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A rapid echocardiographic screening protocol for rheumatic heart disease in Samoa: a high prevalence of advanced disease
Abstract
Background
Echocardiography has been proposed as a method to screen children for rheumatic heart disease. The World Heart Federation has established guidelines for echocardiographic screening. In this study, we describe a rapid echocardiogram screening protocol according to the World Heart Federation guidelines in Samoa, endemic for rheumatic heart disease.
Methods
We performed echocardiogram screening in schoolchildren in Samoa between 2013 and 2015. A brief screening echocardiogram was performed on all students. Children with predefined criteria suspicious for rheumatic hear diseases were referred for a more comprehensive echocardiogram. Complete echocardiograms were classified according to the World Heart Federation guidelines and severity of valve disease.
Results
Echocardiographic screening was performed on 11,434 children, with a mean age of 10.2 years; 51% of them were females. A total of 558 (4.8%) children underwent comprehensive echocardiography, including 49 students who were randomly selected as controls. Definite rheumatic heart disease was observed in 115 students (10.0 per 1000): 92 students were classified as borderline (8.0 per 1000) and 23 with CHD. Advanced disease was identified in 50 students (4.4 per 1000): 15 with severe mitral regurgitation, five with severe aortic regurgitation, 11 with mitral stenoses, and 19 with mitral and aortic valve disease.
Conclusions
We successfully applied a rapid echocardiographic screening protocol to a large number of students over a short time period – 28 days of screening over a 3-year time period – to identify a high prevalence of rheumatic heart disease. We also reported a significantly higher rate of advanced disease compared with previously published echocardiographic screening programmes.
... Allen et al. carried out a screening programme on 11 434 Samoan children using a 60 s focused echocardiogram with a comprehensive echocardiogram as a follow-up for children with suspicious findings. 59 Their rapid screening protocol entailed a parasternal long-axis view, focused on the mitral and aortic valves with and without colour Doppler for each student. Definite RHD was found in 115 students (10.0 per 1000), of which 92 students were classified as borderline (8.0 per 1000), similar to the previously reported prevalence of RHD in Samoa. ...
Rheumatic heart disease (RHD) is the most common cause of valvular heart disease worldwide, affecting millions, especially in low- and middle-income countries. Multiple imaging modalities such as cardiac CT, cardiac MRI, and three-dimensional echocardiography may be utilized in diagnosing, screening, and managing RHD. However, two-dimensional transthoracic echocardiography remains the cornerstone of imaging in RHD. Criteria developed by the World Heart Foundation in 2012 sought to unify the diagnostic imaging criteria for RHD, but concerns remain regarding their complexity and reproducibility. In the intervening years, further measures have been developed to find a balance between simplicity and accuracy. Nonetheless, there remain significant unresolved problems within imaging in RHD, including the development of a practical and sensitive screening tool to identify patients with RHD. The emergence of handheld echocardiography has the potential to revolutionize RHD management in resource-poor settings, but its role as a screening or diagnostic tool is yet to be fully established. The dramatic evolution of imaging modalities over the last few decades has not addressed RHD compared to other forms of structural heart disease. In this review, we examine the current and latest developments concerning cardiac imaging and RHD.
... Echocardiography exceeds clinical examination in detection of RHD [5] and in 2012 the World Heart Federation (WHF) published standardized echocardiographic criteria for diagnosis of RHD [6]. These criteria have been applied in several studies [7][8][9] and have been shown to increase the detection rate of RHD [10]. However, echocardiographic studies aiming to determine the prevalence of RHD in endemic areas continues to be in demand. ...
Background
Rheumatic heart disease (RHD) continues to be a burden in low- and middle-income countries and prevalence estimates are lacking from South America. We aimed to determine the prevalence of RHD in the Brazilian Amazon Basin.
