Impact of commissural calcification on clinical outcome of percutaneous balloon mitral valvuloplasty; a retrospective cohort study of 876 patients.

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

A study protocol to characterise pathophysiological and molecular markers of rheumatic heart disease and degenerative aortic stenosis using multiparametric cardiovascular imaging and multiomics techniques.

INTRODUCTION: Rheumatic heart disease (RHD), degenerative aortic stenosis (AS), and congenital valve diseases are prevalent in sub-Saharan Africa. Many knowledge gaps remain in understanding disease mechanisms, stratifying phenotypes, and prognostication. Therefore, we aimed to characterise patients through clinical profiling, imaging, histology, and molecular biomarkers to improve our understanding of the pathophysiology, diagnosis, and prognosis of RHD and AS. METHODS: In this cross-sectional, case-controlled study, we plan to recruit RHD and AS patients and compare them to matched controls. Living participants will undergo clinical assessment, echocardiography, CMR and blood sampling for circulatory biomarker analyses. Tissue samples will be obtained from patients undergoing valve replacement, while healthy tissues will be obtained from cadavers. Immunohistology, proteomics, metabolomics, and transcriptome analyses will be used to analyse circulatory- and tissue-specific biomarkers. Univariate and multivariate statistical analyses will be used for hypothesis testing and identification of important biomarkers. In summary, this study aims to delineate the pathophysiology of RHD and degenerative AS using multiparametric CMR imaging. In addition to discover novel biomarkers and explore the pathomechanisms associated with RHD and AS through high-throughput profiling of the tissue and blood proteome and metabolome and provide a proof of concept of the suitability of using cadaveric tissues as controls for cardiovascular disease studies.

Correlation between circulating fibrosis biomarkers with left atrial function and left atrial volume index in rheumatic mitral stenosis.

Mitral stenosis is the most common rheumatic heart disease (RHD) disorder worldwide, including in Indonesia. This pathological condition causes left atrial pressure, leading to left atrial fibrosis that affects the structure and function of the left atrial as well as the clinical condition. The aim of this study was to assess the correlation between circulating fibrosis biomarkers with net atrioventricular compliance (Cn) as a parameter of left atrial function, and left atrial volume index (LAVI) as a parameter left atrium structure of changes. A cross-sectional study was conducted at Panti Rahayu Hospital and Permata Bunda Hospital, Purwodadi, Central Java, with a total of 40 RHD patients with severe mitral stenosis. The ELISA was used to measure the levels of carboxy-terminal propeptide of type I procollagen (PICP), matrix metalloproteinase I (MMP-1), tissue inhibitor matrix metalloproteinase 1 (TIMP-1), and transforming growth factor-ß1 (TGF-ß1). The left atrial function was assessed by measuring Cn, and the LAVI parameters were measured to assess left atrium structure/size. The mean levels of circulating fibrosis biomarkers were as follows: PICP 153.96±89.12 ng/mL; MMP-1 1.44±2.12 ng/mL; MMP-1/TIMP-1 ratio 0.38±0.54 and TGF-ß1 2.66±1.96 pg/mL. From the echocardiographic evaluation, the mean Cn was 5.24±1.93 mL/mmHg and the mean LAVI was 152.55±79.36 mL/m2. There were significant correlation between MMP-1 and MMP-1/TIMP-1 ratio with Cn (r=0.345 and r=0.333, respectively; both had p<0.05). PICP and TGF-ß1 biomarkers did not significantly correlate with Cn (p>0.05). Meanwhile, none of the biomarkers had a significant correlation with LAVI (p>0.05). This study highlights that MMP-1 and MMP-1/TIMP-1 ratio are potentially to be used as markers to determine the Cn in RHD patients with severe mitral stenosis. However, further studies with a higher sample size are needed to confirm this finding.

Handheld echocardiography for the screening and diagnosis of rheumatic heart disease: a systematic review to inform WHO guidelines.

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). FINDINGS: 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. INTERPRETATION: 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. FUNDING: World Health Organization. TRANSLATIONS: For the Chinese, French, Italian, Persian, Portuguese, Spanish and Urdu translations of the abstract see Supplementary Materials section.

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

Background: 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. Methods: 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. Results: 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. Conclusion: 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.

