BACKGROUND: Rheumatic heart disease (RHD) continues to pose a significant burden on global health, particularly in socioeconomically disadvantaged populations. We present the case of a 38-year-old woman with severe multivalvular RHD and giant atria, highlighting the challenges and complexities of managing this condition.
METHODS: The patient presented with progressively worsening dyspnoea, signs of right heart failure, and severe valvular abnormalities. Diagnostic evaluations revealed severe mixed mitral valve disease in the form of mitral stenosis and regurgitation, along with involvement of the aortic and tricuspid valves, leading to significant enlargement of both atria. Despite facing socioeconomic constraints and poor adherence to treatment, the patient underwent successful surgical intervention, resulting in remarkable symptomatic improvement.
CONCLUSIONS: Through this case, we emphasise the importance of early detection, comprehensive management strategies, and multidisciplinary care in addressing the complexities of RHD. Despite the challenges posed by socioeconomic disparities, positive outcomes can be achieved with timely diagnosis and appropriate intervention. This case underscores the need for targeted efforts to improve access to healthcare resources and reduce the global burden of RHD.

BACKGROUND: Rheumatic heart disease is a global health concern with a persistently high incidence in developing countries, including Africa. It has a significant economic, morbidity, and mortality burden, particularly for children and young adults during their most productive years. However, in the last ten years, the extent of its impact in Africa has remained unclear. Limited studies conducted on the continent have reported diverse prevalence rates of rheumatic heart disease. As a result of these, the study aimed to aggregate and synthesize findings from population-based studies to offer a comprehensive and updated overview of rheumatic heart disease prevalence and pattern at the African level.
METHODS: The studies were identified through a comprehensive literature search of the electronic databases, including PubMed, Google Scholar, Web searches, and manual searches. The descriptive information for the study is presented in the table, and the quantitative results are presented in forest plots. The Cochrane Q test and I2 test statistic were used to test heterogeneity across studies. The pooled estimate of the prevalence of rheumatic heart disease was computed by a random effects model.
RESULTS: Out of 22 population-based studies analyzed using random-effects, the pooled magnitude of rheumatic heart disease was found to be 18.41/1000 (95% CI: 14.08-22.73/1000). This comprised definite cases of rheumatic heart disease at a prevalence rate of 8.91/1000 (95% CI: 6.50-11.33/1000) and borderline cases at a prevalence rate of 10.69/1000 (95% CI: 7.74-13.65/1000). The combined prevalence of rheumatic heart disease in males was almost equivalent to that in females. Mitral valve regurgitation was the predominant valve affected by rheumatic heart disease, accounting for approximately 73%.
CONCLUSIONS: This study analysis found the prevalence of rheumatic heart disease in Africa is high. Because of this, policies and interventions should give attention to prioritize continuous population based active surveillance for early detection of cases to the reduction of rheumatic heart disease sequel, especially in the children and adolescent population.

UNASSIGNED: Rheumatic heart disease (RHD) poses a substantial global health challenge, especially impacting resource-limited nations, with over 40.5 million cases reported in 2019. The crucial role of Benzathine penicillin G in both primary and secondary prevention, particularly the latter, emphasizes its significance.
UNASSIGNED: Following PRISMA guidelines, our systematic review explored Medline, Scopus, Google Scholar, and Embase databases from 1990 to 2022. Registered with PROSPERO ), the review utilized quality appraisal tools, including the PRISMA checklist, Cochrane bias tool and Newcastle-Ottawa scale. The objective was to identify and stratify the impact of socio-economic factors on adherence to secondary prophylaxis in RHD.
UNASSIGNED: The impact of education on adherence has been found to be significant. Socially disadvantaged environments significantly influenced adherence, shaped by education, socio-economic status, and geographical location and access to healthcare. Surprisingly, lower education levels were associated with better adherence in certain cases. Factors contributing to decreased adherence included forgetfulness, injection-related fears, and healthcare provider-related issues. Conversely, higher adherence correlated with younger age, latent disease onset, increased healthcare resources, and easy access.
UNASSIGNED: Patient education and awareness were crucial for improving adherence. Structured frameworks, community initiatives, and outreach healthcare programs were identified as essential in overcoming barriers to secondary prophylaxis. Taking active steps to address obstacles like long-distance commute, waiting time, injection fears, and financial issues has the potential to greatly improve adherence. This, in turn, can lead to a more effective prevention of complications associated with RHD.

Lateral medullary syndrome, resulting from cerebellar/brainstem infarction, can occur due to cardioembolic stroke from atrial fibrillation caused by rheumatic heart disease. This rare association highlights the importance of strict arrhythmia management, prophylactic anticoagulation, and timely diagnosis to prevent debilitating neurological outcomes.

