Definite Rheumatic Heart Disease Research Articles

The Echocardiographic Diagnosis of Rheumatic Heart Disease: A Review of the Performance of the World Heart Federation Criteria 2012-2023.

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.

Agreement between Handheld and Standard Echocardiography for Diagnosis of Latent Rheumatic Heart Disease in Brazilian Schoolchildren from High-Prevalence Settings (Agreement between Screening and Standard Echo for RHD).

Introduction: Handheld echocardiography (echo) is the tool of choice for rheumatic heart disease (RHD) screening. We aimed to assess the agreement between screening and standard echo for latent RHD diagnosis in schoolchildren from an endemic setting. Methods: Over 14 months, 3 nonphysicians used handheld machines and the 2012 WHF Criteria to determine RHD prevalence in consented schoolchildren from Brazilian low-income public schools. Studies were interpreted by telemedicine by 3 experts (Brazil, US). RHD-positive children (borderline/definite) and those with congenital heart disease (CHD) were referred for standard echo, acquired and interpreted by a cardiologist. Agreement between screening and standard echo, by WHF subgroups, was assessed. Results: 1390 students were screened in 6 schools, with 110 (7.9%, 95% CI 6.5-9.5) being screen positive (14 ± 2 years, 72% women). Among 16 cases initially diagnosed as definite RHD, 11 (69%) were confirmed, 4 (25%) reclassified to borderline, and 1 to normal. Among 79 cases flagged as borderline RHD, 19 (24%) were confirmed, 50 (63%) reclassified to normal, 8 (10%) reclassified as definite RHD, and 2 had mild CHD. Considering the 4 diagnostic categories, kappa was 0.18. In patients with borderline RHD reclassified to non-RHD, the most frequent WHF criterion was B (isolated mitral regurgitation, 64%), followed by A (2 mitral valve morphological features, 31%). In 1 patient with definite RHD reclassified to normal, the WHF criterion was D (borderline RHD in aortic and mitral valves). After standard echo, RHD prevalence was 3.2% (95% CI 2.3-4.2). Conclusions: Although practical, RHD screening with handheld devices tends to overestimate prevalence.

Outcomes of possible and probable rheumatic fever: A cohort study using northern Australian register data, 2013-2019.

In Australia, there is a high burden of acute rheumatic fever (ARF) among Aboriginal and Torres Strait Islander peoples. Clinical diagnostic criteria can result in a diagnosis of 'definite', 'probable' or 'possible' ARF and outcomes range from recovery to severe rheumatic heart disease (RHD). We compared outcomes by ARF diagnosis, where the main outcome was defined as disease progression from: possible to probable ARF, definite ARF or RHD; probable to definite ARF or RHD; or definite ARF to definite ARF recurrence or RHD. Data were extracted from the Northern Territory RHD register for Indigenous Australians with an initial diagnosis of ARF during the 5.5-year study period (01/01/2013-30/06/2019). Descriptive statistics were used to describe cohort characteristics, probability of survival, and cumulative incidence risk of disease progression. Cox proportional hazards regression was used to determine whether time to disease progression differed according to ARF diagnosis. Sub-analyses on RHD outcome, clinical manifestations, and antibiotic adherence were also performed. In total there were 913 cases with an initial ARF diagnosis. Of these, 92 (13%) experienced disease progression. The probability of disease progression significantly differed between ARF diagnoses (p = 0.0043; log rank test). Cumulative incidence risk of disease progression at 5.5 years was 33.6% (95% CI 23.6-46.2) for definite, 13.5% (95% CI 8.8-20.6) for probable and 11.4% (95% CI 6.0-21.3) for possible ARF. Disease progression was 2.19 times more likely in those with definite ARF than those with possible ARF (p = 0.026). Progression to RHD was reported in 52/732 (7%) of ARF cases with normal baseline echocardiography. There was a significantly higher risk of progression from no RHD to RHD if the initial diagnosis was definite compared to possible ARF (p<0.001). These data provide a useful way to stratify risk and guide prognosis for people diagnosed with ARF and can help inform practice.