Lack of awareness of secondary rheumatic prevention in preoperative candidates for mitral valve surgery - alarming situation.

BACKGROUND: We conducted this study to assess the compliance with secondary rheumatic prophylaxis among preoperative patients with rheumatic mitral valve disease undergoing valvular heart surgery at a tertiary care cardiac hospital in a developing country. METHODS: This is a descriptive cross-sectional study conducted at a tertiary care cardiac hospital in Karachi, Pakistan. The inclusion criteria encompassed patients of any sex, aged between 18 and 80 years, who had been diagnosed with rheumatic mitral valve disease through transthoracic echocardiography and had undergone valvular heart surgery. The level of compliance with secondary rheumatic prophylaxis and barriers toward noncompliance was assessed during routine preoperative interview session. This preventive measure plays a crucial role in reducing the progression of the disease and improving patient outcomes. RESULTS: Out of the 239 patients included in the study, 125 (52.3%) were females, with a mean age of 38.8 ± 11.8 years. The majority of patients (88.7%) came from rural areas. Among the patients, 79 (33.1%) received rheumatic prophylaxis, while 160 (66.9%) did not adhere to it regularly. The common barriers for receiving rheumatic prophylaxis were non availability (41.0%) and nonaffordability (40.6%). Additionally, 28.0% of patients had lacked awareness of the importance of rheumatic prophylaxis, and 2.5% expressed fear of injection site pain and subsequent symptoms. CONCLUSIONS: A concerning level of noncompliance with secondary rheumatic prophylaxis was observed. The barriers identified in patients who did not receive rheumatic prophylaxis were primarily related to affordability, availability, lack of awareness, and fear of injection site pain and subsequent symptoms.

Echocardiographic Parameters and Complication Profiles Among Adult Patients with Rheumatic Heart Disease at Jimma Medical Center.

Background: Despite the fact that patients with rheumatic heart disease (RHD) need early medical attention and follow-up, most patients in developing countries tend to present with debilitating complications. The objective of this study was to evaluate the echocardiographic features of adult individuals diagnosed with RHD and examine the associated complications among patients who started follow-up at Jimma Medical Center's (JMC) cardiac follow-up clinic. Methods: A prospective cross-sectional study was conducted at JMC between January 5 and April 15, 2023. Echocardiographic patterns were taken by senior cardiologists; socio-demographic variables, anthropometric measurements, and behavioral factors were collected through a structured questioner. Results: The study recruited a total of 115 participants, of whom 86 (74.8%) were female and 29 (25.2%) were male. The mean age of the patients was 32.31 (SD± 12.16) years. The mitral valve was affected in 98.26% of cases, while the aortic and tricuspid valve abnormalities were diagnosed in 49.5% and 21.7%, respectively. The most frequent combinations of valve lesions were mitral regurgitation (MR) + mitral stenosis (MS) + aortic regurgitation (AR) (15.7%), followed by MR + AR + TR (8.7%). The occurrence of MR+MS+AR was higher in females (17.4%) compared to males (10.3%), whereas the occurrence of MS+MR was higher in males (24.1%) compared to females (20.9%). Females have a severely reduced ejection fraction compared to males (84.8% vs 15.2%, P = 0.044). Nearly two-thirds (63.5%) of individuals experienced RHD-related complications; the most commonly encountered complications were pulmonary hypertension (26.1%) and atrial fibrillation (19.1%). Conclusion: RHD predominantly affects individuals in their active and productive years, particularly females. Most patients have multiple-valve lesions.

Left Atrial Enlargement as a Predictor of Atrial Fibrillation in Rheumatic Mitral Valve Disease: An Echocardiography-based Retrospective Cross-sectional Study.