Rheumatic heart disease (RHD) is a chronic complication arising from acute rheumatic fever (ARF), an autoimmune response triggered by group A streptococcal infection. It primarily affects children and young adults in developing countries. RHD continues to show substantial global heterogeneity. Socioeconomic factors lead the virtual disappearance of RHD in industrialized countries with the introduction of penicillin. By contrast, RHD is still endemic in Africa, Asia, South America, and developing communities of Australasia. We provide an estimate of the current prevalence of latent RHD in Africa using the echocardiographic diagnostic criteria of the World Heart Federation (WHF). Systematic review and meta-analysis of 21 studies reporting the prevalence of RHD, encompassing 40.639 patients. Estimated prevalence of RHD was 25.5 cases per 1000 population (P ≤0.02; 95 % CI, 18.1-32.9 per 1000): definite RHD 13.1 cases per 1000 population (95 % CI, 7.7-18.5 per 1000): and borderline 12,4 cases per 1000 population (95 % CI, 7.7-17.0 per 1000). The prevalence of definite RHD was a significantly higher in adults (M = 28.2, SD = 6.1) compared to children (M = 10.3, SD = 9.2), t(17) = 2.6, p = .0179. Prevalence of definite RHD in schools was 7,92 cases per 1000 population (95 % CI, 4,49-11,35 per 1000) and in community was 26,17 cases per 1000 population (95 % CI, 12,27-40,06 per 1000). This meta-analysis may have produced a better estimate of the prevalence of RHD in Africa using only studies performed according to the 2012 WHF, and clearly showed the high prevalence of RHD in the community and in adults.

The World Heart Federation (WHF) published the first evidence-based guidelines on the echocardiographic diagnosis of rheumatic heart disease (RHD) in 2012. These guidelines have since been applied internationally in research and clinical practice. Substantial research has assessed the utility of the 2012 WHF criteria, including its applicability in low-resource settings. This article summarises the evidence regarding the performance of the guidelines.
A scoping review assessing the performance of the guidelines was performed. Cochrane, Embase, Medline, PubMed Lilacs, Sielo, and Portal BVS databases were searched for studies on the performance of the guidelines between January 2012-March 2023, and 4047 manuscripts met the search criteria, of which 34 were included. This included papers assessing the specificity, inter-rater reliability, application using hand-carried ultrasound, and modification of the criteria for simplicity. The review followed the PRISMA Extension for Scoping Reviews guideline.
The WHF 2012 criteria were 100% specific for definite RHD when applied in low-prevalence populations. The criteria demonstrated substantial and moderate inter-rater reliability for detecting definite and borderline RHD, respectively. The inter-rater reliability for morphological features was lower than for valvular regurgitation. When applied to hand-carried ultrasound performed by an expert, modified versions of the criteria demonstrated a sensitivity and specificity range of 79-90% and 87-93% respectively for detecting any RHD, performing best for definite RHD. The sensitivity and the specificity were reduced when performed in task-sharing but remains moderately accurate.
The WHF 2012 criteria provide clear guidance for the echocardiographic diagnosis of RHD that is reproducible and applicable to a range of echocardiographic technology. Furthermore, the criteria are highly specific and particularly accurate for detecting definite RHD. There are limitations in applying all aspects of the criteria in specific settings, including task-sharing. This summary of evidence can inform the updated version of the WHF guidelines to ensure improved applicability in all RHD endemic regions.

BACKGROUND: Early detection and diagnosis of acute rheumatic fever and rheumatic heart disease are key to preventing progression, and echocardiography has an important diagnostic role. Standard echocardiography might not be feasible in high-prevalence regions due to its high cost, complexity, and time requirement. Handheld echocardiography might be an easy-to-use, low-cost alternative, but its performance in screening for and diagnosing acute rheumatic fever and rheumatic heart disease needs further investigation.
METHODS: In this systematic review and meta-analysis, we searched Embase, MEDLINE, LILACS, and Conference Proceedings Citation Index-Science up to Feb 9, 2024, for studies on the screening and diagnosis of acute rheumatic fever and rheumatic heart disease using handheld echocardiography (index test) or standard echocardiography or auscultation (reference tests) in high-prevalence areas. We included all studies with useable data in which the diagnostic performance of the index test was assessed against a reference test. Data on test accuracy in diagnosing rheumatic heart disease, acute rheumatic fever, or carditis with acute rheumatic fever (primary outcomes) were extracted from published articles or calculated, with authors contacted as necessary. Quality of evidence was appraised using GRADE and QUADAS-2 criteria. We summarised diagnostic accuracy statistics (including sensitivity and specificity) and estimated 95% CIs using a bivariate random-effects model (or univariate random-effects models for analyses including three or fewer studies). Area under the curve (AUC) was calculated from summary receiver operating characteristic curves. Heterogeneity was assessed by visual inspection of plots. This study was registered with PROSPERO (CRD42022344081).
RESULTS: Out of 4868 records we identified 11 studies, and two additional reports, comprising 15 578 unique participants. Pooled data showed that handheld echocardiography had high sensitivity (0·87 [95% CI 0·76-0·93]), specificity (0·98 [0·71-1·00]), and overall high accuracy (AUC 0·94 [0·84-1·00]) for diagnosing rheumatic heart disease when compared with standard echocardiography (two studies; moderate certainty of evidence), with better performance for diagnosing definite compared with borderline rheumatic heart disease. High sensitivity (0·79 [0·73-0·84]), specificity (0·85 [0·80-0·89]), and overall accuracy (AUC 0·90 [0·85-0·94]) for screening rheumatic heart disease was observed when pooling data of handheld echocardiography versus standard echocardiography (seven studies; high certainty of evidence). Most studies had a low risk of bias overall. Some heterogeneity was observed for sensitivity and specificity across studies, possibly driven by differences in the prevalence and severity of rheumatic heart disease, and level of training or expertise of non-expert operators.
CONCLUSIONS: Handheld echocardiography has a high accuracy and diagnostic performance when compared with standard echocardiography for diagnosing and screening of rheumatic heart disease in high-prevalence areas.
BACKGROUND: World Health Organization.
UNASSIGNED: For the Chinese, French, Italian, Persian, Portuguese, Spanish and Urdu translations of the abstract see Supplementary Materials section.

