The most frequent abnormality of the valves involved in rheumatic heart disease is mitral regurgitation. A promising supportive diagnostic tool for rheumatic heart disease is the N-terminal pro-brain natriuretic peptide, which can identify mitral valve damage.

To prove a positive correlation between N-terminal pro-brain natriuretic peptide levels and the severity of mitral regurgitation or accompanied by mild aortic valve dysfunction in children with rheumatic heart disease.

Children aged 1–18 years who had been identified as having rheumatic heart disease with a single mitral regurgitation or accompanied by mild aortic issues at Sanglah General Hospital, Denpasar, met the inclusion criteria for this cross-sectional study. Mitral regurgitation severity was determined using the parameters of vena contracta width, effective regurgitation orifice area, regurgitant jet area, and regurgitation volume. Bivariate analysis using the Spearman correlation test.

From 36 research subjects, the mean age was 11.32 years. In this study, there was a moderate positive correlation between the levels of N-terminal pro-brain natriuretic peptide and the regurgitation jet area. N-terminal pro-brain natriuretic peptide levels also had a moderately positive correlation with the vena contracta width and a weakly positive correlation with the regurgitation volume. Effective regurgitation orifice area and N-terminal pro-brain natriuretic peptide levels did not significantly correlate.

There was a moderately positive correlation between N-terminal pro-brain natriuretic peptide levels and the severity of mitral regurgitation on regurgitation jet area, a moderately positive correlation with the vena contracta width, and a weakly positive correlation with regurgitation volume in rheumatic heart disease patients.

In this study, it was aimed to examine the serum endocan levels in patients with rheumatic aortic regurgitation and to investigate whether it has a value in differentiating it from aortic regurgitation due to bicuspid aortic valve.

Blood samples were collected from patients with rheumatic aortic regurgitation (Group 1), incidentally diagnosed patients with borderline or definite rheumatic aortic regurgitation (Group 2), children with bicuspid aortic valve accompanied by aortic regurgitation (Group 3) and healthy children (Group 4) of similar age.

There were 12 children in Group 1, 13 in Group 2, 25 in Group 3, and 25 in Group 4. Groups were similar in terms of age (p = 0.291). There was no statistically significant difference between median serum endocan levels of Group 1 and Group 2 (p = 0.624), and Group 3 and Group 4 (p = 0.443). Despite that, the median serum endocan levels of Group 1 and Group 2 were significantly higher than that of both Group 3 and Group 4 (p = 0.000 for all).

Our results indicate that serum endocan level can be used to differentiate rheumatic aortic regurgitation from non-rheumatic aortic regurgitation. It is thought that the prognostic role of this marker should be confirmed in long-term, prospective studies with larger samples.

The present study aimed to investigate the outcomes of psychiatric symptoms and family functions on treatment adherence in children, in addition to sociodemographic characteristics and clinical factors related to the disease.

The research sample consisted of 43 children who were followed up with rheumatic heart disease diagnosis during the study. Clinical features were obtained from the patient files. The family assessment device evaluating family functioning and the strengths and difficulties questionnaire scale to screen emotional and behavioural problems in children were used.

Considering the regularity of treatment in our patients, there were 31 (72%) patients adherent to secondary prophylaxis regularly, 7 (6.9%) patients were partially adherent, and 5 (11.6%) patients non-adherent. Patients were divided into treatment adherent (Group 1) and non-adherent (Group 2). There was no statistically significant impact on treatment adherence whether the patients receive enough information, lifestyle, fear of developing adverse effects, fear of addiction, lack of health insurance, difficulties in reaching the drug or hospital. However, the fear of syringes on treatment adherence had an effect statistically significantly (p = 0.047). Forgetting to get a prescription and/or take the drug when the time comes was statistically higher in the non-adherent group (p = 0.009). There was no statistically significant effect of psychosocial factors on treatment adherence between groups.

Providing an effective active recall system, involving primary care workers, providing training on the disease and its management, and a comprehensive pain management programme can improve the process, especially for cases where secondary prophylaxis is missed.

Mitral stenosis is the most common valvular heart disease during pregnancy. When severe, it leads to significant maternal and fetal morbidity and mortality. Percutaneous mitral valve balloon commissurotomy can be performed during pregnancy, and the present study aimed to describe the immediate maternal and fetal outcomes after percutaneous mitral valve balloon commissurotomy was done in a cohort of 23 pregnant patients with severe mitral stenosis in Addis Ababa, Ethiopia.