Outcomes of latent rheumatic heart disease: External validation of a simplified score in patients with and without secondary prophylaxis

BackgroundSecondary antibiotic prophylaxis reduces progression of latent rheumatic heart disease (RHD) but not all children benefit. Improved risk stratification could refine recommendations following positive screening. We aimed to evaluate the performance of a previously developed echocardiographic risk score to predict mid-term outcomes among children with latent RHD. MethodsWe included children who completed the GOAL, a randomized trial of secondary antibiotic prophylaxis among children with latent RHD in Uganda. Outcomes were determined by a 4-member adjudication panel. We applied the point-based score, consisting of 5 variables (mitral valve (MV) anterior leaflet thickening (3 points), MV excessive leaflet tip motion (3 points), MV regurgitation jet length ≥ 2 cm (6 points), aortic valve focal thickening (4 points) and any aortic regurgitation (5 points)), to panel results. Unfavorable outcome was defined as progression of diagnostic category (borderline to definite, mild definite to moderate/severe definite), worsening valve involvement or remaining with mild definite RHD. Results799 patients (625 borderline and 174 definite RHD) were included, with median follow-up of 24 months. At total 116 patients (14.5%) had unfavorable outcome per study criteria, 57.8% not under prophylaxis. The score was strongly associated with unfavorable outcome (HR = 1.26, 95% CI 1.16–1.37, p < 0.001). Unfavorable outcome rates in low (≤6 points), intermediate (7–9 points) and high-risk (≥10 points) children at follow-up were 11.8%, 30.4%, and 42.2%, (p < 0.001) respectively (C-statistic = 0.64 (95% CI 0.59–0.69)). ConclusionsThe simple risk score provided an accurate prediction of RHD status at 2-years, showing a good performance in a population with milder RHD phenotypes.

Leveraging School Health Programs in Africa: Integrated Screening for Rheumatic Heart Disease and Dental Caries.

Rheumatic heart disease (RHD) and dental caries (DC) disproportionately affect children and young adults in sub-Saharan countries, with major impact on schoolchildren's health and education. DC in children with RHD constitutes an important risk for fatal complications. Our study aimed at assessing the feasibility of simultaneous RHD and DC screening in school environment. March 20-24, 2022, we performed an observational descriptive study of schoolchildren in a public school in Maputo City, Mozambique. RHD screening involved two stages: first, a physical examination (including cardiac auscultation and direct observation of the oral cavity), and second, an abbreviated echocardiography performed by a cardiologist. Rapid testing for group A Streptococcus (GAS) was done to every eighth child in the classroom and for those with signs suggesting recent infection, in accordance with the study protocol developed for screening. A multidisciplinary team collected the data. Data were analyzed using descriptive statistics. A total of 954 students (median age 9; range 6-15) were screened. One hundred and twenty-five participants were eligible for a rapid antigen test, of which 6 (4.8%) tested positive. On clinical evaluation 52 children (5.3%) presented a heart murmur. Echocardiography on 362 children showed borderline RHD in 35 children and definite RHD in 2 (0.6%); 1 child had a ventricular septal defect. Dental cavities were present in 444 (48.4%), despite 904 out of 917 students reporting brushing of their teeth once to three times daily (98.6%). School-based integrated oral and cardiovascular screenings and use of rapid tests for GAS carriage provide crucial information to create customized preventive strategies for rheumatic fever (RF) and RHD in low- and middle-income countries (LMICs), in addition to detecting children at very high risk of bacterial endocarditis. The sustainability of such interventions and acceptability by health providers needs to be assessed.

