Recurrent Acute Rheumatic Fever Research Articles

Local anaesthetic to reduce injection pain in patients who are prescribed intramuscular benzathine penicillin G: a systematic review and meta-analysis

Three to 4-weekly intramuscular injections of benzathine penicillin G (BPG) for a prolonged period (e.g., 10 years, until age 40 years, or lifelong) are recommended for preventing group A streptococcal infections that cause recurrent acute rheumatic fever (ARF) and potential progression to rheumatic heart disease (RHD). The duration of treatment, frequency and local pain associated with BPG injections may lead to reduced compliance. Shorter courses of BPG are recommended for the treatment of syphilis and Streptococcal infections. We aimed to assess the effects of local anaesthesia in reducing injection pain in patients who are being treated with BPG. In this systematic review and meta-analysis, we searched the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, Conference Proceedings Citation Index-Science and LILACS from database inception up to May 4, 2024, and performed additional searches for grey literature. Randomised controlled trials comparing BPG vs. BPG administered alongside local anaesthetics were included. Randomized controlled trials using BPG, irrespectively of indication, and testing any local anaesthetic agent for pain alleviation were considered eligible. We applied GRADE to assess the quality of evidence. Summary data were extracted from included trials. The primary outcome was injection pain, assessed through mean differences. A random-effects model was utilized to account for study heterogeneity. This study is registered with PROSPERO, CRD42022342437. Database searches identified a total of 3958 records, and 3 additional records were retrieved from grey literature searches. After removal of duplicates, screening of abstracts and full-text review, eight trials were included, combining a total of 489 patients (151 patients with RHD). Immediate pain level, as reported by patients, was of high intensity in most studies. Low intensity pain was still reported at 24h. Administration of lidocaine mixed with BPG was associated with a significant reduction in immediate post-injection pain (mean difference-3.84, 95% confidence interval-6.19 to-1.48, P=0.0001; 4 studies; I2=98%; GRADE: moderate quality), pain at 5 min (mean difference-2.85, 95% CI confidence interval-3.78 to-1.92, P<0.0001; 1 study; GRADE: moderate quality), and pain at 20 min (mean difference-1.85, 95% confidence interval-2.61 to-1.09, P<0.0001; 1 study; GRADE: moderate quality) on a 1 to 10 scale. One study assessed lidocaine cream applied to the skin prior to BPG injection and showed no significant reduction in injection pain (mean difference=-0.54, 95% CI confidence interval-1.17 to 0.09, P=0.13; 1 study; GRADE: low quality). Mepivacaine mixed with BPG in patients with syphilis showed a significant reduction of immediate post-injection pain (mean difference-2.19, 95% CI confidence interval-2.49 to-1.89, P<0.0001; 1 study; GRADE: moderate quality). Two studies assessed procaine mixed with BPG and reported: lower immediate pain levels or pain assessed at 1h (mean difference and 95% CI confidence intervals not provided, P=0.001 and P=0.008, respectively; 1 study; GRADE: low quality), or less immediate pain and pain at 24h on the buttock injected with procaine mixed with BPG (mean difference and 95% CI confidence intervals not provided, P<0.001 for both; 1 study; Grade: low quality). No severe adverse reactions were reported. In patients receiving intramuscular BPG injections, moderate quality quantitative evidence suggests that BPG injections diluted with lidocaine or mepivacaine may improve post-injection pain scores compared to BPG injections diluted with sterile water. Procaine may also have a benefit, but quality of evidence was lower. Most studies included small patient samples and assessed pain levels at different timepoints. Due to insufficient data we were not able to assess the impact of injection volume, and local anaesthetics' dose on pain intensity and duration of pain relief. WHO.

Clinical profile of paediatric acute rheumatic fever and rheumatic heart disease in Western Australia: 1987 to 2020.