Included in the current study were all pregnant mothers who had severe rheumatic mitral valve stenosis and who underwent percutaneous mitral valve balloon commissurotomy at the Cardiac Center of Ethiopia over 6-year period. Data were collected through chart abstraction using a structured proforma and then analysed using STATA version 13.0.

Median gestational age was 22 weeks and percutaneous mitral valve balloon commissurotomy was successful resulting in a significant increase in the mean mitral valve area of the group from 0.78 ± 0.20 cm2 to 1.89 ± 0.31 cm2 (p < 0.001). The mean mitral valve inflow gradient of the group was 23.95 ± 6.27 mmHg and 6.80 ± 2.44 mmHg, respectively, before and after the percutaneous mitral valve balloon commissurotomy procedure (p < 0.001). Post-procedure, there was no significant increment in mitral valve incompetence. The mean pulmonary artery pressure of the group decreased from 77.68 ± 23.19 mmHg to 42.31 ± 9.95 mmHg (p < 0.001). There was no fetal or maternal death following the procedure. Pregnancy ended at term gestation for 19/23 (82.6%) of the mothers and the mean birth weight of the neonates was 2800 g.

Percutaneous mitral valve balloon commissurotomy procedure can safely be done for severe symptomatic rheumatic mitral stenosis in pregnancy in our setting.

The present study aimed to quantify the burden of structural heart disease in Nepali children.

We performed a school-based cross-sectional echocardiographic screening study with cluster random sampling among children 5–16 years of age.

Between December 2012 and January 2019, 6573 children (mean age 10.6 ± 2.9 years) from 41 randomly selected schools underwent echocardiographic screening. Structural heart disease was detected in 14.0 per 1000 children (95% CI 11.3–17.1) and was congenital in 3.3 per 1000 (95% CI 2.1–5.1) and rheumatic in 10.6 per 1000 (95% CI 8.3–13.4). Rates of rheumatic heart disease were higher among children attending public as compared to private schools (OR 2.8, 95% CI 1.6–5.2, p = 0.0001).

Rheumatic heart disease accounted for three out of four cases of structural heart disease and was more common among children attending public as compared to private schools.

Rheumatic heart disease is among the leading causes of acquired valvular heart disease in the developing world. However, there is no data available for rheumatic heart disease in the paediatric population of Sabah. This study collected data for acute rheumatic fever admissions among the paediatric population in Sabah over a period of 3 years.

This is a retrospective cohort study. All records for admissions to paediatric wards in Sabah for acute rheumatic fever from January 2016 to December 2018 were collected. The patient records were then traced and required information were collected.

A total of 52 cases of acute rheumatic fever were admitted. It was observed that the incidence of acute rheumatic fever was 74.4 per 100,000 paediatric admissions. Patients from the West Coast Division made up most of the admissions (n = 24, 46.2%). Male patients (n = 35, 67.3%) of the indigenous Kadazan-Dusun ethnicity (n = 21, 40.4%) were most commonly encountered. The mean age at time of presentation was 9.58 years. Most cases admitted (n = 38, 73.1%) were categorised as Priority 1 (severe rheumatic heart disease).

Most patients who were admitted had symptoms of heart failure and were diagnosed with severe rheumatic heart disease. Although this disease is preventable, the incidence in Sabah remains high. This study was limited as we only looked at patients who were admitted and we foresee the real incidence to be higher. Hence, there is an urgent need for a rheumatic heart disease registry in Malaysia to gather more data for prevention and early intervention.

Rheumatic heart disease is the most common cardiac diseases in developing countries including Ethiopia. The current study aimed to describe the immediate surgical outcome following valve surgery for rheumatic heart disease in Ethiopia.

Data were collected through chart abstraction from two centres in Addis Ababa, Ethiopia: the Cardiac Center of Ethiopia and El Ouzier cardiac centre. Included were all patients who were operated for rheumatic valvular heart disease in the mentioned centres by local cardiac surgical team during the period from June 2017 to April 2020. Demographic and clinical characteristics of the study population at admission and within 30 days of the index cardiac surgery were collected. Statistical Package for Social Sciences version 20.0 for windows was used to analyse the data.

Of the 114 patients included in the study (median age 31 years with interquartile range of 23–40), 62 (54.4%) of them were female. Surgical procedures done were triple valve surgery 9 (7.9%) patients, mitral and tricuspid valves 26 (22.8%) patients, double-valve 16 (14.0%) patients, single-valve surgery 50.9% (11 aortic and 47 mitral valves) of patients, redo mitral valve surgery 3 (2.6%) patients, and left maze with mitral valve surgery 2 (1.8%) patients. Of the total, 103 (90.4%) of them had mitral valve surgery. Post-operatively, 5 (4.4%) patients died within 30 days following the index surgery.

