valve surgery at one year for the TMPG < 5mmHg group was 0.94 (95% CI 0.86-0.97), compared to 0.62 (95% CI 0.42-0.77) in the ≥ 5mmHg group. Trans-mitral pressure gradient is a strong predictor of future mitral valve surgery in children and young people with significant rheumatic MR without MS. This non-invasive measure could be used to signal the need for more aggressive monitoring in order to optimize the timing of intervention.

Secondary prophylaxis and specialist review compliance in paediatric patients with acute rheumatic fever and rheumatic heart disease in a Western Australian metropolitan setting.

Rheumatic heart disease (RHD), a consequence of acute rheumatic fever (ARF), continues to cause significant morbidity and mortality in susceptible young people. Secondary antibiotic prophylaxis is an effective strategy to prevent ARF recurrence and RHD progression. However, the proportion of patients receiving the recommended minimum benzathine penicillin injections (>80%) remains low. This retrospective cohort study reviews the utility of the Hospital in the Home nursing service in providing secondary prophylaxis for outpatients in a metropolitan state capital. Fifty-eight patients with ARF/RHD receiving secondary prophylaxis through the Hospital in the Home service between 1 July 2012 and 30 June 2020 were included. Compliance of secondary prophylaxis, specialist review and echocardiogram frequency were compared against the recommendations from the Australian RHD guidelines. Forty-six (79%) patients received >80% of recommended doses, with 36% (n = 21) receiving 100% and a further 29% (n = 17) achieving compliance of 90%-99%. We found that 35% of patients attended all specialist reviews (including echocardiography) at the recommended frequency, with 75% of those not attending all reviews, missing only one or two appointments. Compliance was greater in those with more severe disease. In the 12 (21%) patients who did not meet the >80% target, compliance ranged between 33% and 79% (mean 64.5%) and all had priority 3 disease. Community-based administration of secondary prophylaxis through a registry-based system is an effective strategy of improving compliance within a metropolitan area and should be utilised in a culturally safe and collaborative manner to increase uptake.

Intramuscular versus enteral penicillin prophylaxis to prevent progression of rheumatic heart disease: Study protocol for a noninferiority randomized trial (the GOALIE trial)

Rheumatic Heart Disease (RHD) persists as a major cardiovascular driver of mortality and morbidity among young people in low-and middle-income countries. Secondary antibiotic prophylaxis (SAP) with penicillin remains the cornerstone of RHD control, however, suboptimal treatment adherence undermines most secondary prevention programs. Many of the barriers to optimal SAP adherence are specific to the intramuscular form of penicillin and may potentially be overcome by use of oral penicillin. This noninferiority trial is comparing the efficacy of intramuscular to oral penicillin SAP to prevent progression of mild RHD at 2 years. The Intramuscular vs Enteral Penicillin Prophylaxis to Prevent Progression of Rheumatic Heart Disease (GOALIE) trial is randomizing Ugandan children aged 5 to 17 years identified by echocardiographic screening with mild RHD (Stage A or B as defined by 2023 World Heart Federation criteria) to Benzathine Benzyl Penicillin G (BPG arm, every-28-day intramuscular penicillin) or Phenoxymethyl Penicillin (Pen V arm, twice daily oral penicillin) for a period of 2 years. A blinded echocardiography adjudication panel of 3 RHD experts and 2 cardiologists is determining the echocardiographic stage of RHD at enrollment and will do the same at study completion by consensus review. Treatment adherence and study retention are supported through peer support groups and case management strategies. The primary outcome is the proportion of children in the Pen V arm who progress to more advanced RHD compared to those in the BPG arm. Secondary outcomes are patient-reported outcomes (treatment acceptance, satisfaction, and health related quality of life), costs, and cost-effectiveness of oral compared to intramuscular penicillin prophylaxis for RHD. A total sample size of 1,004 participants will provide 90% power to demonstrate noninferiority using a margin of 4% with allowance for 7% loss to follow-up. Participant enrollment commenced in October 2023 and final participant follow-up is expected in December 2026. The graphical abstract (Fig. 1) summarizes the flow of echocardiographic screening, participant enrollment and follow-up. The GOALIE trial is critical in global efforts to refine a pragmatic approach to secondary prevention for RHD control. GOALIE insists that the inferiority of oral penicillin be proven contemporarily and against the most important near-term clinical outcome of progression of RHD severity. This work also considers other factors that could influence the adoption of oral prophylaxis and change the calculus for acceptable efficacy including patient-reported outcomes and costs. ClinicalTrials.gov: NCT05693545.