BACKGROUND: Rheumatic heart disease is a major cause of cardiovascular morbidity and mortality in developing nations and is a leading cause of hospital admission due to cardiac problems in our country. This study will evaluate the association between left atrial size and the occurrence of atrial fibrillation and describe the clinical characteristics along with complications related to Rheumatic Mitral valve disease. METHODS: A retrospective cross-sectional study was conducted at a tertiary care center from January 2018 to December 2019. Reports of 207 patients admitted to medical and/or surgical wards with echocardiographic diagnosis of rheumatic mitral valve disease with or without atrial fibrillation were reviewed. Data were collected, entered, and analyzed using the Statistical Package for the Social Science version 25.0. RESULTS: Among 207 patients, atrial fibrillation was present in 90 (43.5%) patients. Atrial fibrillation was higher in patients with mixed mitral valvular lesions compared to isolated mitral stenosis or mitral regurgitation. Univariate and multivariate analysis revealed left atrial size [aOR=1.067, 95% CI: 1.023 - 1.113, P= 0.002] and age [aOR = 1.073, 95% CI: 1.042 - 1.105, P<0.001] as an independent predictor of atrial fibrillation. CONCLUSIONS: Larger left atrium was an independent predictor of atrial fibrillation. Besides this, atrial fibrillation was associated with increasing age, mixed mitral valvular lesion, and moderately reduced left ventricular ejection fraction, but not associated with gender and mitral stenosis severity. Left atrial clot was significantly higher in patients with atrial fibrillation than in sinus rhythm.

Routine Antenatal Echocardiography in High-Prevalence Areas of Rheumatic Heart Disease: A WHO-Guideline Systematic Review.

Background: Rheumatic Heart Disease (RHD) is the most common cause of valvular heart disease worldwide. Undiagnosed or untreated RHD can complicate pregnancy and lead to poor maternal and fetal outcomes and is a significant factor in non-obstetric morbidity. Echocardiography has an emerging role in screening for RHD. We aimed to critically analyse the evidence on the use of echocardiography for screening pregnant women for RHD in high-prevalence areas. Methods: We searched MEDLINE and Embase to identify the relevant reports. Two independent reviewers assessed the reports against the eligibility criteria in a double-blind process. Results: The searches (date: 4 April 2023) identified 432 records for screening. Ten non-controlled observational studies were identified, five using portable or handheld echocardiography, comprising data from 23,166 women. Prevalence of RHD varied across the studies, ranging from 0.4 to 6.6% (I2, heterogeneity >90%). Other cardiac abnormalities (e.g., congenital heart disease and left ventricular systolic dysfunction) were also detected <1% to 2% of cases. Certainty of evidence was very low. Conclusion: Echocardiography as part of antenatal care in high-prevalence areas may detect RHD or other cardiac abnormalities in asymptomatic pregnant women, potentially reducing the rates of disease progression and adverse labor-associated outcomes. However, this evidence is affected by the low certainty of evidence, and lack of studies comparing echocardiography versus standard antenatal care. Prospective Registration: PROSPERO 2022 July 4; CRD42022344081 Available from: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=344081. Research question: 'In areas with a high prevalence of rheumatic heart disease, should handheld echocardiography be added to routine antenatal care?'

Combined transcatheter aortic valve and tricuspid valve-in-valve implantation in a patient with a mitral mechanical prosthesis.

Despite progressively uncommon in Western countries, rheumatic heart disease still portrays a significant global burden. In elderly or high-surgical risk patients, plurivalvular disease may require a complex percutaneous approach. Transcatheter aortic valve implantation (TAVI) in patients with previous monoleaflet mitral prosthesis is challenging due to interference between the aortic valve and the rigid mitral mechanical prosthesis "ring." Prior cases report the use of CoreValve or Edwards Sapien aortic valves in patients with adequate mitro-aortic distance. Performing a second major procedure, such as tricuspid valve-in-valve (TVIV), sequentially during a single percutaneous intervention, increases treatment complexity. An 83-year-old woman with rheumatic heart disease, with previous implantation of a Bjork-Shiley monoleaflet mitral prosthesis, and Carpentier-Edwards 29 tricuspid bioprosthesis presented with decompensated heart failure due to severe aortic stenosis and tricuspid bioprosthesis stenosis. After HeartTeam discussion, the patient was deemed as inoperable due to a prohibitive surgical risk. As an alternative, a TAVI (Navitor FlexNav) and a transcatheter TVIV replacement (Edwards Sapiens 3 Ultra) were discussed and proposed, with both techniques being performed sequentially in a single procedure. TAVI in a patient with a previous monoleaflet mitral mechanical prosthesis and TVIV may be a feasible approach in inoperable patients with plurivalvular disease.

Successful treatment of severe primary mitral regurgitation due to rheumatic aetiology using a novel-designed transcatheter edge-to-edge repair system.