Immediate surgical outcome following valve surgery for rheumatic heart disease had excellent outcome in our setting. This evidence can be taken as a show of success in building local capacity to manage rheumatic heart disease surgically.

The aim of this study is to investigate the prevalence of subclinical rheumatic heart disease in schoolchildren aged 5–18 by using portable echocardiography in Ankara, Turkey.

The portable echocardiography screening was performed by a paediatric cardiologist for all of the cases. The mean age of 2550 healthy students was 11.09 ± 2.91 years (1339 females, 1211 males) in three private and three public schools. Echocardiographic studies were assessed according to 2012 World Heart Federation criteria for rheumatic heart disease.

After reviewing the echocardiographic images, 73 students were reevaluated by an advanced echocardiography device in the university hospital. Evidence of definite subclinical rheumatic heart disease was found in 39 students (15/1000) and borderline rheumatic heart disease in 20 students (8/1000). No children had any clinical symptoms. The mean age of children diagnosed with rheumatic heart disease and borderline rheumatic heart disease is 12.4 and 11.4 years, respectively. The risk of rheumatic heart disease was found to be increased sevenfold in girls between 14 and 18 years. We surprisingly observed that the prevalence of definite rheumatic heart disease in private schools located in high-income areas of Ankara was higher than that in public schools.

This is the first and largest single-centred echocardiographic screening study for subclinical rheumatic heart disease in Turkish schoolchildren. The frequency of rheumatic heart disease has been found to be 15/1000. This finding is similar to those of recent echocardiographic screening studies performed in middle and high-risk populations. We conclude that to decrease the burden of rheumatic heart disease, echocardiographic screening studies are necessary, and long-term follow-up of children with echocardiographically diagnosed subclinical rheumatic heart disease is needed.

There is overlap between pathological mitral regurgitation seen in borderline rheumatic heart disease using World Heart Federation echocardiography criteria and physiologic regurgitation found in normal children. One possible contributing factor is higher rates of anaemia in endemic countries.

To investigate the contribution of anaemia as a potential confounder in the diagnosis of rheumatic heart disease detected in echocardiographic screening.

A novel Server 2012 data warehouse tool was used to incorporate haematology and echocardiography databases. The study included a convenience sample of patients from 5 to 18 years old without structural or functional heart disease that had a haemoglobin value within 1 month prior to an echocardiogram. Echocardiogram images were reviewed to determine presence or absence of World Heart Federation criteria for rheumatic heart disease. The rate of rheumatic heart disease among anaemic and non-anaemic children according to gender- and age-based norms groups was compared.

Of the 935 patients who met the study inclusion criteria, 406 were classified as anaemic. There was no difference in the rate of echocardiograms meeting criteria for borderline rheumatic heart disease in anaemic (2.0%, 95% CI 0.6–3.3%) and non-anaemic children (1.3%, 95% CI 0.3–2.3%). However, there was a statistically significant increase in rates of mitral regurgitation of unclear significance among anaemic versus non-anaemic patients (8.6 versus 3.6%; p = 0.0012).

Anaemia does not increase the likelihood of meeting echocardiographic criteria for borderline rheumatic heart disease. Future studies should evaluate for the correlation between anaemia and mitral regurgitation in endemic settings.

The aim of the present study was to describe the clinical course, laboratory tests, and the cardiac involvement in rheumatic carditis patients in functional class III and IV, submitted to pulse therapy combined with oral prednisone.

A total of 120 patients with severe carditis due to acute rheumatic fever were treatment with three cycles of pulse therapy combined with oral corticosteroids. The patients were followed up from the hospital admission until the end of the treatment and returned after 30, 60, and 90 days to control. The patients were evaluated by clinical, laboratory, and transthoracic echocardiogram.

In total, 23 (19.2%) patients at first attack of rheumatic fever and 97 (80.8%) with recurrent carditis were evaluated. Cardiac surgery was performed in 8 (6.6%) patients. The patients showed improved laboratory and radiological parameters (p<0.001) and were discharged, 74 (61.7%) in functional class I and 46 (38.3%) in functional class II. Hospitalisation time ranged from 21 to 176 days, with a mean of 69.1 days. Reduction of left atrium and ventricle diameters was observed, measured by means of transthoracic echocardiography, at hospital admission and discharge (p<0.001). None of the patients experienced rebound.

The pulse therapy was effective in controlling severe rheumatic carditis and the oral corticosteroid prevented rebound episodes. Prolonged hospital stay was required for the clinical stabilisation of patients and to avoid the interruption of medication.