Prognostic value of left atrial volume index in patients with rheumatic giant left atrium

Abstract Background Giant left atrium (GLA), conventionally defined as an anteroposterior diameter of more than 65 mm, is a rare condition mostly resulted from rheumatic mitral valve disease. Assessment of left atrial volume index (LAVI), in place of LA diameter, may have important prognostic implications in rheumatic GLA patients, incremental to clinical and additional imaging parameters. Purpose This study investigated whether initial assessment of LAVI is associated with short-term outcomes in rheumatic GLA patients who did not undergo surgery. Methods This was a single-center, prospective cohort study in 58 patients with definite rheumatic heart disease and echocardiographic-confirmed GLA who refused valve surgery and/or LA reduction procedure. Assessment of clinical, laboratory, echocardiography, and CT angiography thorax with contrast were performed on their first hospital admission. The study population was subdivided based on a LAVI value of 254 mL/m², following the prior ROC curve analysis. Both groups were followed for up to 17 months. The primary endpoint was all-cause mortality. The association between variables and all-cause mortality was analyzed using the Kaplan-Meier curve and Cox proportional hazard regression models. Results A total of 54 out of 58 patients (age 46 ± 11 years; 90.7% women) were included. At baseline, patients with LAVI ≥ 254 mL/m² exhibited significantly higher NT-proBNP level, reduced estimated glomerular filtration rate (eGFR), lower TAPSE, higher systolic pulmonary arterial pressure (sPAP), larger LA diameter, and more prone to atrial fibrillation and LA appendage aneurysm, rather than patients with LAVI &amp;lt; 254 mL/m² (all p&amp;lt;0.05). During a median follow-up of 12 (8–17) months, 11 (20.4%) patients died. Patients with LAVI ≥ 254 mL/m² demonstrated significantly higher mortality rates at 3-, 6-, and 12-months follow up (4%, 6%, and 16%, respectively) when compared with patients with LAVI &amp;lt; 254 mL/m² (2%, 2% and 4%, respectively, log-rank p=0.03). LA appendage aneurysm [hazard ratio (HR): 4.17; 95% confidence interval (CI): 1.15–6.52; p=0.035] and LAVI (HR: 1.07; 95% CI: 1.05–1.1; p=0.042) were independently associated with all-cause mortality. The addition of LAVI to a clinical model (including eGFR, NT-proBNP, LA diameter, cardiogenic shock, echo signs of chambers compression, and LA appendage aneurysm by CT) was associated with significant increase in the X² value (X² difference = 7.19; p&amp;lt;0.001), suggesting the incremental prognostic value of LAVI in patients with rheumatic GLA. Conclusions LAVI is independently associated with all-cause mortality in rheumatic GLA patients not undergoing surgery and provides incremental prognostic value over clinical and imaging variables for short-term survival. LAVI may therefore be useful in the risk stratification of patients with rheumatic GLA and optimize timing of surgical intervention.Baseline characteristics of the studyPrognostic value of LAVI on mortality

Abstract 14701: Agreement Between Handheld and Standard Echocardiography for Diagnosis of Latent Rheumatic Heart Disease in Brazilian Schoolchildren From High-Prevalence Settings

Background: Handheld echocardiography (echo) is the tool of choice for Rheumatic Heart Disease (RHD) screening, based on the 2012 World Heart Federation (WHF) Criteria. We aimed to assess the agreement between screening and standard echo for diagnosis of latent RHD in schoolchildren from an endemic setting. Methods: Over 14 months, 3 non-physicians used handheld machines (GE, VSCAN) and the 2012 WHF Criteria to determine RHD prevalence in low-income public schools in Minas Gerais, Southeast Brazil. Consented children were eligible for participation. Studies were interpreted remotely using a telemedicine cloud-server by 2 experts in Brazil and the US. RHD-positive children (borderline and definite) and those with congenital heart disease (CHD) were referred for standard echo with portable devices (GE, Vivid IQ), with acquisition and interpretation by an expert cardiologist. Agreement between screening and standard echo, by category and according to the WHD criteria, was assessed, and the kappa statistic was applied. Results: A total of 1,390 students were screened in 6 schools and, among these, 110 (7.9%, 95% CI 6.5 - 9.5) were screen-positive and referred for standard echo, with mean age of 14±2 years, 80 (72%) women. Among the 16 cases initially diagnosed as definite RHD, 11 (69%) were confirmed, 4 (25%) reclassified to borderline and 1 to normal. Among the 79 cases flagged as borderline RHD, 19 (24%) were confirmed, 50 (63%) reclassified to normal, 8 (10%) to definite RHD and 2 had mild CHD. Among 11 children with CHD in screening, 4 were confirmed, 5 were reclassified to normal and 2 to RHD. Considering the 4 diagnostic categories, kappa was 0.18. In patients with borderline RHD reclassified to non-RHD, the most frequent WHF criteria was B (isolated mitral regurgitation): 64%, followed by A (2 morphological features of the mitral valve): 31%. In the only patient with definite RHD reclassified to normal, WHF criteria was D (borderline RHD in aortic and mitral valves). After standard echo, RHD prevalence was 3.2% (95% CI 2.3 - 4.2). Conclusions: Although practical, RHD screening with handheld devices tend to overestimate prevalence. Even in endemic settings, continuous education for detection, and application of risk stratification for management are crucial.