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

Relationship between New York functional class and duke activity status index with the severity of mitral valve stenosis and echocardiographic parameters: is left atrial strain a better predictor?

Purpose This study aimed to investigate the relationship between symptoms of patients with severe mitral stenosis (MS), evaluated by the New York Heart Association (NYHA) functional class and Duke Activity Status Index (DASI) score, and echocardiographic parameters. We evaluated patients with severe rheumatic MS diagnosed as mitral valve area (MVA) less than 1.5 cm2. All patients underwent transthoracic echocardiography and the left atrium (LA) reservoir auto-strain (LASr) analysis. In addition, DASI and NYHA scores were determined to evaluate the functional capacity and symptoms of MS patients. We evaluated 60 patients with MS with a mean age of 50.13 ± 10.28 and a median DASI score of 26.95 (26.38). There were 6 (10%) and 28 (46.7%) patients with NYHA class I and II, and 25 (40.0%) and 2 (3.3%) patients with NYHA class III and IV, respectively. NYHA class was positively correlated with LA area (LAA, r = 0.638), LA volume (LAV, r = 0.652), LAV index (LAVI, r = 0.62), E (r = 0.45), A (r = 0.25), and pulmonary artery pressure (PAP, r = 0.34), while negatively correlated with LASr (r = - 0.73) and MVA (r = - 0.417). Furthermore, the DASI score was positively associated with LASr (r = 0.81) and MVA (r = 0.52) while negatively correlated with LAA (r = - 0.62), LAV (r = - 0.65), LAVI (r = - 0.56), E (r = - 0.46), A (r = - 0.3), and PAP (r = - 0.32). Our findings indicate that LAA, LAV, LAVI, E, A, PAP, MVA, and LASr are associated with NYHA and DASI scores in MS patients. Additionally, the LASr had the strongest correlation between all measured parameters in severe MS patients.

Application of Transthoracic Echocardiography for Cardiac Safety Evaluation in the Clinical Development Process of Vaccines Against Streptococcus pyogenes.

Superficial infections with Streptococcus pyogenes (Strep A), pharyngitis and impetigo can induce acute rheumatic fever, an autoimmune sequela manifesting mostly with arthritis and rheumatic carditis. Valvular heart damage can persist or advance following repeated episodes of acute rheumatic fever, causing rheumatic heart disease. Acute rheumatic fever and rheumatic heart disease disproportionately affect children and young adults in developing countries and disadvantaged communities in developed countries. People living with rheumatic heart disease are at risk of experiencing potentially fatal complications such as heart failure, bacterial endocarditis or stroke. Transthoracic echocardiography plays a central role in diagnosing both rheumatic carditis and rheumatic heart disease. Despite the obvious medical need, no licensed Strep A vaccines are currently available, as their clinical development process faces several challenges, including concerns for cardiac safety. However, the development of Strep A vaccines has been recently relaunched by many vaccine developers. In this context, a reliable and consistent safety evaluation of Strep A vaccine candidates, including the use of transthoracic echocardiography for detecting cardiac adverse events, could greatly contribute to developing a safe and efficacious product in the near future. Here, we propose a framework for the consistent use of transthoracic echocardiography to proactively detect cardiac safety events in clinical trials of Strep A vaccine candidates.

Throat and skin infections caused by certain types of bacteria, named Streptococcus pyogenes, are frequent worldwide; however, in many children from less developed countries and disadvantaged communities, infections with S. pyogenes lead to a condition called acute rheumatic fever, which usually affects the joints and the heart. Damage to the heart valves may evolve to rheumatic heart disease, a permanent condition with often life-threatening complications. Rheumatic heart disease is an important health problem in places and communities where S. pyogenes infections occur frequently. A vaccine against these bacteria would help lower the number of people with valvular heart disease; however, no such vaccine exists yet. Research on vaccines against S. pyogenes was on hold for almost 30 years because of initial concerns that vaccinated children might develop acute rheumatic fever more frequently. Recently, researchers started working again on vaccines against S. pyogenes, but concerns about the safety of such vaccines persist. Doctors can reliably use echocardiography to diagnose cases of rheumatic carditis (as a sign of acute rheumatic fever) and rheumatic heart disease. Here, we propose a simple approach for the consistent use of echocardiography in clinical research of vaccines against S. pyogenes that will allow the detection of any potential heart-related side effects of the vaccine.