Effectivity and adherence of secondary prevention on clinical outcome of rheumatic heart disease in high-burden setting: a systematic review and meta-analysis

Abstract Background Rheumatic heart disease (RHD) is often diagnosed late in low-resource settings due to limited health-seeking behavior and varied presentations. Optimal delivery of regular antibiotics prescribed as secondary prevention is vital to preventing disease progression. However, poor adherence of secondary prevention remains a significant challenge to these populations. Objective To assess the efficacy and adherence of patients to secondary prevention of rheumatic heart disease. Methods This systematic review and meta-analysis was carried out according to the PRISMA statement. Literature search was performed on four databases using keywords "Rheumatic heart disease", "secondary prevention", and their synonyms. Adherence and disease progression were the primary outcome. The overall proportion was estimated with R software (version 4.1.3) using a random-effects model of proportional meta-analysis. Results Thirteen studies were included in this study. Benzathine Penicillin G (BPG) injection every 4 weeks was the most common secondary prophylaxis regimen used. In 2052 patients, only 46% (27-66%) patients received &amp;gt;80% BPG prophylaxis injection. The effectiveness of secondary prophylaxis was powerful, evidenced by only 7% (2-12%) patients having disease progression of rheumatic heart disease. Conclusions BPG was found to be effective in the secondary prevention of rheumatic heart disease. Nonetheless, patient adherence to injection remains extremely low. Methods to improve patient adherence are imminently required.Figure 1Figure 2

Seven year follow up of subclinical rheumatic disease using WHF criteria

Abstract Background The prevalence of rheumatic heart disease (RHD) is higher using echocardiographic screening compared with clinical examination. It is proven that clinical screening underestimates the prevalence, but it still remains the cornerstone for the purpose of intervention. Echocardiography can identify subclinical RHD and can be used for subclinical cases and to see progression /regression on follow up. Follow-up data of children with subclinical lesions are very much needed before implementing large scale screening programs in endemic regions. We are submitting the 7 year follow up data for subclinical RHD who were on RF prophylaxis. Method A total of 3000 school going children were screened echocardiographically during November 2015 to December 2018 for subclinical rheumatic heart disease using WHF criteria. Subjects with subclinical rheumatic heart disease were given prophylaxis for rheumatic fever in form of injection Benzathine penicillin or oral penicillin or tablet erythromycin depending upon tolerance and acceptance. These cases were followed up at 2-year and 7-year for echocardiographic evaluation and compliance to the prophylaxis. Results Out of 3000 cases screened, 114 were found to be having subclinical rheumatic heart disease. 39 subjects turned up for 2-year follow up and 13 subjects turned up for 7-year follow up(Table 1). All 13 were on rheumatic fever prophylaxis. 23% ( 3 out of 13) turned to be with normal echocardiography findings using WHF 2012 criteria. Limitation: Children who were not taking prophylaxis were not evaluated echocardiographically, therefore comparison could not be done. Conclusion Our study shows screening of school going children by echocardiography can detect subclinical rheumatic heart disease and rheumatic fever prophylaxis in such cases can results in reversion of the lesion and prevention of progression of RHD.

Plausible Role of NLRP3 Inflammasome and Associated Cytokines in Pathogenesis of Rheumatic Heart Disease.

Rheumatic heart disease (RHD) is a post-streptococcal sequela caused by Streptococcus pyogenes. The global burden of disease is high among people with low socio-economic status, with significant cases emerging every year despite global eradication efforts. The current treatment includes antibiotic therapies to target strep throat and rheumatic fever and valve replacement strategies as a corrective measure for chronic RHD patients. Valvular damage and valve calcification are considered to be the end-stage processes of the disease resulting from impairment of the endothelial arrangement due to immune infiltration. This immune infiltration is mediated by a cascade of events involving NLRP3 inflammasome activation. NLRP3 inflammasome is activated by wide range of stimuli including bacterial cell wall components like M proteins and leukocidal toxins like nicotinamide dehydrogenase (NADase) and streptolysin O (SLO) and these play a major role in sustaining the virulence of Streptococcus pyogenes and progression of RHD. In this review, we are discussing NLRP3 inflammasome and its plausible role in the pathogenesis of RHD by exploiting the host-pathogen interaction mainly focusing on the NLRP3 inflammasome-mediated cytokines IL-1β and IL-18. Different therapeutic approaches involving NLRP3 inflammasome inactivation, caspase-1 inhibition, and blockade of IL-1β and IL-18 are discussed in this review and may be promising for treating RHD patients.