To describe the clinical profile of acute rheumatic fever (ARF) presentations to paediatric cardiology tertiary services in Western Australia (WA). A retrospective clinical audit of individuals with confirmed ARF referred to the only paediatric tertiary cardiac service in WA (1 January 1987 to 31 December 2020). Comparisons between inpatient, outpatient, remote and non-remote groups were assessed. Four hundred seventy-one episodes of ARF in 457 individuals (235 male; median age = 8 years) met clinical criteria. The majority were Aboriginal and Torres Strait Islander children (91.2%), with 62.1% living in remote areas. The number of ARF and rheumatic heart disease (RHD) diagnoses per year increased from 1987 to 2017 with notable peaks in 2013 and 2017. The average annual incidence of tertiary-referred ARF in WA of 4-15-year-olds from 1987 to 2020 was 4.96 per 100 000. ARF features included carditis (59.9%), chorea (31%), polyarthritis (30%) and polyarthralgia (24.2%). RHD was evident in 61.8% of cases and predominantly manifested as mitral regurgitation (55.7%). Thirty-four children (7.4%) with severe RHD underwent valvular surgery. 12% had at least one recurrent ARF episode. Remote individuals had more than double the rate of recurrence compared to non-remote individuals (P = 0.0058). Compared to non-remote episodes, remote presentations had less polyarthritis (P = 0.0022) but greater proportions of raised ESR (P = 0.01), ASOT titres (P = 0.0073), erythema marginatum (P = 0.0218) and severe RHD (P = 0.0133). The high proportion of Aboriginal and Torres Strait Islander Australians affected by ARF/RHD in WA reflects the significant burden of disease within this population. Children from remote communities were more likely to present with concurrent severe RHD. Our study reinforces the persisting need to improve primary and secondary ARF initiatives in rural and remote communities.

Persistent Valvular Regurgitation After Acute Rheumatic Fever: Early Predictors of Long Term Outcomes in a Pediatric Retrospective Cohort.

Describe the echocardiographic evolution of valvular regurgitation in patients with rheumatic carditis (RC) and to establish which features may predict long-term outcome, in the absence of acute rheumatic fever (ARF) relapse. Retrospective cohort study. 123 patients with confirmed RC, diagnosed at Turin Children's Hospital between 2010 and 2019. We reviewed the echocardiographic images recorded at diagnosis, after 6-8weeks, after 6months, then yearly, to assess which predictors at diagnosis are associated with the degree of improvement at 6months. Secondly, we tested which variables predict the regression of pathological regurgitation of mitral (MV) or aortic valve (AV) during follow-up. At onset, 90.2% patients had MV regurgitation while 42.3% had AV involvement. 115 (93.5%) patients were treated with steroids and 70.8% experienced a downgrading of RC after 6months. Steroids were associated with better outcomes at six months (p = 0.01). During follow-up (median 56.1months), MV improved in 58.6% patients, AV in 46.2%. At multivariate analysis, erythrocyte sedimentation rate (ESR) was positively associated with regression of MV regurgitation (OR 1.02, p = 0.02), while higher degree of carditis at onset was negatively associated (OR 0.04, p < 0.01). Conversely, regression of AV regurgitation was more frequent in patients with bi-valvular involvement(OR 20.5, p = 0.03) and in absence of murmur at onset (OR 0.04, p = 0.01). This study indicates that valvular regurgitation improves overtime if there are no ARF recurrences during follow-up, especially when the MV is involved and in patients treated with steroids.

Comparative Study of Oral Naproxen and Aspirin for Acute Rheumatic Fever Treatment: Safety And Efficacy Analysis

Objectives: This observational case-control study aimed to assess the safety and efficacy of oral Naproxen as an alternative to oral Aspirin/acetylsalicylic acid (ASA) for treating acute rheumatic fever (ARF). Methodology: Patients meeting the revised Jones criteria 2015 for ARF were enrolled from November 2018 to May 2019 at a single tertiary care Children’s Hospital. They were divided into two groups: Group-A receiving ASA (control) and Group-B receiving Naproxen (case). Primary outcome measures included the number of days until complete resolution of arthralgia or arthritis, while secondary outcome measures included resolution of fever and normalization of erythrocyte sedimentation rate (ESR). Results: Sixty-four consecutive patients with ARF were enrolled, with 32 in each group, matched for age and gender. The majority (80%) had recurrent ARF. Median age at presentation was similar in both groups. ESR levels did not differ between the groups at admission or at the end of treatment. Median time for resolution of fever was 9(6-11) days in Group-A and 7.5(5-10) days in Group-B. Resolution time for arthritis was similar in both groups, with a median of 3(2-4) days. Gastric pain and vomiting were significantly lower in Group-B than in Group-A. Overall response rates were comparable between the groups. Conclusion: Naproxen demonstrates equal effectiveness, safety, and better tolerance compared to Aspirin in treating ARF patients.