Methods
We examined a random sample of adults (≥18 years) from the general population, who underwent echocardiographic image acquisition by a medical doctor. All images were analyzed according to (i) the 2012 World Heart Federation criteria and (ii) a simplified algorithm for RHD from a previously validated risk score (categories: low-, medium-, high-risk) which involved assessment of the mitral valve (leaflet thickening and excessive motion, regurgitation jet length) and aortic valve (thickening and any regurgitation).
Results
A total of 488 adults were screened (mean age 40 ± 15 years, 38% men). The prevalence of RHD was 39/1000 adults (n = 17 definite and n = 2 borderline). Fourteen (74%) had pathological mitral regurgitation, four (21%) mitral stenosis, 0 (0%) pathological aortic regurgitation and six (32%) both mitral and aortic valve disease. None had a prior diagnosis of RHD, 10 (53%) had positive cardiac auscultation and two (11%) reported a history of rheumatic fever. The simplified algorithm identified four (21%) adults as low-risk, six (32%) as intermediate, and nine (47%) as high risk.
Conclusions
The prevalence of RHD was 39/1000 in adults from the Brazilian Amazon Basin, indicating the need for screening programs in remote areas. A simplified model was only able to categorize every second case of RHD as high-risk. External validation of simplified screening models to increase feasibility in clinical practice are encouraged.
... Our echocardiography-based screening study identified a very high prevalence of RHD among young people in Timor-Leste, comparable with the highest documented rates of RHD else- where (Box 5). 1,6,[16][17][18][19][20][21][22][23][24] The prevalence of definite RHD in our study (18 cases per 1000 population) is higher than the rate for Fiji (7 cases per 1000), and similar to that in other regional neigh- bours, including the Top End of Australia (15 cases per 1000 for Indigenous Australians at high risk). 1, 18 The prevalence in Timor-Leste may be higher than esti- mated; we did not detect any cases of mitral stenosis or other forms of severe RHD, and it is possible that stu- dents with severe RHD were too unwell to attend school, and therefore not screened. ...
Objectives:
To determine the prevalence of rheumatic heart disease (RHD) in school-aged children and young people in Timor-Leste.
Design:
Prospective cross-sectional survey. Echocardiography was performed by Australian cardiologists to determine the presence of RHD. Demographic data were also collected. Patients in whom RHD was detected were entered into a register to allow monitoring of adherence to secondary prophylaxis; the first dose of benzathine penicillin G (BPG) was administered on the day of screening.
Setting:
Schools in urban (Dili) and rural (Ermera) Timor-Leste.
Participants:
School students aged 5-20 years.
Outcome measures:
Definite and borderline RHD, as defined by World Heart Federation echocardiographic criteria.
Results:
1365 participants were screened; their median age was 11 years (IQR, 9-14 years), and 53% were girls. The estimated prevalence of definite RHD was 18.3 cases per 1000 population (95% CI, 12.3-27.0 per 1000), and of definite or borderline RHD 35.2 per 1000 (95% CI, 26.5-46.4 per 1000). Definite (adjusted odds ratio [aOR], 3.5; 95% CI, 1.3-9.4) and definite or borderline RHD (aOR, 2.7; 95% CI, 1.4-5.2) were more prevalent among girls than boys. Eleven children (0.8%) had congenital heart disease. Of the 25 children in whom definite RHD was identified, 21 (84%) received education and a first dose of BPG on the day of screening; all 25 have since received education about primary care for RHD and have commenced penicillin prophylaxis.
Conclusions:
The rates of RHD in Timor-Leste are among the highest in the world, and prevalence is higher among girls than boys. Community engagement is essential for ensuring follow-up and the effective delivery of secondary prophylaxis.