Abstract 11455: Outcomes of Latent Rheumatic Heart Disease: External Validation of a Simplified Score in Patients With And Without Secondary Prophylaxis

Background: Secondary antibiotic prophylaxis reduces progression in children with latent rheumatic heart disease (RHD). However, nearly half of children show improvement with or without prophylaxis. Improved risk stratification could refine prophylaxis recommendations following positive screening echo. We aimed to validate a previously developed echo risk score to predict mid-term RHD outcomes among children with latent RHD. Methods: Our population included children who completed the GOAL Trial, a randomized controlled trial of secondary antibiotic prophylaxis among children with latent RHD in Uganda. These children underwent echo at enrollment and study completion, with rigorous blinded adjudication by a 4-member panel to establish diagnosis. We applied a point-based risk score developed from prior Brazilian and Ugandan cohorts, consisting of 5 variables (mitral valve (MV) anterior leaflet thickening (3 points), MV excessive leaflet tip motion (3), MV regurgitation jet length ≥2 cm (6), aortic valve focal thickening (4) and any aortic regurgitation (5)) to predict unfavorable outcome, defined as progression of diagnostic category (borderline to definite, definite mild valvular disease to definite moderate/severe disease) or remaining with definite RHD. Results: At total 801 patients (626 borderline and 175 definite RHD, mean age 12.5±2.9 years) were included, with median follow-up of 24 months. RHD progression was observed in 3 patients in the Penicillin group, vs. 33 in the control group, and overall, 386 patients regressed and 377 remained stable. The echo score was strongly associated with unfavorable outcome (OR: 1.25, 95% CI 1.15 - 1.36, p&lt;0.001). Unfavorable outcome rates in low (≤6 points), intermediate (7 - 9) and high-risk (≥10) children at follow-up were 12.2%, 31.1%, and 42.2%, (p&lt;0.001) respectively, with area under the ROC curve of 0.64 (95% CI 0.59 - 0.69). Among children without prophylaxis, the score had a similarly good performance: OR: 1.24 (95% CI 1.12 - 1.39), area under the ROC curve: 0.64 (95% CI 0.58 - 0.70). Conclusions: The simple risk score provided an accurate prediction of RHD status at 2-years, showing a good performance in a population with milder RHD phenotypes, with a potential value for global risk stratification.

Abstract 12284: Utilizing Automated Machine Learning for Rheumatic Heart Disease Detection in Doppler Echocardiography

Introduction: Rheumatic heart disease (RHD) is the number one cause globally of morbidity and mortality from heart disease in children and young adults. The mitral regurgitation (MR) jet length on color Doppler echocardiography is an important index for diagnosis, but its measurement and interpretation vary. Objective: Develop an automatic machine learning approach to identify and measure the MR jet length on color Doppler for RHD detection. Methods: We used 316 echocardiograms from 95 children (mean age 12±2 years; range 5 to 17 years) with DICOM color Doppler images of the mitral valve taken from parasternal long axis (PLAX) and apical 4 chamber (AP4) views. All echocardiograms were independently reviewed by an adjudication panel consisting of four expert pediatric cardiologists to determine maximum MR jet length and diagnosis (RHD or normal). Among 95 cases, 29 were normal and 66 had RHD. Our automated method included. (1) Selection of frames during ventricular systole using a convolutional neural network architecture. (2) Localization of left atrium using convolutional neural networks with LinkNet structure. (3) Measurement of MR jet length by image color analysis. (4) Detection of RHD by applying a generalized regression model based on the maximum MR jet length measured and maximizing the balanced accuracy using cross validation. Results: Machine learning selected the correct systolic frame with an average accuracy of 0.95 (sensitivity 97%/specificity 93%) and 0.94 (sensitivity 94%/specificity 94%) for the AP4 and PLAX view, respectively. It localized the atrium with an average Dice coefficient of 0.89 and 0.9 for the AP4 and PLAX view, respectively. We estimated the MR jet length with an average absolute error of 0.33±0.4 cm (p-value = 0.15 compared to manual measurements). Our deep learning approach performed similar to or better than previously published manual methods for categorization of RHD positive vs negative. The accuracy of RHD detection was 0.84 (sensitivity 86%/specificity 79%). Conclusions: Our automatic method has the potential to reliably detect RHD as accurately as expert cardiologists. This innovative approach holds promise to scale echocardiography screening for RHD and expand prophylaxis to prevent progression of RHD globally.