Outcomes of rheumatic fever in Uganda: a prospective cohort study

Rheumatic heart disease is the largest contributor to cardiac-related mortality in children worldwide. Outcomes in endemic settings after its antecedent illness, acute rheumatic fever, are not well understood. We aimed to describe 3-5 year mortality, acute rheumatic fever recurrence, changes in carditis, and correlates of mortality after acute rheumatic fever. We conducted a prospective cohort study of Ugandan patients aged 4-23 years who were diagnosed with definite acute rheumatic fever using the modified 2015 Jones criteria from July 1, 2017, to March 31, 2020, enrolled at three rheumatic heart disease registry sites in Uganda (in Mbarara, Mulago, or Lira), and followed up for at least 1 year after diagnosis. Patients with congenital heart disease were excluded. Patients underwent annual review, most recently in August, 2022. We calculated rates of mortality and acute rheumatic fever recurrence, tabulated changes in carditis, performed Kaplan-Meier survival analyses, and used Cox regression models to identify correlates of mortality. Data were collected between Sept 1 and Sept 30, 2022. Of 182 patients diagnosed with definite acute rheumatic fever, 156 patients were included in the analysis. Of these 156 patients (77 [49%] male and 79 (51%) female; data on ethnicity not collected), 25 (16%) died, 21 (13%) had a cardiac-related death, and 17 (11%) had recurrent acute rheumatic fever over a median of 4·3 (IQR 3·0-4·8) years. 16 (24%) of the 25 deaths occurred within 1 year. Among 131 (84%) of 156 survivors, one had carditis progression by echo. Moderate-to-severe carditis (hazard ratio 12·7 [95% CI 3·9-40·9]) and prolonged PR interval (hazard ratio 4·4 [95% CI 1·7-11·2]) at acute rheumatic fever diagnosis were associated with increased cardiac-related mortality. These are the first contemporary data from sub-Saharan Africa on medium-term acute rheumatic fever outcomes. Mortality rates exceeded those reported elsewhere. Most decedents already had chronic carditis at initial acute rheumatic fever diagnosis, suggesting previous undiagnosed episodes that had already compounded into rheumatic heart disease. Our data highlight the large burden of undetected acute rheumatic fever in these settings and the need for improved awareness of and diagnostics for acute rheumatic fever to allow earlier detection. Strauss Award at Cincinnati Children's Hospital, American Heart Association, and Wellcome Trust.

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.

Echocardiographic Screening of Rheumatic Heart Disease: Current Concepts and Challenges.

The incidence of acute rheumatic fever (ARF), which most commonly affects children aged 5-15 years after group A Streptococcus (GAS) infection, ranges from 8 to 51 per 100 000 people worldwide. Rheumatic heart disease (RHD), which occurs when patients with ARF are inappropriately treated or not given regular prophylaxis, is the most common cause of non-congenital heart disease in children and young adults in low-income countries. Timely treatment of GAS infection can prevent ARF, and penicillin prophylaxis can prevent recurrence of ARF. Secondary prophylaxis with benzathine penicillin G has been shown to decrease the incidence of RHD and is a key aspect of RHD control. The most important factor determining the prognosis of RHD is the severity of cardiac involvement. Although approximately 70% of patients with carditis in the acute phase of the disease recover without sequelae, carditis is important because it is the only complication of ARF that causes sequelae. One-third of patients with ARF are asymptomatic. Patients with mild symptoms of recurrent ARF and silent RHD will develop severe morbidities within 5-10 years if they do not receive secondary preventive treatments. A new screening program should be established to prevent cardiac morbidities of ARF in moderate- and highrisk populations. In the present study, we examined the applicability of echocardiographic screening programs for RHD. Cite this article as: Topçu S, Uçar T. Echocardiographic screening of rheumatic heart disease: Current concepts and challenges. Turk Arch Pediatr. 2024;59(1):3-12.

Current Clinical Profile of Acute Rheumatic Fever and Recurrent Acute Rheumatic Fever in Pakistan.