Screening for the prevalence of rheumatic heart disease among school children in Egypt.

In low-income nations, Rheumatic valvular heart disease remains a major cause of morbidity and premature deaths. Accurate prevalence data in Egypt are still lacking yet highly desirable to facilitate health care planning. An cluster sample of school children in eastern Egyptian Governorates was examined clinically and echocardiographically (using abbreviated protocol) for detection of rheumatic valvular heart disease based on 2012 World Heart Federation criteria (the Doppler and the morphological criteria) for diagnosis. According to echocardiography interpretation, participants were categorized as having definite rheumatic heart disease (RHD), borderline RHD or no RHD. A total of 1680 students aged between 6 and 18 years were screened, 119 echocardiographic studies were non-interpretable; so, a total of 1560 studies were evaluated. From the total screened students, 1560 studies were adequate and interpretable according to the criteria specified in the methods section. This revealed a prevalence of 2.3% of the sample with RHD without adding the equivocal cases. The most prevalent lesion as detected by echocardiography was mitral regurgitation (29% of definite RHD cases). Rheumatic valvular heart disease remains prevalent in Egypt and the findings of this study should influence early detection, primary and secondary prevention, and adequate future national health plans.

Morpho-mechanistic screening criteria for the echocardiographic detection of rheumatic heart disease

IntroductionScreening echocardiography, guided by the current World Heart Federation (WHF) criteria, has important limitations that impede the establishment of large-scale rheumatic heart disease (RHD) control programmes in endemic regions. The...

High burden of rheumatic heart disease confirmed by echocardiography among Pacific adults living in New Zealand

BackgroundDespite numerous echocardiographic screening studies of children in high incidence acute rheumatic fever (ARF)/rheumatic heart disease (RHD) communities, little is known about the prevalence of RHD in adults in these...

Abbreviated Echocardiographic Screening for Rheumatic Heart Disease by Nonexperts with and without Offsite Expert Review: A Diagnostic Accuracy Study

Early detection of rheumatic heart disease (RHD) through echocardiographic screening can facilitate early access to effective treatment, which reduces the risk for progression. Accurate, feasible approaches to echocardiographic screening that can be incorporated into routine health services are needed. The authors hypothesized that offsite expert review could improve the diagnostic accuracy of nonexpert-obtained echocardiographic images. This prospective cross-sectional study was performed to evaluate the diagnostic accuracy of health worker-conducted single parasternal long-axis view with a sweep of the heart using hand-carried ultrasound for the detection of RHD in high-risk populations in Timor-Leste and Australia. In the primary analysis, the presence of any mitral or aortic regurgitation met the criteria for a positive screening result. Sensitivity and specificity were calculated for a screen-and-refer approach based on nonexpert practitioner assessment (approach 1) and for an approach using offsite expert review of nonexpert practitioner-obtained images to decide onward referral (approach 2). Each participant had a reference test performed by an expert echocardiographer on the same day as the index test. Diagnosis of RHD was determined by a panel of three experts, using 2012 World Heart Federation criteria. The prevalence of borderline or definite RHD among 3,329 participants was 4.0% (95% CI, 3.4%-4.7%). The sensitivity of approach 1 for borderline or definite RHD was 86.5% (95% CI, 79.5%-91.8%), and the specificity was 61.4% (95% CI, 59.7%-63.1%). Approach 2 achieved similar sensitivity (88.4%; 95% CI, 81.5%-93.3%) and improved specificity (77.1%; 95% CI, 75.6%-78.6%). Nonexpert practitioner-obtained single parasternal long-axis view with a sweep of the heart images, reviewed by an offsite expert, can detect borderline and definite RHD on screening with reasonable sensitivity and specificity. Brief training of nonexpert practitioners with ongoing support could be used as an effective strategy for scaling up echocardiographic screening for RHD in high-risk settings.