Lectin complement pathway components as risk factors for rheumatic heart disease in Assam, India

Lectin complement pathway is an integral component of the innate immune response and has been associated with pathogenesis of rheumatic heart disease (RHD). Thus, we investigated the serum concentration and single nucleotide polymorphism (SNP) in different components of the lectin pathway to understand their role in susceptibility and progression of RHD. Sixty RHD patients and equal number of age and sex matched healthy controls from Assam Medical College and Hospital (AMCH), Assam were enrolled for the study. We analyzed mannose binding lectin (MBL2), Ficolin2, MBL-associated serine protease 2 (MASP2) and MBL-associated protein 44 (MAp44) for serum concentration using ELISA along with identification of gene polymorphism using nucleotide sequencing. MBL2 SNP rs11003125 was analyzed in the representative North-East India cohort and the GG genotype [OR=; 95% CI=, p < 0.05)] was observed to impart a protective effect against RHD. Promoter region variants of ficolin2 (−4 A > G) [p = 0.0181, OR 6.5102 (1.3764–30.7931)] were observed in RHD patients. Further, low serum levels of MASP2 (patients- 641.16 pg/ml, ±288.21; controls-1168.71 pg/ml, ±300.03) and high levels of MAp44 (patients- 70 pg/ml, ±31.5; controls-63.3 pg/ml ±17.9) were observed. Based on our results, −4 A > G variant in promoter region of FCN2 is associated with increased susceptibility and low serum levels of Ficolin2 in RHD patients. Elevated MAp44 levels and low MASP2 serum levels in patients suggest inhibition of activation of complement system and thus less clearance of pathogen in RHD. Overall, our study adds to the understanding on the role of lectin pathway proteins in the predisposition and progression of RHD.

The pathophysiology of RHD and outstanding gaps

Rheumatic heart disease (RHD) is the major cause of cardiovascular morbidity and mortality in children and young adults in low- and middle-income countries. Acute rheumatic fever (ARF) is characterised by multiorgan inflammatory symptoms initiated through cross reaction of immune responses (IRs) to group A streptococcus (GAS) proteins to host proteins. Recurrence of these IRs targeting the heart valves may lead to permanent damage, a sequela which is termed RHD. Preliminary studies suggested genetic associations in RF reactions, but that other host factors are also involved, leaving the determinants of RHD progression incompletely understood. Previous clinical and recent epidemiological studies support differential clinical phenotypes, with varying history from different settings. This review summarises the protein-centric biomolecular changes in RHD and highlights outstanding molecular gaps where urgent focus is required to improve our understanding RHD pathophysiology. Numerous studies have confirmed alterations in the expression of structural and immune response proteins, but the modifications giving rise to neo-epitopes and their involvement in RHD have not been established. As RHD is associated with poor living conditions, identification of other factors driving inflammation to enhance RHD progression is necessary to advance our knowledge and improve patient management. Furthermore, biomarkers for early identification, disease stratification, and alternative therapeutic strategies are necessary to improve treatment and prevention strategies in order to reduce the burden of RHD. Relevance: Despite the explosion of scientific innovation over the last few decades, fundamental scientific studies to understand the pathophysiological mechanisms of RHD remain in their infancy and the determinants of RHD progression thus remain uncertain. Moreover, inconsistency in natural history and phenotypic presentations are seen between Africans and other cohorts in which preliminary studies were conducted, implying that differences in genetic complexity and environmental factors may be responsible for the differential disease progression rates.