Inclusion of echocardiography as diagnostic tool and polyarthralgia and monoarthritis as major criteria for high-risk populations in the Revised Jones Criteria 2015 is likely to surface substantial variability in clinical manifestations among various populations. This study aimed to compare clinical profile of patients presenting with first and recurrent episodes of acute rheumatic fever (ARF) using most recent criteria. 130 consecutive patients with ARF were included in the study from August 2019 to March 2022. World Heart Federation standardized echocardiographic criteria were used for cardiac evaluation. The socio-demographic variables, clinical details and relevant investigations were recorded. Median age was 13(6-26) years. Male to female ratio was 1.6:1. Majority was of low socioeconomic status (90%) and with > 5 family members in a house (83.8%). 27 patients (20.8%) were with ARF while 103 (79.2%) with recurrent ARF. Carditis was the most common presenting feature (n = 122, 93.8%), followed by polyarthralgia (n = 46, 35.4%), polyarthritis (n = 32, 24.6%), subcutaneous nodules (n = 10, 7.7%), monoarthritis (n = 10, 7.7%), and chorea (n = 5, 3.8%). Monoarthralgia was more common in ARF than recurrence (29.4% vs. 3.2%, p = 0.004). Carditis (97.1% vs. 81.5%, p = 0.01) and congestive cardiac failure (18.5% vs. 5.9%, p = 0.001) were more common in recurrent ARF than ARF. Diagnostic categorization of Jones criteria for different populations has highlighted important variability in clinical presentation of ARF. Monoarthralgia is common in first episode of ARF. Carditis is the most common feature in recurrent ARF. Polyarthralgia is seen with higher frequency that polyarthritis. Subcutaneous nodules seem to be more common in our population.

Abstract 12362: High Rates of Mortality in Uganda Following Acute Rheumatic Fever: Findings From a Community-Based Prospective Cohort Study

Introduction: Rheumatic heart disease (RHD) is the largest contributor to cardiac mortality in children worldwide. Little contemporary data exist on its antecedent illness, acute rheumatic fever (ARF). There is an urgent need to understand outcomes following ARF in RHD-endemic settings. We aimed to describe medium-term mortality, disease recurrence, and change in carditis among Ugandan children following ARF diagnosis, and to identify correlates of mortality. Methods: We conducted a prospective cohort study of children diagnosed with definite ARF in 2017-2020 during a community-based incidence study. Patients were enrolled in a national registry and followed with annual review, most recently in August 2022. Kaplan-Meier (KM) survival analysis was used to describe all-cause mortality and individual Cox proportional hazards regression models were used to identify correlates of death. Descriptive statistics were used to characterize changes in carditis. Results: Among 135 children with definite ARF, 22/135 (16.3%) experienced death, 19/135 (14.1%) cardiac death, 5/135 (3.7%) ARF recurrence, and 5/135 (3.7%) surgery over a median (interquartile range) of 4.25 (3.4, 5.1) years. KM analysis saw 14/22 (63.6%) of deaths occur by one year, 16/22 (72.7%) by two years, and 20/22 (90.9%) by three years. The presence of moderate/severe carditis (HR 14.1, 95% CI 4.2, 47.7) and PR prolongation (HR 3.9, 95% CI 1.5, 9.9) at diagnosis was associated with increased cardiac mortality. Among 107 survivors included in echocardiographic sub-analysis, 27/107 (25.2%) had improvement in carditis, 70/107 (65.4%) no change, and 10/107 (9.4%) progression. Conclusions: These are the first contemporary data from sub-Saharan Africa reviewing medium-term outcomes following ARF. Adverse outcome rates exceeded those reported elsewhere. Most decedents already had advanced cardiac involvement upon first ARF diagnosis, as evidenced by PR prolongation and high-grade carditis, suggesting a history of prior undiagnosed ARF recurrences that had already compounded into RHD. These data indicate that a high burden of ARF exists in the community that is undetected by current methods, emphasizing the need for better point-of-care diagnostics to improve early ARF detection.