This systematic review and meta-analysis aimed to provide a contemporaneous estimate of the global burden of rheumatic heart disease (RHD) from echocardiographic population-based studies. We searched multiple databases between January 01, 1996 and October 17, 2017. Random-effect meta-analysis was used to pool data. We included 82 studies (1,090,792 participant) reporting data on the prevalence of RHD and 9 studies on the evolution of RHD lesions. The pooled prevalence of RHD was 26.1‰ (95%CI 19.2–33.1) and 11.3‰ (95%CI 7.2–16.2) for studies which used the World Heart Federation (WHF) and World Health Organization (WHO) criteria, respectively. The prevalence of RHD varied inversely with the level of a country’s income, was lower with the WHO criteria compared to the WHF criteria, and was lowest in South East Asia. Definite RHD progressed in 7.5% (95% CI 1.5–17.6) of the cases, while 60.7% (95% CI 42.4–77.5) of cases remained stable over the course of follow-up. The proportion of cases borderline RHD who progressed to definite RHD was 11.3% (95% CI 6.9–16.5). The prevalence of RHD across WHO regions remains high. The highest prevalence of RHD was noted among studies which used the WHF diagnostic criteria. Definite RHD tends to progress or remain stable over time.
Background
Echocardiographic screening in school-aged children can detect rheumatic heart disease (RHD) prior to the manifestation of symptoms of heart failure. The challenge is making this practical and affordable on a global scale. This study aims to evaluate the diagnostic utility of an ultra-abbreviated echocardiographic screening protocol involving a single parasternal-long-axis-view-sweep of the heart (SPLASH) in two dimensional (2D) and colour Doppler imaging (index test).
Methods
This prospective study of diagnostic accuracy compared the diagnostic utility of the index screening test with a comprehensive reference test (standard echocardiographic screening protocols) as per World Heart Federation (WHF) echocardiographic criteria. School students in Timor-Leste aged 5–20 years were enrolled. Both index and reference test images were acquired by cardiologists on Vivid I or Q machines (GE Healthcare, Marlborough, MA, USA).
Results
A total of 1,365 participants were screened; median age was 11 years. The estimated prevalence of definite and borderline RHD was 35.2 per 1,000. Congenital heart disease was identified in 11 children (0.8%) with two needing cardiac surgery. Abnormal SPLASH views were found in 109/1365 (7.99%). No cases of RHD or significant congenital heart disease were missed. Sensitivity and specificity of the abbreviated protocol for detecting RHD were 1.0 and 0.95 respectively.
Conclusions
A simplified echocardiography screening protocol using SPLASH is highly sensitive and specific and could significantly improve the efficiency of RHD screening. It has the potential to expedite training of health workers whilst protecting the modesty of students.
Importance
Although rheumatic heart disease has been nearly eradicated in high-income countries, 3 in 4 children grow up in parts of the world where it is still endemic.Objectives
To determine the prevalence of clinically silent and manifest rheumatic heart disease as a function of age, sex, and socioeconomic status and to estimate age-specific incidence.Design, Setting, and Participants
In this school-based cross-sectional study with cluster sampling, 26 schools in the Sunsari district in Eastern Nepal with 5467 eligible children 5 to 15 years of age were randomly selected from 595 registered schools. After exclusion of 289 children, 5178 children were enrolled in the present study from December 12, 2012, through September 12, 2014. Data analysis was performed from October 1, 2014, to April 15, 2015. Exposures
Demographic and socioeconomic characteristics were acquired in a standardized interview by means of a questionnaire customized to the age of the children. A focused medical history was followed by a brief physical examination. Cardiac auscultation and transthoracic echocardiography were performed by 2 independent physicians.Main Outcomes and Measures
Rheumatic heart disease according to the World Heart Federation criteria.Results
The median age of the 5178 children enrolled in the study was 10 years (interquartile range, 8-13 years), and 2503 (48.3%) were female. The prevalence of borderline or definite rheumatic heart disease was 10.2 (95% CI, 7.5-13.0) per 1000 children and increased with advancing age from 5.5 (95% CI, 3.5-7.5) per 1000 children 5 years of age to 16.0 (95% CI, 14.9-17.0) in children 15 years of age, whereas the mean incidence remained stable at 1.1 per 1000 children per year. Children with rheumatic heart disease were older than children without rheumatic heart disease (median age [interquartile range], 11 [9-14] years vs 10 [8-13] years; P = .03), more commonly female (34 [64.2%] vs 2469 [48.2%]; P = .02), and more frequently went to governmental schools (40 [75.5%] vs 2792 [54.5%]; P = .002). Silent disease (n = 44) was 5 times more common than manifest disease (n = 9).Conclusions and Relevance
Rheumatic heart disease affects 1 in 100 schoolchildren in Eastern Nepal, is primarily clinically silent, and may be more common among girls. The overall prevalence and the ratio of manifest to subclinical disease increase with advancing age, whereas the incidence remains stable at 1.1 per 1000 children per year. Early detection of silent disease may help prevent progression to severe valvular damage.