The correlation between cytokine concentrations and IgG/IgM antibodies to viruses of the Herpesviridae family in children with rheumatic heart disease

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are still urgent medical issues in developing countries and in some communities of high-income countries. The aim of our study was to evaluate plasma cytokine concentrations in children with RHD and their correlation with antibody concentrations to viruses of the Herpesviridae family. Sixty-two patients with RHD aged 8 to 17 years were included in the study. The concentrations of TNF-α, IL-4, IL-8, IL-10 and IFN-γ were evaluated; IgG and IgM antibodies to cytomegalovirus (CMV), herpes simplex virus (HSV) groups I-II and Epstein-Barr virus (EBV) in serum were determined by ELISA method. A significant increase of cytokine concentrations (TNF-α, IL-8, IL-4 and IL-10) was recorded in RHD patients. Antibodies to intracellular Herpesviridae family viruses were detected in all children with RHD. Children with RHD were significantly more likely to have IgG HSV I-II antibodies, and IgG and IgM EBV antibodies compared to controls (p&lt;0.05). The concentration of IgM antibody to CMV showed positive correlation with TNF-α; the concentration of IgG antibody to EBV showed negative correlation with TNF-α; and the concentration of IgG and IgM antibodies to EBV showed positive correlation with IL-10 levels. Considering positive correlation of IL-10 concentration with IgG and IgM antibody concentrations to EBV, and the significance of IL-10 in the progression of RHD, the possible predisposing role of EBV in the progression of RHD can be postulated, which needs further study. Key words: child; cytokines ; Herpesviridae; rheumatic heart disease

Modelling rheumatic heart disease progression in australia using disease register data linked to administrative records

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Australian Government National Health and Medical Research Council OnBehalf ERASE project Background Rheumatic Heart Disease (RHD) is a major contributor to morbidity and mortality globally, and is endemic among Indigenous Australians. The RHD Endgame strategy was recently launched, outlining comprehensive methods for eliminating RHD in Australia by 2031. However, there is currently limited information on national rates of RHD and progression to severe or complicated RHD. Purpose This study provides current estimates of RHD progression prior to RHD Endgame Strategy implementation. We estimate the probability and predictors of progressing from RHD diagnosis to cardiovascular complications, death, or need for surgical intervention in the Australian population from expanded data sources, addressing methodological shortcomings in existing evidence by using cross-jurisdictional administrative datasets and a competing risks approach. Methods This retrospective cohort study used linked RHD register, hospital and death data from five Australian jurisdictions (&amp;gt;70% Australians). Progression from RHD diagnosis to all-cause mortality, non-fatal cardiovascular complications (heart failure, stroke, endocarditis, atrial fibrillation), or need for surgical intervention were estimated for people aged &amp;lt;35years diagnosed with first-ever RHD between 2010 and 2018. A minimum 8.5-year look-back excluded prevalent cases; maximum follow-up was 8 years. Proportional cause-specific hazard regression modelling investigated independent predictors of outcomes, with death treated as a competing risk. Sensitivity analyses compared results between all-sources and register-only cohorts. Results We identified 1714 first-ever RHD cases aged &amp;lt;35years in the all-sources cohort (84% Indigenous, 11% migrant, 63% women, 40% age 5-14years, 85% non-metropolitan). Six months after diagnosis, 8.1% (95%CI:6.9-9.5%) had experienced heart failure, other cardiovascular complications or surgical intervention and 23.6% (95%CI:20.2-27.5%) progressed to these outcomes within 8 years. The register-only cohort experienced less disease progression with estimated composite event rates of 5.6% (95%CI:4.7-6.6%) and 18.4% (95%CI:16.6-20.5%) at 6 month and 8 years respectively. Death rate in the all-sources cohort was 0.5% at 6 months and 3.2% at 8 years. Older age, Metropolitan residence, and history of acute rheumatic fever, but not sex or Indigenous status, were independent predictors of major cardiovascular outcomes. Conclusions This study provides the most definitive and contemporary estimates of RHD disease progression in young Australians. Despite Australia"s excellent healthcare system infrastructure, RHD complication rates remain high. Improvements in healthcare systems for diagnosis, monitoring, and management of RHD cases will need to be implemented in both Metropolitan and remote settings as Australia implements its Endgame strategy against RHD. However, primordial and primary prevention provides the best potential to reduce the burden of RHD in Australia and beyond.