An outbreak of acute rheumatic fever in a remote Aboriginal community

ObjectivesWe describe the public health response to an outbreak of acute rheumatic fever (ARF) in a remote Aboriginal community. MethodsIn August 2021, the Northern Territory Rheumatic Heart Disease Control Program identified an outbreak of acute rheumatic fever in a remote Aboriginal community. A public health response was developed using a modified acute poststreptococcal glomerulonephritis protocol and the National Acute Rheumatic Fever Guideline for Public Health Units. Results12 cases were diagnosed during the outbreak; six-times the average number of cases in the same period in the five years prior (n=1.8). Half (n=6) of the outbreak cases were classified as recurrent episodes with overdue secondary prophylaxis. Contact tracing and screening of 11 households identified 86 close contacts. ConclusionsThis outbreak represented an increase in both first episodes and recurrences of acute rheumatic fever and highlights the critical need for strengthened delivery of acute rheumatic fever secondary prophylaxis, and for improvements to the social determinants of health in the region. Implications for Public HealthOutbreaks of acute rheumatic fever are rare despite continuing high rates of acute rheumatic fever experienced by remote Aboriginal communities. Nevertheless, there can be improvements in the current national public health guidance relating to acute rheumatic fever cluster and outbreak management.

Clinical profile of acute rheumatic fever in children in Pakistan: A study at a tertiary care hospital.

Objective: To determine the clinical profile of children presenting with acute rheumatic fever (ARF) at a tertiary care healthcare facility. Study Design: Cross-sectional study. Setting: Department of Pediatrics, Rai Medical College Teaching Hospital, Sargodha Pakistan. Period: January 2021 to December 2022. Material &amp; Methods: We analyzed children of both genders aged between 1 month to 18 years and presenting with ARF. At the time of enrollment, medical history was obtained and physical as well as clinical examinations were performed. The demographical and clinical information were recorded. ARF was labeled as per “Modified Jones Criteria”. A special proforma was formed to record study data. Results: In a total of 154 children, 96 (62.3%) were female while the mean age was 9.3±4.6 years. Forty four (28.6%) patients were having first episode of ARF while 110 (71.4%) had recurrence of ARF. Fever was noted to be the most common presentation noted in 121 (78.6%) patients of ARF. Arthritis was significantly more common among patients with first episode of ARF when compared to recurrence of ARF (70.5% vs. 29.1%, p&lt;0.0001). Degree of carditis was significantly more severe among patients with recurrence of ARF (p&lt;0.0001). Conclusion: Majority of the acute rheumatic fever patients present with recurrence of acute rheumatic fever. Fever was the commonest clinical presentation while arthritis was significantly more prevalent among patients with first episode of acute rheumatic fever.

Positive impact of training rural health workers in identification and prevention of acute rheumatic fever in eastern Uganda.

Diagnosis of acute rheumatic fever (ARF) is mainly clinical. Delayed or missed diagnosis and failure to administer appropriate and timely treatment of ARF leads to rheumatic heart disease (RHD), which could necessitate expensive treatments such as open-heart surgery. Implementation of preventative guidelines depends on availability of trained healthcare workers. As part of the routine support supervision, the Uganda Heart Institute sent out a team to rural eastern Uganda to evaluate health workers' knowledge level regarding management of ARF. Health workers from selected health facilities in Tororo district, eastern Uganda, were assessed for their knowledge on the clinical features and role of benzathine penicillin G (BPG) in the treatment and prevention of ARF recurrence. Using the RHD Action Needs assessment tool, we generated and administered a pre-test, then conducted training and re-administered a post-test. Eight months later, health workers were again assessed for knowledge retention and change in practices. Statistical analysis was done using Stata version 15. During the initial phase, 34 of the 109 (31%) health workers passed the pre-test, indicating familiarity with clinical features of ARF. The level of knowledge of BPG use in ARF was very poor in all the health units [25/109 (22.6%)] but improved after training to 80%, as shown by the chi-squared test ( χ2 = 0.000). However, retention of this knowledge waned after eight months and was not significantly different compared to pre-training (χ2 ≥ 0.2). A critical knowledge gap is evident among health workers, both in awareness and treatment of ARF, and calls for repetitive training as a priority strategy in prevention.