Comparison of approaches to determine echocardiographic outcomes for children with latent rheumatic heart disease

BackgroundScreening programmes using echocardiography offer opportunity for intervention through identification and treatment of early (latent) rheumatic heart disease (RHD). We aimed to compare two methods for classifying progression or regression...

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 15349: Factors Associated With Progression of Latent Rheumatic Heart Disease

Introduction: Secondary antibiotic prophylaxis improves outcomes for children diagnosed with latent rheumatic heart disease (RHD). However, many children with latent RHD show improvement without prescription of prophylaxis. The objective of this study was to determine if specific sociodemographic or echocardiographic features are associated with progression of latent RHD. Methods: This is a retrospective analysis of the GOAL Trial, a randomized controlled trial of secondary antibiotic prophylaxis in children with latent RHD conducted in Uganda. Sociodemographic and echocardiographic variables were collected at trial entry. Progression was defined by a change in echocardiographic category according to the World Heart Federation Criteria (WHF; normal, borderline RHD, mild definite RHD, moderate/severe RHD), determined by consensus of a blinded 4-member adjudication panel. The association of risk factors with progression were calculated as odds ratios (OR) with 95% confidence intervals (CI) using logistic regression models adjusted for the randomized treatment arm and the stratification variable (definite/borderline RHD). Results: Outcomes for the 799 children who completed the GOAL Trial were included in this analysis. Female sex (OR 2.6, 95% CI 1.19-5.68, p=0.016) and poorer socioeconomic conditions (WAMI Index 0-1 with 1 being the least deprived, OR 0.48 for every 0.10-point increase) were associated with progression. There was no difference in risk of progression or regression between those with borderline RHD or mild definite RHD. Conclusions: Females and those living in less advantaged conditions were more likely to show progression of latent RHD. The strength of these associations was relatively low and does not warrant restricting prophylaxis to subgroups based on risk factors. The lack of difference in progression risk between those with borderline and those with definite RHD may impact revisions of the World Heart Federation Criteria for latent RHD diagnosis and calls into question the use of the term ‘borderline RHD’.

Echocardiographic Screening for Rheumatic Heart Disease in a Ugandan Orphanage: Feasibility and Outcomes.

Background: Rheumatic heart disease (RHD) is a major cause of cardiovascular disease in developing nations, leading to more than 230,000 deaths annually. Most patients seek medical care only when long-term structural and hemodynamic complications have already occurred. Echocardiographic screenings ensure the early detection of asymptomatic subjects who could benefit from prophylaxis, monitoring and intervention, when appropriate. The aim of this study is to assess the feasibility of a screening program and the prevalence of RHD in a Ugandan orphanage. Methods: We performed an RHD-focused echocardiogram on all the children (5–14 years old) living in a north Ugandan orphanage. Exams were performed with a portable machine (GE Vivid-I). All the time intervals were recorded (minutes). Results: A total of 163 asymptomatic children were screened over 8 days (medium age 9.1; 46% male; 17% affected by severe motor impairment). The feasibility rate was 99.4%. An average of 20.4 exams were performed per day, with an average of 15.5 images collected per subject. Pathological mitral regurgitation (MR) was found in 5.5% of subjects, while at least two morphological features of RHD were found in 4.3%, leading to 1 “definite RHD” (0.6%) case and 13 “borderline RHD” cases (8.1%). Six congenital heart defects were also noted (3.7%): four atrial septal defects, one coronary artery fistula and one Patent Ductus Arteriosus. Conclusions: We demonstrated the feasibility of an echocardiographic screening for RHD in an orphanage in Uganda. A few factors, such as good clinical and hygienic care, the availability of antibiotics and closeness to a big hospital, may account for the low prevalence of the disease in our population.