Clonidine for pain-related distress in Aboriginal children on a penicillin regimen to prevent recurrence of rheumatic fever.

Indigenous children and adolescents in Australia and globally bear the burden of acute rheumatic fever (ARF). It has been virtually eliminated in well-resourced, developed settings. ARF is an autoimmune response to infection with group A Streptococcus. The mainstay of management is long-acting intramuscular penicillin injections to prevent recurrence of ARF and development of rheumatic heart disease (RHD), comprising valvular pathology and attendant complications. In Australia, penicillin injections are currently prescribed every 28 days for 5-10 years after diagnosis of ARF, depending on cardiac involvement. Adherence to this regimen reduces ARF recurrences and RHD progression. 'Days at risk' of ARF recurrence are calculated as the number of days after day 28 that an injection is not received. Adherence to the injection schedule has been reported as difficult in most global locations due to the painful nature of the injections, the long timeframes of the prescription, young age of patients, access problems and costs in some locations. The newly updated Australian guideline on the prevention, diagnosis and management of ARF and RHD has a chapter dedicated to secondary prophylaxis. This chapter takes into account cultural considerations and advises on ways to minimise pain and distress of injections in children such as pain gate strategies, distraction techniques and concurrent injection of local anaesthetic. Some children continue to find the injection regimen traumatising despite strategies to reduce pain and fear. Clinicians providing the injections to children also find the injecting episodes distressing if pain is not effectively minimised. An Aboriginal Community Controlled Health Service in a remote setting in northern Australia addressed the issue of severe trauma of injection episodes experienced by an Aboriginal boy aged 7 years. Usual strategies were not effective, so advice was sought from an expert anaesthetist at a tertiary hospital. As a result, oral clonidine 3 &micro;g/kg was trialled 45 minutes prior to the penicillin injection. Procedural coaching and monitoring protocols specific to administration of clonidine in children under their care were created by the health service. The initial dose of clonidine was delivered with the child as an inpatient. Clonidine was successful in reducing pain related distress and facilitating adherence to the penicillin regimen. Subsequent doses were delivered and monitored in a remote setting by nurses. After 18 months, the boy no longer required clonidine due to his increased coping capacity. A second child was recognised with similar trauma and has been taking clonidine for pre-procedural sedation for 6 months with good effect and no adverse effects. An additional child was similarly prescribed clonidine without success. Failure in that instance was attributed to lack of procedural coaching and receiving the initial dose of clonidine in an emergency department in hurried circumstances. Individualised child-focused and culturally appropriate care in remote settings is feasible: in this instance team planning for use of clonidine and procedural coaching when other measures have failed. However, for children with RHD, or other comorbidities, advice from the child's treating cardiologist is required prior to prescribing clonidine due to possible adverse consequences. These include hypotension and atrioventricular block, which could lead to haemodynamic compromise in the setting of moderate to severe RHD.

Prevalence and progression of rheumatic heart disease: a global systematic review and meta-analysis of population-based echocardiographic studies

This systematic review and meta-analysis aimed to provide a contemporaneous estimate of the global burden of rheumatic heart disease (RHD) from echocardiographic population-based studies. We searched multiple databases between January 01, 1996 and October 17, 2017. Random-effect meta-analysis was used to pool data. We included 82 studies (1,090,792 participant) reporting data on the prevalence of RHD and 9 studies on the evolution of RHD lesions. The pooled prevalence of RHD was 26.1‰ (95%CI 19.2–33.1) and 11.3‰ (95%CI 7.2–16.2) for studies which used the World Heart Federation (WHF) and World Health Organization (WHO) criteria, respectively. The prevalence of RHD varied inversely with the level of a country’s income, was lower with the WHO criteria compared to the WHF criteria, and was lowest in South East Asia. Definite RHD progressed in 7.5% (95% CI 1.5–17.6) of the cases, while 60.7% (95% CI 42.4–77.5) of cases remained stable over the course of follow-up. The proportion of cases borderline RHD who progressed to definite RHD was 11.3% (95% CI 6.9–16.5). The prevalence of RHD across WHO regions remains high. The highest prevalence of RHD was noted among studies which used the WHF diagnostic criteria. Definite RHD tends to progress or remain stable over time.