The evidence that rheumatic heart disease control programs in Australia are making an impact

ObjectiveRheumatic heart disease (RHD) comprises heart-valve damage caused by acute rheumatic fever (ARF). The Australian Government Rheumatic Fever Strategy funds RHD Control Programs to support detection and management of ARF and RHD. We assessed epidemiological changes during the years of RHD Control Program operation. MethodsLinked RHD register, hospital and death data from four Australian jurisdictions were used to measure ARF/RHD outcomes between 2010 and 2017, including: 2-year progression to severe RHD/death; ARF recurrence; secondary prophylaxis delivery and earlier disease detection. ResultsDelivery of secondary prophylaxis improved from 53% median proportion of days covered (95%CI: 46-61%, 2010) to 70% (95%CI: 71-68%, 2017). Secondary prophylaxis adherence protected against progression to severe RHD/death (hazard ratio 0.2, 95% CI 0.1-0.8). Other measures of program effectiveness (ARF recurrences, progression to severe RHD/death) remained stable. ARF case numbers and concurrent ARF/RHD diagnoses increased. ConclusionsRHD Control Programs have contributed to major success in the management of ARF/RHD through increased delivery of secondary prevention yet ARF case numbers, not impacted by secondary prophylaxis and sensitive to increased awareness/surveillance, increased. Implications for public healthRHD Control Programs have a major role in delivering cost-effective RHD prevention. Sustained investment is needed but with greatly strengthened primordial and primary prevention.

Rheumatic Heart Disease Secondary Prophylaxis Prescribing Patterns and Outcomes

Background: Rheumatic heart disease (RHD) is a leading cause of heart disease in children and young adults in developing countries and is considered a significant cause of cardiovascular diseases. Antimicrobial prophylaxis is recommended as secondary prevention for heart complications caused by recurrent acute rheumatic fever. Objective: Our study aims to describe the prescribing patterns of RHD antibiotic secondary prophylaxis among eligible patients and highlight the frequency of valvular heart disease complications requiring intervention. Materials and Methods: A single-center retrospective study was conducted at a tertiary hospital in Saudi Arabia. The study included all patients 5 years old and above diagnosed with rheumatic heart fever and/or RHD and eligible for secondary prevention between January 2009 and December 2018. The primary outcome is the frequency of RHD secondary prophylaxis prescribed for eligible patients. Results: Among 806 patients who were screened, 272 patients were eligible for inclusion. A total of 194 (71.3%) were female, and 146 (53.67%) were older than 40. RHD secondary prophylaxis prescribing adherence was found in only 14 of 272 (5.14%). A total of 185 of 272 (68%) patients had at least one cardiac-related intervention during the follow-up period. Valvular heart disease progression was found in 72 of 185 (38.9%) after a primary intervention. Overall mortality was 30 (11%). Conclusion: Our study found low prescribing adherence to RHD secondary prevention among eligible patients in an area with a high disease prevalence. A larger sample size is needed to confirm these findings.

Secondary Prevention of Rheumatic Heart Disease in Nepal: Are We Going Backward?

A secondary level of prophylaxis has proven to be the most successful in Nepal, a country with an endemic rate of rheumatic heart disease, in combating the severe issues associated with rheumatic heart disease. The use of benzathine penicillin G in secondary prophylaxis of rheumatic heart disease, recommended by several guidelines, has been increasingly abandoned in Nepal due to a lack of national guidelines and the termination of the prior programs. The use of oral penicillin and alternative oral antibiotics, which are less effective in preventing the recurrence of acute rheumatic fever, is on the rise. Nepal urgently needs to develop new national guidelines and ensure their effective implementation in order to slow the increase in the number of rheumatic heart disease patients. In this article, we explore the limitations, challenges, and advantages of using the consensus-supported intramuscular benzathine penicillin G as the first-line drug for the secondary prevention of rheumatic heart disease.

An Uncommon Association of Ebstein’s Anomaly and Acute Rhumatic Fever

Background: Ebstein’s anomaly is a relatively rare congenital heart disease (atrialisation of the right ventricle). Anomalies of left side of the heart have been described with this condition. We however present a case of Ebstein's anomaly with recurrent acute rheumatic fever (ARF) and mitral stenosis. Case report: A 13-year-old boy with not followed for heart disease and with a history of notion of repetitive angina, is admitted to the pediatric emergency room for fever and polyarthritis. General examination not showed subcutaneous nodules and laboratory investigations revealed leucocytosis, elevated erythrocyte sedimentation rate (ESR) and antistreptolysin O (ASO) titre. Echocardiography revealed Ebstein anomaly with moderate mitral stenosis. A diagnosis of recurrent ARF was made based on the carditis, arthritis, history of recurrence of sore throat and evidence of recent streptococcal infection. He was transferred to the cardiology department and initially put on medical treatment. Its clinical course was marked by the appearance of unresolved ventricular tachycardia despite drug cardioversion. His prognosis was unfavorable with death after 10 days of hospitalization under antiarrhythmic treatment. Conclusion: Ebstein's disease is a rare disease. Cardiac arrhythmias are deleterious and the association of rheumatic fever with co-morbidity increases its lethality.