Objectives:
Handheld echocardiography (HAND) has good sensitivity and specificity for rheumatic heart disease (RHD) when performed by cardiologists. However, physician shortages in RHD-endemic areas demand less-skilled users to make RHD screening practical. We examine nurse performance and interpretation of HAND using a simplified approach for RHD screening.
Methods:
Two nurses received training on HAND and a simplified screening approach. Consented students at two schools in Uganda were eligible for participation. A simplified approach (HAND performed and interpreted by a non-expert) was compared with the reference standard (standard portable echocardiography, performed and interpreted by experts according to the 2012 World Heart Federation guidelines). Reasons for false-positive and false-negative HAND studies were identified.
Results:
A total of 1002 children were consented, with 956 (11.1 years, 41.8% male) having complete data for review. Diagnoses included: 913 (95.5%) children were classified normal, 32 (3.3%) borderline RHD and 11 (1.2%) definite RHD. The simplified approach had a sensitivity of 74.4% (58.8% to 86.5%) and a specificity of 78.8% (76.0% to 81.4%) for any RHD (borderline and definite). Sensitivity improved to 90.9% (58.7% to 98.5%) for definite RHD. Identification and measurement of erroneous colour jets was the most common reason for false-positive studies (n=164/194), while missed mitral regurgitation and shorter regurgitant jet lengths with HAND were the most common reasons for false-negative studies (n=10/11).
Conclusions:
Non-expert-led HAND screening programmes offer a potential solution to financial and workforce barriers that limit widespread RHD screening. Nurses trained on HAND using a simplified approach had reasonable sensitivity and specificity for RHD screening. Information on reasons for false-negative and false-positive screening studies should be used to inform future training protocols, which could lead to improved screening performance.
In Africa, screening for asymptomatic rheumatic heart disease (RHD) has been conducted in single communities using non-standardised echocardiographic criteria. The use of different diagnostic criteria has led to widely variable estimates of the prevalence of RHD in the same communities.
Randomly selected school pupils, from 4 to 24 years of age in Bonteheuwel and Langa communities of Cape Town, South Africa, and Jimma, Ethiopia, respectively, were screened for RHD according to standardised evidence-based echocardiographic diagnostic criteria of the World Heart Federation (WHF).
We screened 4720 scholars. In South Africa (n=2720), 1604 (58.9%) were female and the mean age was 12.2±4.2 years. In Ethiopia (n=2000), 1012 (50.6%) were female and the mean age was 10.7±2.5 years. Echocardiographic screening revealed 55 cases of definite and borderline RHD by WHF criteria in South Africa and 61 cases in Ethiopia, corresponding to a prevalence of 20.2 cases per 1000 (95% CI 15.3 to 26.2) and 31 cases per 1000 (95% CI 23.4 to 39.0), respectively. The odds of detecting a scholar with RHD in Ethiopia were 1.5 times higher than in South Africa (OR 1.5; 95% CI 1.04 to 2.2, p=0.02). The prevalence of RHD was 27 cases per 1000 (95% CI 19.3 to 36.8) in Langa, and 12.5 cases per 1000 (95% CI 7.1 to 20.2) in Bonteheuwel. The odds of detecting a schoolchild with RHD in Langa compared with Bonteheuwel were 2.2 (OR 2.2; 95% CI 1.2 to 4.2, p=0.0071).