Follow-up echocardiographic changes in children and youth aged <25 years with latent rheumatic heart disease: A systematic review and meta-analysis of global data.

OBJECTIVES: To estimate progression, regression and persistence rates for borderline and mild-definite latent RHD in children and youth diagnosed at age < 25 years. METHODS: A review was conducted in accordance with Preferred Reporting Items for Systematic reviews and Meta-Analysis guidelines. Electronic databases were searched for latent RHD echocardiography follow-up studies which used World Heart Federation diagnostic criteria. A meta-analysis of outcomes was conducted for borderline and mild-definite disease subcategories. RESULTS: Data for 1618 individuals from 12 studies were included. For borderline cases, 48.51% regressed (95%CI 45.10-51.93), 13.99% progressed (95%CI 9.72-18.25), and 38.61% had persistent (unchanged) disease at follow-up (95%CI 29.68-47.54). For mild-definite cases, 34.01% regressed (95%CI 28.88-39.15), 8.06% progressed (95%CI 3.65-16.90), and 60.23% had persistent disease (95%CI 55.08-67.38). CONCLUSIONS: Borderline and mild-definite latent RHD show variable evolution following initial diagnosis. While 8% of mild-definite and 14% borderline cases had signs of disease progression at follow-up, a third of mild-definite and half of borderline cases had disease regression, even with sub adequate antibiotic prophylaxis. The significant variability between study cohorts suggests latent RHD natural history is likely variable between different endemic regions globally. Future research is needed to identify those individuals who would most benefit from antibiotic prophylaxis and determine regional natural history of latent RHD.

At the Heart of the Community: Implementation of Echocardiographic Screening for Rheumatic Heart Disease in Primary Care Facilities of Northern Borneo Island.

BACKGROUND: Rheumatic heart disease (RHD) is a potentially life-threatening condition that causes long-standing public health concerns. Echocardiography is a reliable diagnostic and screening technique for many cardiovascular conditions, including RHD. It is commonly used in tertiary care facilities worldwide but less so in the community setting. The primary aim of this study was to introduce and elaborate on the echocardiographic screening for RHD that was implemented by a Malaysian primary care clinic in Penampang district, Sabah. We also set out to present the uptake of the service in its initial years of establishment, as well as the challenges faced. METHODS: In the first part of this study, document reviews were conducted to compile relevant information about the conceptualization and implementation of this service. Following that, we also obtained secondary data on the echocardiographic screening service from its first implementation in April 2020 until May 2021 to examine the uptake and the patient profile. RESULTS: From April 2020 to May 2021, a total of 189 echocardiographic screening was conducted by primary care doctors using handheld ultrasound. Of the 189 children screened, 19 (10.1%) were found to have cardiac anomalies and were referred for a formal echocardiogram. Upon follow-up, 8 were detected with mild mitral regurgitation and referred to the nearest tertiary hospital for further management. CONCLUSION: Based on our review, the echocardiographic screening for RHD among children conducted by the Penampang Health Clinic was deemed successful. Echocardiogram service provided by primary care centers located in suburban and rural areas is highly beneficial for patients with poor access to specialized health care services because they stay far away from tertiary care facilities. Tapping into family medicine physicians located closer to communities to conduct echocardiographic screening and review the results can improve the detection of cardiac anomalies requiring further investigation. With the success of this project, echocardiographic services in the primary healthcare setting can be expanded by garnering the necessary collaborative efforts and consistent support from various stakeholders.

Evaluation of Jaw Bone Change in Children and Adolescents with Rheumatic Heart Disease by Fractal Analysis.