Secondary prevention of rheumatic heart disease in Ethiopia: a multicenter study

BackgroundEthiopia has a high acute rheumatic fever (ARF) and rheumatic heart disease (RHD) prevalence, and to our knowledge, there are no data on the status of secondary prevention in children with RHD. This study describes the status of secondary RHD prevention.MethodsA multicenter, prospective study was performed on children aged 5–17 years with RHD in Ethiopia. Good adherence was defined as at least 80% completion of benzathine penicillin (BPG) or oral Amoxicillin within the previous year. The primary outcome measure was adherence to prophylaxis, expressed as a proportion. Socio-demographics, severity of RHD, and ARF recurrence were evaluated.ResultsA total of 337 children with a mean age of 12.9 ± 2.6 years were included. The majority (73%) had severe aortic/mitral disease. Participants were on BPG (80%) or Amoxicillin (20%) prophylaxis. Female sex (P = 0.04) use of BPG (0.03) and shorter mean duration of prophylaxis in months (48.5 ± 31.5 vs. 60.7 ± 33, respectively, P < 0.008) predicted good adherence. Running out of medications (35%), interrupted follow-up (27%), and the COVID-19 pandemic (26%) were the most common reasons for missing prophylaxis. Recurrence of ARF was higher in participants on Amoxicillin compared with BPG (40% vs. 16%, P < 0.001) and in those with poor adherence compared with good adherence (36.8% vs. 17.9%, respectively, P = 0.005). Type and duration of prophylaxis (OR 0.5, CI = 0.24, 0.9, P = 0.02; OR = 1.1, CI = 1.1, 1.2, P = 0.04, respectively), and sex (OR = 1.9, CI = 1.1, 3.4, P = 0.03) were independent predictors of poor adherence.ConclusionPoor adherence is prevalent in Ethiopian children living with RHD. Amoxicillin is a suboptimal option for prophylaxis as its use is associated with lower adherence and a higher rate of ARF recurrence.

Evaluation of Children with Acute Rheumatic Fever: A Single-Center Experience.

Objective:The epidemiological characteristics, risk factors, complications, recurrence status, clinical and laboratory features, and treatment methods of the patients who admitted to our Pediatric Cardiology Outpatient Clinic with a pre-diagnosis of acute rheumatic fever (ARF) were evaluated.Materials and Methods:The data of 166 patients who admitted with a pre-diagnosis of ARF and were diagnosed with ARF, and the data of 51 patients who were not diagnosed with ARF, were retrospectively analyzed.Results:The patients with ARF were between the ages of 5 and 18. Most of the patients with ARF attack admitted in December (15.6%), January (13.8%), and February (13.2%). The most common complaints of the patients diagnosed with ARF were isolated joint pain and/or swelling, at 50.6%. While 91.5% of the patients were diagnosed for the first time, 8.5% had ARF recurrence. It was seen that the most common major criterion was carditis (94.6%). The severity of valve regurgitation and the rates of monoarthritis were significantly higher in patients with recurrence (P < .05). Non-compliance with prophylaxis was observed in 10 (71.4%) of 14 patients with recurrence, and in 43 (28.2%) of 152 patients without recurrence. Anti-streptolysin O was lower (P = .021) and alanine transaminase (ALT) was higher (P = .019) in the recurrence group.Conclusion:Our study showed that in patients with a pre-diagnosis of ARF, a differential diagnosis should be made with other diseases. Especially in patients with joint complaints as the only major symptom, a differential diagnosis should be made. ARF recurrence is associated with non-compliance with prophylaxis, and both the severity of valve regurgitation and monoarthritis rates are higher in patients who develop recurrence. Alanine aminotransferase is significantly higher in patients with ARF recurrence.