Echocardiographic screening represents an opportunity for reduction in the global burden of rheumatic heart disease. A focussed single-view screening protocol could allow for the rapid training of healthcare providers and screening of patients.

The aim of this study was to determine the sensitivity and specificity of a focussed single-view hand-held echocardiographic protocol for the diagnosis of rheumatic heart disease in children.

A total of nine readers were divided into three reading groups; each interpreted 200 hand-held echocardiography studies retrospectively as screen-positive, if mitral regurgitation ⩾1.5 cm and/or any aortic insufficiency were observed, or screen-negative from a pooled study library. The performance of experts receiving focussed hand-held protocols, non-experts receiving focussed hand-held protocols, and experts receiving complete hand-held protocols were determined in comparison with consensus interpretations on fully functional echocardiography machines.

In all, 587 studies including 76 on definite rheumatic heart disease, 122 on borderline rheumatic heart disease, and 389 on normal cases were available for analysis. The focussed single-view protocol had a sensitivity of 81.1%, specificity of 75.5%, negative predictive value of 88.5%, and a positive predictive value of 63.2%; expert readers had higher specificity (86.1 versus 64.8%, p<0.01) but equal sensitivity. Sensitivity – experts, 96% and non-experts, 95% – and negative predictive value – experts, 99% and non-experts, 98% – were better for definite rheumatic heart disease. False-positive screening studies resulting from erroneous identification of mitral regurgitation and aortic insufficiency colour jets increased with shortened protocols and less experience (p<0.01).

Our data support a focussed screening protocol limited to parasternal long-axis images. This holds promise in making echocardiographic screening more practical in regions where rheumatic heart disease remains endemic.

Identification of patients with latent rheumatic heart disease by echocardiography presents a unique opportunity to prevent disease progression. Myocardial strain is a more sensitive indicator of cardiac performance than traditional measures of systolic function.

The objective of this study was to test the hypothesis that abnormalities in myocardial strain may be present in children with latent rheumatic heart disease.

Standard echocardiography images with electrocardiogram gating were obtained from Ugandan children found to have latent rheumatic heart disease as well as control subjects. Traditional echocardiography measures of systolic function were obtained, and offline global longitudinal strain analysis was performed. Comparison between groups was performed using strain as a continuous (Mann–Whitney U-test) and categorical (cut-off 5th percentile for age) variable.

Our study included 14 subjects with definite rheumatic heart disease, 13 with borderline rheumatic heart disease, and 112 control subjects. None of the subjects had abnormal left ventricular size or ejection fraction. Global longitudinal strain was lower than the 5th percentile in 44% of the subjects with any rheumatic heart disease (p=0.002 versus controls) and 57% of the subjects with definite rheumatic heart disease (p=0.03). The mean absolute strain values were significantly lower when comparing subjects with any rheumatic heart disease with controls (20.4±3.95 versus 22.4±4.35, p=0.025) and subjects with definite rheumatic heart disease with controls (19.9±4.25 versus 22.4±4.35, p=0.033).

Global longitudinal strain is decreased in subjects with rheumatic heart disease in the absence of abnormal systolic function. Larger studies with longer-term follow-up are required to determine whether there is a role for strain to help better understand the pathophysiology of latent rheumatic heart disease.

Echocardiographic screening for rheumatic heart disease in asymptomatic children may result in early diagnosis and prevent progression. Physician-led screening is not feasible in Malawi. Task shifting to mid-level providers such as clinical officers may enable more widespread screening.

With short-course training, clinical officers can accurately screen for rheumatic heart disease using focussed echocardiography.

A total of eight clinical officers completed three half-days of didactics and 2 days of hands-on echocardiography training. Clinical officers were evaluated by performing screening echocardiograms on 20 children with known rheumatic heart disease status. They indicated whether children should be referred for follow-up. Referral was indicated if mitral regurgitation measured more than 1.5 cm or there was any measurable aortic regurgitation. The κ statistic was calculated to measure referral agreement with a paediatric cardiologist. Sensitivity and specificity were estimated using a generalised linear mixed model, and were calculated on the basis of World Heart Federation diagnostic criteria.

The mean κ statistic comparing clinical officer referrals with the paediatric cardiologist was 0.72 (95% confidence interval: 0.62, 0.82). The κ value ranged from a minimum of 0.57 to a maximum of 0.90. For rheumatic heart disease diagnosis, sensitivity was 0.91 (95% confidence interval: 0.86, 0.95) and specificity was 0.65 (95% confidence interval: 0.57, 0.72).