There were significant differences in detecting asymptomatic RHD in school pupils of different countries and in different communities within a country in sub-Saharan Africa. The variation in the prevalence of RHD between countries and communities has important implications for the modelling of the global burden of RHD.
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Screening for rheumatic heart disease (RHD) requires workers skilled in echocardiography, which typically involves prolonged, specialized training. Task shifting echocardiographic screening to nonexpert health workers may be a solution in settings with limited human resources. An 8-week training program was designed to train health workers without any prior experience in focused echocardiography for RHD screening. Seven health workers participated. At the completion of training, the health workers performed unsupervised echocardiography on 16 volunteer children with known RHD status. A pediatric cardiologist assessed image quality. Participants provided qualitative feedback. The quality of echocardiograms were high at completion of training (55 of 56 were adequate for diagnosis) and all cases of RHD were identified. Feedback was strongly positive. Training health workers to perform focused echocardiography for RHD screening is feasible. After systematic testing for accuracy, this training program could be adapted in other settings seeking to expand echocardiographic capabilities.
Background: Up-to-date evidence about levels and trends in disease and injury incidence, prevalence, and years lived with disability (YLDs) is an essential input into global, regional, and national health policies. In the Global Burden of Disease Study 2013 (GBD 2013), we estimated these quantities for acute and chronic diseases and injuries for 188 countries between 1990 and 2013.
Methods: Estimates were calculated for disease and injury incidence, prevalence, and YLDs using GBD 2010 methods with some important refinements. Results for incidence of acute disorders and prevalence of chronic disorders are new additions to the analysis. Key improvements include expansion to the cause and sequelae list, updated systematic reviews, use of detailed injury codes, improvements to the Bayesian meta-regression method (DisMod-MR), and use of severity splits for various causes. An index of data representativeness, showing data availability, was calculated for each cause and impairment during three periods globally and at the country level for 2013. In total, 35 620 distinct sources of data were used and documented to calculated estimates for 301 diseases and injuries and 2337 sequelae. The comorbidity simulation provides estimates for the number of sequelae, concurrently, by individuals by country, year, age, and sex. Disability weights were updated with the addition of new population-based survey data from four countries.
Findings: Disease and injury were highly prevalent; only a small fraction of individuals had no sequelae. Comorbidity rose substantially with age and in absolute terms from 1990 to 2013. Incidence of acute sequelae were predominantly infectious diseases and short-term injuries, with over 2 billion cases of upper respiratory infections and diarrhoeal disease episodes in 2013, with the notable exception of tooth pain due to permanent caries with more than 200 million incident cases in 2013. Conversely, leading chronic sequelae were largely attributable to non-communicable diseases, with prevalence estimates for asymptomatic permanent caries and tension-type headache of 2·4 billion and 1·6 billion, respectively. The distribution of the number of sequelae in populations varied widely across regions, with an expected relation between age and disease prevalence. YLDs for both sexes increased from 537·6 million in 1990 to 764·8 million in 2013 due to population growth and ageing, whereas the age-standardised rate decreased little from 114·87 per 1000 people to 110·31 per 1000 people between 1990 and 2013. Leading causes of YLDs included low back pain and major depressive disorder among the top ten causes of YLDs in every country. YLD rates per person, by major cause groups, indicated the main drivers of increases were due to musculoskeletal, mental, and substance use disorders, neurological disorders, and chronic respiratory diseases; however HIV/AIDS was a notable driver of increasing YLDs in sub-Saharan Africa. Also, the proportion of disability-adjusted life years due to YLDs increased globally from 21·1% in 1990 to 31·2% in 2013.
Interpretation: Ageing of the world's population is leading to a substantial increase in the numbers of individuals with sequelae of diseases and injuries. Rates of YLDs are declining much more slowly than mortality rates. The non-fatal dimensions of disease and injury will require more and more attention from health systems. The transition to non-fatal outcomes as the dominant source of burden of disease is occurring rapidly outside of sub-Saharan Africa. Our results can guide future health initiatives through examination of epidemiological trends and a better understanding of variation across countries.