Subjective wellbeing among rheumatic heart disease patients at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia: observational cross-sectional study

BackgroundSubjective wellbeing (SWB) is a self-reported positive life judgment and good feeling. RHD, rheumatic heart disease, is a long-term sequel of single or recurrent acute rheumatic fever. There are no studies that assessed SWB in RHD patients. We aimed to assess SWB among RHD subjects enrolled in chronic care at Tikur Anbessa Specialized Hospital (TASH), Ethiopia.MethodsThis observational cross-sectional study employed a validated Amharic WHO-5 wellbeing index to assess SWB. Sociodemographic and clinical data were collected using structured questionnaire. RHD subjects aged 9 years and above were included. Factors associated with SWB were assessed using logistic regression models.ResultsThe study included 384 subjects, females 68.2% (262). Children, < 18 years, constituted one third of study subjects, 32.8% (126). Moderate and severe echocardiographic RHD dominated, 85.9% (330) with no associated comorbidity, 84.4% (324). Only 17.2% (66) had surgical or device intervention. Poor SWB was documented in 9.6% of study subjects (95% CI: 6.88–13.04). On multivariable regression, those with younger age RHD diagnosis, < 20 years, had almost three times higher odds of poor SWB, adjusted odds ratio (aOR) 2.69(95% CI: 1.30–5.58, P 0.008). Those with monthly family income of < 1000 Ethiopian Birr had three times higher odds of poor SWB, aOR 2.97(95% CI: 1.24–7.1, P 0.014). Study subjects who had good medication adherence had reduced odds of poor SWB, aOR 0.37(95% CI: 0.18–0.77, P 0.028). Those who received psychologic support from their families also had reduced odds of poor SWB, aOR 0.26(95% CI: 0.11–0.64, P 0.003).ConclusionPoor SWB was documented in one-tenth of RHD patients. Family income, younger age at RHD diagnosis, medication adherence and psychological support predicted poor SWB. Poor SWB has to be considered and assessed among RHD patients particularly among those with younger age at RHD diagnosis and poor family income. Further mixed studies are recommended to assess how medication adherence and psychological supports associate with positive SWB among RHD patients.

Abstract 13547: Secondary Prevention of Rheumatic Heart Disease in Ethiopia

Introduction: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are significant causes of morbidity and mortality. Ethiopia has a high RHD prevalence but has no national strategic guidelines for preventing RHD and no data on the status of secondary prevention in children with RHD. Generating local information is essential to designing effective prevention and control strategies. This study describes the status of secondary RHD prevention in Ethiopian children. Methods: A multicenter, cross-sectional study was performed in children aged 5-17 years with an echocardiogram-based RHD diagnosis. Participants with congenital heart disease and a recent RHD diagnosis (&lt;1 year) were excluded. Good adherence was defined as at least 80% completion of intramuscular benzathine penicillin (BPG) or oral Amoxicillin within the previous year. The primary outcome measure was adherence to prophylaxis, expressed as a proportion. Covariates included socio-demographics, type and severity of RHD, and ARF recurrence. Chi-square test and Student’s t-test were used to assess differences. Multivariate logistic regression was performed. Results: We included 337 participants with a mean age (±standard deviation) of 12.9±2.6 years. The majority were females (54%) and 73% had severe aortic/mitral disease. BPG (80%) and Amoxicillin (20%) were the prophylaxis of choice. Predictors of good adherence were female sex (P=0.04), use of BPG (0.03), and shorter mean duration of prophylaxis in months (48.5±31.5 vs. 60.7±33, P&lt;0.008). Running out of medicines (35%), interrupted follow-up (27%), and the COVID pandemic (26%) were the three most common reasons for missing prophylaxis. Recurrence of ARF was higher in participants on Amoxicillin (40% vs. 16%, P&lt;0.001) and in those with poor adherence (36.8% vs. 17.9%, P=0.005). Type and duration of prophylaxis (OR 0.5, P =0.02; OR=1.1, P =0.04, respectively), and sex (OR=1.9, P =0.03) were independent predictors of poor adherence. Conclusion: Poor adherence is prevalent in Ethiopian children with RHD. Amoxicillin is a suboptimal option for prophylaxis associated with lower adherence and a higher rate of ARF recurrence.