Screening for subclinical rheumatic heart disease: addressing borderline disease in a real-world setting.

AimsThe World Heart Federation (WHF) criteria identify a large borderline rheumatic heart disease (RHD) category that has hampered the implementation of population-based screening. Inter-scallop separations (ISS) of the posterior mitral valve leaflet, a recently described normal variant of the mitral valve, appears to be an important cause of mild mitral regurgitation (MR) leading to misclassification of cases as WHF ‘borderline RHD’. This study aims to report the findings of the Echo in Africa project, a large-scale RHD screening project in South Africa and determine what proportion of borderline cases would be re-classified as normal if there were a systematic identification of ISS-related MR.Methods and resultsA prospective cross-sectional study of underserved secondary schools in the Western Cape was conducted. Participants underwent a screening study with a handheld (HH) ultrasound device. Children with an abnormal HH study were re-evaluated with a portable laptop echocardiography machine. A mechanistic evaluation was applied in cases with isolated WHF ‘pathological’ MR (WHF ‘borderline RHD’). A total of 5255 participants (mean age 15± years) were screened. A total of 3439 (65.8%) were female. Forty-nine cases of WHF ‘definite RHD’ [9.1 cases/1000 (95% confidence interval, CI, 6.8–12.1 cases/1000)] and 104 cases of WHF ‘borderline RHD’ [19.5 cases/1000 (95% CI, 16.0–23.7 cases/1000)] were identified. Inter-scallop separations-related MR was the underlying mechanism of MR in 48/68 cases classified as WHF ‘borderline RHD’ with isolated WHF ‘pathological’ MR (70.5%).ConclusionIn a real-world, large-scale screening project, the adoption of a mechanistic evaluation based on the systematic identification of ISS-related MR markedly reduced the number of WHF ‘screen-positive’ cases misclassified as WHF ‘borderline RHD’. Implementing strategies that reduce this misclassification could reduce the cost- and labour burden on large-scale RHD screening programmes.

Abstract 10180: Clinical and Subclinical Rheumatic Heart Disease Among Adults in the Amazon Basin: A Cross-Sectional Study

Introduction: Rheumatic heart disease (RHD) remains a significant cause of morbidity and mortality in middle- and low-income countries. However, the prevalence of RHD is often underestimated in resource limited areas due to lack of proper screening programs. Purpose: We aimed to determine the prevalence of clinical and subclinical RHD among adults in the Northwestern part of the Amazon Basin, Brazil. In addition, we evaluated the diagnostic yield of a history of self-reported rheumatic fever (RF) and cardiac auscultation Methods: We included a probability sample of adults from the general population in the Amazon Basin. Participants underwent questionnaire on medical history, cardiac auscultation and echocardiography. Participants were assessed according to the 2012 World Heart Federation (WHF) Criteria for echocardiographic diagnosis of RHD (Figure A) Results: We included a total of 591 participants (mean age 41+/-15 years, 60% women, 5% &lt;20 years old). 3% (n=19) had definite RHD (age 50 [IQR 29-70] years, 53% women) and &lt;1% (n=2) had borderline RHD. The distribution of definite RHD according to the WHF Criteria 2012 was: A (n=8), B (n=3), C (n=0), D (n=8). Valve pathologies are displayed in Figure B. Among definite RHD cases, 16% (n=3) were considered clinical (prevalence ~1%) and 84% (n=16) were subclinical (prevalence ~3%). A self-reported history of RF had a sensitivity of 3% and a specificity of 92% (PPV 2%/ NPV 95%) to detect RHD, whereas cardiac auscultation had a sensitivity of 38% and a specificity of 80% (PPV 5%/ NPV 97%). When combining a self-reported history of RF with cardiac auscultation, this yielded a sensitivity of 3% and a specificity of 98% (PPV 10%/ NPV 96%) Conclusions: By applying the 2012 WHF Criteria, the prevalence of clinical and subclinical RHD in adults from the Northwestern part of the Amazon Basin was 1% and 3%, respectively. A combination of a self-reported history of RF and cardiac auscultation provided low sensitivity but high specificity for RHD.