There was substantial agreement between clinical officers and paediatric cardiologists on whether to refer. Clinical officers had a high sensitivity in detecting rheumatic heart disease. With short-course training, clinical officer-led echo screening for rheumatic heart disease is a viable alternative to physician-led screening in resource-limited settings.

The aim of the present study was to investigate the relationships between red blood cell distribution width, platelet distribution width, and mean platelet volume and the presence and severity of valvular involvement in patients with rheumatic heart disease.

Between April, 2012 and December, 2015, 151 patients who were admitted to the Pediatric Cardiology Unit with diagnosis of rheumatic heart disease and 148 healthy children were included to our study. Transthoracic echocardiography for all children was performed, and the values of red blood cell distribution width, platelet distribution width, and mean platelet volume, besides other blood count parameters, erythrocyte sedimentation rate, and C-reactive protein levels were recorded.

Red blood cell distribution width, platelet distribution width, mean platelet volume, and C-reactive protein levels were significantly higher in patients with rheumatic heart disease when compared with healthy controls (p<0.01). Red blood cell distribution width was positively correlated with both C-reactive protein (r=0.271, p=0.035) and erythrocyte sedimentation rate (r=0.308, p=0.006). When single valve involvement was compared with both aortic valve and mitral valve involvement in the study group, red blood cell distribution width and platelet distribution width were higher in patients with double valve involvement; however, this was not statistically significant (p>0.05).

This is the first study in children with rheumatic heart disease that demonstrated significantly increased red blood cell distribution width, platelet distribution width, and mean platelet volume levels, as well as evaluated all three parameters together. Furthermore, red blood cell distribution width values in the chronical period of acute rheumatic fever, due to the positive correlation with the other chronic inflammatory markers, may help make the diagnosis in children.

Rheumatic heart disease causes substantial morbidity in children in low-income countries. School-based echocardiographic screening has been suggested as a means to identify children with latent disease; however, little is known about the experience of children and teachers participating in screenings. The aim of our study was to assess students’ and teachers’ experience of school-based echocardiographic screening and identify areas for improvement.

A school-based echocardiographic screening programme was conducted in five schools in Northern Uganda in 2013. After 8 months, an age- and gender-stratified population that included 5% of the participating students and teachers completed a questionnaire via an in-person interview. Responses were reviewed by question and coded to identify key themes.

A total of 255 students (mean 10.7 years; 48% male) and 35 teachers participated in our study. In total, 95% of the students and 100% of the teachers were happy to have participated in the screening; however, students reported feeling scared (35%) and nervous (48%) during the screening process. Programmatic strengths included the following: knowing one’s health status, opportunity to receive treatment, and staff interactions. Although 43% of the patients did not suggest a change with open-ended questioning, concerns regarding privacy, fear of the screening process, and a desire to include others in the community were noted.

School-based echocardiographic rheumatic heart disease screening was well received by students and teachers. Future programmes would likely benefit from improved pre-screening education regarding the screening process and diagnosis of rheumatic heart disease. Furthermore, education of teachers and students could improve screening perception and establish realistic expectations regarding the scope of screening.

Rheumatic heart disease is an inflammatory disease of cardiac tissue. The underlying pathogenic mechanisms highlight a complex interplay of immunological, genetic, and environmental factors. The aim of the present study was to investigate whether IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms could be associated with susceptibility and/or severity of rheumatic heart disease among patients from the Egyptian population.

A cohort of 140 Egyptian children with rheumatic heart disease and 100 healthy controls were enrolled in this case–control study. Genotyping for IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms was carried out for all patients using a polymerase chain reaction-based analysis.

No significant difference in the distribution of genotypes and allelic frequencies between rheumatic heart disease cases and controls for IL-4 (intron 3) (p=0.17; OR 1.07, 95% CI 0.82–3.74) and IL-10 (-1082) (p=0.49; OR 1.03, 95% CI 0.65–2.71) gene polymorphisms was observed. Further categorisation of patients into mitral valve disease and combined valve disease subgroups showed that cases with mitral valve disease have significantly higher frequency of the RP2 allele of IL-4 (intron 3) (p=0.03; OR 2.98, 95% CI 1.93–6.15) and the G allele of IL-10 (-1082) (p=0.04; OR 2.14, 95% CI 1.62–4.95) when compared with controls.

Our study shows that IL-4 (intron 3) and IL-10 (-1082) gene polymorphisms are not significantly associated with susceptibility to rheumatic heart disease, but they might play a role in the pathogenesis of patients with mitral valve disease.