BACKGROUND: Recognizing the oral needs of people living with rheumatic heart disease (RHD), particularly in relation to their jaws, is of paramount importance. AIMS: This study aimed to evaluate potential jaw bone changes in children and adolescents diagnosed with RHD and compare them with a group of healthy children and adolescents. METHODS AND MATERIAL: The dental panoramic radiographs (DPRs) of 32 children and adolescents diagnosed with RHD comprised the case group. In contrast, the control group consisted of DPRs from 32 children and adolescents without any known diseases. Two regions of interest (ROI1 and ROI2) of trabecular bone and a separate region of interest (ROI3) of cortical bone were selected for fractal analysis. The mandibular cortical index (MCI) was evaluated. RESULTS: The ROI1 fractal dimension (FD) value of the individuals in the case group was statistically higher in the right jaw than in the left jaw (P < 0.01). Significant differences were determined between the case and control groups regarding the left ROI1, and the left and the right ROI2 (P < 0.05). No significant difference was found between the patient and control groups regarding the MCI value (P > 0.05). CONCLUSIONS: The results of this study showed that bone metabolism was affected unilaterally in the condyle region and bilaterally in the gonial region of patients with RHD. It also revealed that the cortical layer was not affected.

Silent Acute Rheumatic Fever Unmasked by Using Handheld Echocardiography for Febrile Children Presenting in a Rheumatic Heart Disease-Endemic Area.

OBJECTIVE: To understand the burden of acute rheumatic fever (ARF) among children living in low-income countries who present to the hospital with febrile illness and to determine the role of handheld echocardiography (HHE) in uncovering subclinical carditis as a major manifestation of ARF. STUDY DESIGN: This was a cross-sectional study carried at the Pediatric Hospital in Al Obeid, North Kordofan, Sudan, from September 2022 to January 2023 and including febrile children 3 through 18 years of age with or without clinical features of ARF and without another cause for their fever (not excluding malaria). History, examination, blood investigations, and HHE were done. ARF was diagnosed according to the Jones criteria. Clinical ARF was diagnosed if there was a major clinical Jones criterion and silent ARF if the only major Jones criteria was subclinical carditis. RESULTS: The study cohort included 400 children with a mean age of 9 years. Clinical ARF was diagnosed in 95 patients (95/400, 24%), most of whom presented with a joint major manifestation (88/95, 93%). Among the 281 children who did not present with a clinical manifestation of ARF, HHE revealed rheumatic heart disease (RHD) in 44 patients (44/281, 16%); 31 of them fulfilled criteria for silent ARF (31/281, 11%). HHE increased the detection of ARF by 24%. HHE revealed mild RHD in 41 of 66 (62%) and moderate or severe RHD in 25 of 66 (38%) patients. Both sensitivity and specificity of HHE compared with standard echocardiography were 88%. CONCLUSIONS: There is a significant burden of ARF among febrile children in Sudan. HHE increased the sensitivity of diagnosis, with 11% of children having subclinical carditis as their only major manifestation (ie, silent ARF). RHD-prevention policies need to prioritize decentralization of echocardiography to improve ARF detection.

Using Artificial Intelligence for Rheumatic Heart Disease Detection by Echocardiography: Focus on Mitral Regurgitation.

BACKGROUND: Identification of children with latent rheumatic heart disease (RHD) by echocardiography, before onset of symptoms, provides an opportunity to initiate secondary prophylaxis and prevent disease progression. There have been limited artificial intelligence studies published assessing the potential of machine learning to detect and analyze mitral regurgitation or to detect the presence of RHD on standard portable echocardiograms. METHODS AND RESULTS: We used 511 echocardiograms in children, focusing on color Doppler images of the mitral valve. Echocardiograms were independently reviewed by an expert adjudication panel. Among 511 cases, 229 were normal, and 282 had RHD. Our automated method included harmonization of echocardiograms to localize the left atrium during systole using convolutional neural networks and RHD detection using mitral regurgitation jet analysis and deep learning models with an attention mechanism. We identified the correct view with an average accuracy of 0.99 and the correct systolic frame with an average accuracy of 0.94 (apical) and 0.93 (parasternal long axis). It localized the left atrium with an average Dice coefficient of 0.88 (apical) and 0.9 (parasternal long axis). Maximum mitral regurgitation jet measurements were similar to expert manual measurements (P value=0.83) and a 9-feature mitral regurgitation analysis showed an area under the receiver operating characteristics curve of 0.93, precision of 0.83, recall of 0.92, and F1 score of 0.87. Our deep learning model showed an area under the receiver operating characteristics curve of 0.84, precision of 0.78, recall of 0.98, and F1 score of 0.87. CONCLUSIONS: Artificial intelligence has the potential to detect RHD as accurately as expert cardiologists and to improve with more data. These innovative approaches hold promise to scale echocardiography screening for RHD.