Rheumatic heart disease remains a major cause of cardiovascular death worldwide. Limited real-world nationwide data are available to compare the long-term outcomes between mitral valve repair and replacement in rheumatic heart disease. For patients with RHD, MVP is the superior choice of surgical intervention owing to better long-term survival, reduced incidence of early mortality and thromboembolic events. However, it entails higher chances of re-operation at follow-up at four, eight and twelve years. Although feasible, surgeons may opt for MVR in patients with a worse prognosis. Whereas degenerative mitral repair for severe MR has been proven superior to replacement, the optimal operative strategy for mitral RHD remains unclear. In developing countries, mitral RHD commonly develops in young patients, predominantly consists of MR rather than MS, and occurs more frequently than in the United States. In addition, the predominant MR etiology (rather than MS), relatively early intervention in the RHD timeline, and variation in Carpentier MR types among developing world populations further make these rheumatic MVs more amenable to repair than replacement. Patients should be carefully selected for mitral valve repair because of its higher reoperation rate, particularly those with previous percutaneous transvenous mitral commissurotomy. Careful assessment of anterior leaflet mobility/calcification to determine mitral repair or replacement was associated with improved outcomes. This decision-making strategy may alter the threshold for rheumatic mitral replacement in the current valve-in-valve era.

Secondary prevention with penicillin aims to prevent further episodes of acute rheumatic fever and subsequent development of rheumatic heart disease (RHD). Penicillin allergy, self-reported by 10% of the population, can affect secondary prevention programs. We aimed to assess the role for (i) routine penicillin allergy testing and the (ii) safety of penicillin allergy delabeling approaches in this context. We searched MEDLINE, Embase, CENTRAL, ClinicalTrials.gov, WHO ICTRP, ISRCTN, and CPCI-S to identify the relevant reports. We found 2419 records, but no studies addressed our initial question. Following advice from the WHO-Guideline committee and experts, we identified 6 manuscripts on allergy testing focusing on other populations showing that the prevalence of allergy confirmed by testing was low and the incidence of life-threatening reactions to BPG was very low (< 1-3/1000 individuals treated). A subsequent search addressed penicillin allergy delabeling. This found 516 records, and 5 studies addressing the safety of direct oral drug challenge vs. skin testing followed by drug administration in patients with suspected penicillin allergy. Immediate allergic reactions of minor severity were observed for a minority of patients and occurred less frequently in the direct drug challenge group: 2.3% vs. 11.5%; RR = 0.25, 95%CI 0.15-0.45, P < 0.00001, I2 = 0%. No anaphylaxis or deaths were observed. Severe allergic reactions to penicillin are extremely rare and can be recognized and dealt by trained healthcare workers. Confirmation of penicillin allergy diagnosis or delabeling using direct oral drug challenge or penicillin skin testing seems to be safe and is associated with a low rate of adverse reactions.

Acute rheumatic fever-related acquired heart disease in children and young adults is a worldwide issue, but it poses a serious public health threat in developing nations like India. We present a case report of a young adult who collapsed suddenly during a routine walk and was declared dead in an emergency ward on arrival. There was no significant past medical history. On autopsy, massive cardiomegaly and a \"butter and bread\" appearance of the pericardium, along with findings suggestive of mitral stenosis and aortic regurgitation, raised suspicion about rheumatic heart disease. Rheumatic carditis was confirmed by a microscopic examination that showed Aschoff bodies in the left ventricle, mitral valve and interventricular septum, in addition to uncommon Anitschkow cells in the left ventricle. A sudden death due to undiagnosed rheumatic carditis causing such massive cardiomegaly is rare. This case highlights the need to keep rheumatic carditis as a cause of sudden death.