Outcomes of Echocardiography-Detected Rheumatic Heart Disease: Validating a Simplified Score in Cohorts From Different Countries.

BackgroundThe 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 ResultsPatient‐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.ConclusionsThe 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.

Single-View Echocardiography by Nonexpert Practitioners to Detect Rheumatic Heart Disease: A Prospective Study of Diagnostic Accuracy.

Echocardiographic screening can detect asymptomatic cases of rheumatic heart disease (RHD), facilitating access to treatment. Barriers to implementation of echocardiographic screening include the requirement for expensive equipment and expert practitioners. We aimed to evaluate the diagnostic accuracy of an abbreviated echocardiographic screening protocol (single parasternal-long-axis view with a sweep of the heart) performed by briefly trained, nonexpert practitioners using handheld ultrasound devices. Participants aged 5 to 20 years in Timor-Leste and the Northern Territory of Australia had 2 echocardiograms: one performed by an expert echocardiographer using a GE Vivid I or Vivid Q portable ultrasound device (reference test), and one performed by a nonexpert practitioner using a GE Vscan handheld ultrasound device (index test). The accuracy of the index test, compared with the reference test, for identifying cases with definite or borderline RHD was determined. There were 3111 enrolled participants; 2573 had both an index test and reference test. Median age was 12 years (interquartile range, 10-15); 58.2% were female. Proportion with definite or borderline RHD was 5.52% (95% CI, 4.70-6.47); proportion with definite RHD was 3.23% (95% CI, 2.61-3.98). Compared with the reference test, sensitivity of the index test for definite or borderline RHD was 70.4% (95% CI, 62.2-77.8), specificity was 78.1% (95% CI, 76.4-79.8). Nonexpert practitioners can be trained to perform single parasternal-long-axis view with a sweep of the heart echocardiography. However, the specificity and sensitivity are inadequate for echocardiographic screening. Improved training for nonexpert practitioners should be investigated.

Echocardiography for latent rheumatic heart disease in first degree relatives of children with acute rheumatic fever: Implications for active case finding in family members.

BackgroundIndividuals with Acute Rheumatic Fever (ARF) often report a family history of ARF or Rheumatic Heart Disease (RHD) however the degree of familial susceptibility to RHD is poorly defined. This study aimed to determine RHD prevalence among first degree relatives of ARF patients using echocardiography.MethodsChildren with ARF were recruited from Auckland, New Zealand. Parents and siblings ≥ 4years were offered echocardiography. Echocardiograms were reported according to World Heart Federation 2012 criteria. RHD prevalence in first degree relatives was compared to previously established population rates in the region.FindingsIn total, 70 index cases with ARF were recruited. Echocardiography was performed in 94 parents and 132 siblings. There were 3 siblings with definite RHD and 9 with borderline RHD. There were 4 parents with definite RHD. Overall prevalence of RHD (definite and borderline) in siblings was 90/1,000 (95% CI 45–143/1,000) compared to 36/1,000 (95% CI 30–42/1,000) in New Zealand children from high ARF incidence populations (p 0.001). Prevalence of definite RHD in parents was 42/1,000 (95% CI 7–87/1,000) compared to 22/1,000 (95% CI 9–36/1,000) in adults from a high ARF incidence New Zealand population (p 0.249).InterpretationRHD prevalence in siblings and parents of ARF cases is significantly greater than in comparable background populations. The contribution of hereditary versus environmental risk factors remains uncertain. We recommend targeted echocardiographic case-finding among siblings and parents of ARF/RHD cases in order to detect previously unrecognized latent RHD.