Public health and economic perspectives on acute rheumatic fever and rheumatic heart disease.

Public health and economic perspectives on acute rheumatic fever and rheumatic heart disease.

Relationship between Patient Understanding of Disease and Rheumatic Heart Disease Progression in American Samoa

Relationship between Patient Understanding of Disease and Rheumatic Heart Disease Progression in American Samoa

Relationship between Patient Understanding of Disease and Rheumatic Heart Disease Progression in American Samoa

Introduction: While mostly eradicated in developed nations, acute rheumatic fever (ARF) and its sequela, rheumatic heart disease (RHD), are still the leading causes of preventable cardiovascular disease in children and young adults. In particular, the population of American Samoa has a high prevalence of RHD at 12.9%. ARF is a delayed consequence of a pharyngeal infection with Group A β-hemolytic streptococci, causing high-pressure valvular regurgitation and later stenosis which can lead to cardiac failure and even death. When surgical valve repair is necessary for this population, families incur vast expenses to travel to the mainland United States due to …

Impact of Socio-Economic Factors on Increased Risk and Progression of Rheumatic Heart Disease in Developing Nations.

Rheumatic heart disease (RHD) is a global health concern especially in low-income settings. Morbidity and mortality data from the World Health Organization (WHO) and global burden studies emphasizes on the prioritization of RHD on a global platform. Genetic, environmental, and socio-economic factors determine the sustainability and progression of RHD in various populations. In developing countries, low socioeconomic status (SES) is a vast and inevitable challenge in combating RHD. Concurrence between low SES and RHD has been well documented by several studies, but there is a paucity of data to understand comprehensive interdependency of low SES and RHD. In this review, we have made an attempt to present the overall correlation between SES and increased risk of RHD by examining and highlighting the role of key components of SES in different populations throughout the world as reported in literature. In the recent past, developed countries have reported success stories regarding amelioration of RHD due to improved living conditions and better access to healthcare. Whereas, in low-income settings, various socio-economic parameters such as overcrowding, illiteracy, low monthly income, maternal employment, rural dwelling, and less access to good quality healthcare are the core challenges which significantly impose an increased risk of RHD. Overall, there is significant evidence which confirms the role of SES in increased risk and progression of RHD, but vigorous and systematic studies need to be done to evaluate the cumulative effect of SES. Also, it will be helpful in dissemination of efficient primary and secondary prevention of RHD. Additionally, another aspect of this review was to assess the plausible impact of low SES on the clinical spectrum of RHD which might characterize SES as an authoritative marker for disease progression and severity.

Two-year evolution of latent rheumatic heart disease in Malawi.

In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of "definite," "borderline," or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi. Our objective was to evaluate 2-year RHD evolution among those diagnosed through screening. Two-year follow-up echocardiograms of those diagnosed with latent RHD were read by a primary, secondary, then third reader if there was disagreement. Progression or regression of both definite and borderline groups were tabulated. Penicillin adherence, age, gender, number in home, and household income were compared between those with definite RHD who regressed to borderline and those that stayed definite. We utilized the local system used to track HIV defaulters in order to bring participants back into care. Comparisons were made using Fisher's exact and Wilcoxon rank-sum tests. Of the 39 with borderline RHD, 1 was lost to follow-up (2.6%), 1 progressed to definite (2.6%), 19 remained borderline (48.7%), 17 (43.6%) regressed to normal, and 1 was reclassified as mitral valve prolapse (2.6%). Of the 11 with definite RHD, 6 (54.5%) remained definite, 4 regressed to borderline (36.4%), and 1 regressed to normal (9.1%). Two of 11 with definite RHD had penicillin adherence above 80% for the 2-year follow-up period. There were no differences in adherence, gender, age, household income, or number in household between those with definite RHD that regressed to borderline and those who did not (P>.19). Borderlines had a very low progression rate to definite RHD. A strength of our study was a high retention rate (98%). Longer follow-up is needed to determine expected disease evolution.

PO631 Prevalence and Progression of Rheumatic Heart Disease: A Global Systematic Review and Meta-Analysis of Population-Based Echocardiographic Studies

PO631 Prevalence and Progression of Rheumatic Heart Disease: A Global Systematic Review and Meta-Analysis of Population-Based Echocardiographic Studies