The World Heart Federation (WHF) guidelines for rheumatic heart disease (RHD) are designed for a standard portable echocardiography (STAND) machine. A recent study in a tertiary care centre demonstrated that they also had good sensitivity and specificity when modified for use with handheld echocardiography (HAND). Our study aimed to evaluate the performance of HAND for early RHD diagnosis in the setting of a large-scale field screening.
STAND was performed in 4773 children in Gulu, Uganda, with 10% randomly assigned to also undergo HAND. Additionally, any child with mitral or aortic regurgitation also underwent HAND. Studies were performed by experienced echocardiographers and blindly reviewed by cardiologists using 2012 WHF criteria, which were modified slightly for HAND-due to the lack of spectral Doppler capability. Paired echocardiograms were performed in 1420 children (mean age 10.8 and 53% female), resulting in 1234 children who were normal, 133 who met criteria for borderline RHD, 47 who met criteria for definite RHD, and 6 who had other diagnoses. HAND had good sensitivity and specificity for RHD detection (78.9 and 87.2%, respectively), but was most sensitive for definite RHD (97.9%). Inter- and intra-reviewer agreement ranged between 66-83 and 71.4-94.1%, respectively.
HAND has good sensitivity and specificity for diagnosis of early RHD, performing best for definite RHD. Protocols for RHD detection utilizing HAND will need to include confirmation by STAND to avoid over-diagnosis. Strategies that evaluate simplified screening protocols and training of non-physicians hold promise for more wide spread deployment of HAND-based protocols.
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.
Over the past 5 years, the advent of echocardiographic screening for rheumatic heart disease (RHD) has revealed a higher RHD burden than previously thought. In light of this global experience, the development of new international echocardiographic guidelines that address the full spectrum of the rheumatic disease process is opportune. Systematic differences in the reporting of and diagnostic approach to RHD exist, reflecting differences in local experience and disease patterns. The World Heart Federation echocardiographic criteria for RHD have, therefore, been developed and are formulated on the basis of the best available evidence. Three categories are defined on the basis of assessment by 2D, continuous-wave, and color-Doppler echocardiography: 'definite RHD', 'borderline RHD', and 'normal'. Four subcategories of 'definite RHD' and three subcategories of 'borderline RHD' exist, to reflect the various disease patterns. The morphological features of RHD and the criteria for pathological mitral and aortic regurgitation are also defined. The criteria are modified for those aged over 20 years on the basis of the available evidence. The standardized criteria aim to permit rapid and consistent identification of individuals with RHD without a clear history of acute rheumatic fever and hence allow enrollment into secondary prophylaxis programs. However, important unanswered questions remain about the importance of subclinical disease (borderline or definite RHD on echocardiography without a clinical pathological murmur), and about the practicalities of implementing screening programs. These standardized criteria will help enable new studies to be designed to evaluate the role of echocardiographic screening in RHD control.
Epidemiologic studies of the prevalence of rheumatic heart disease have used clinical screening with echocardiographic confirmation of suspected cases. We hypothesized that echocardiographic screening of all surveyed children would show a significantly higher prevalence of rheumatic heart disease.
Randomly selected schoolchildren from 6 through 17 years of age in Cambodia and Mozambique were screened for rheumatic heart disease according to standard clinical and echocardiographic criteria.
Clinical examination detected rheumatic heart disease that was confirmed by echocardiography in 8 of 3677 children in Cambodia and 5 of 2170 children in Mozambique; the corresponding prevalence rates and 95% confidence intervals (CIs) were 2.2 cases per 1000 (95% CI, 0.7 to 3.7) for Cambodia and 2.3 cases per 1000 (95% CI, 0.3 to 4.3) for Mozambique. In contrast, echocardiographic screening detected 79 cases of rheumatic heart disease in Cambodia and 66 cases in Mozambique, corresponding to prevalence rates of 21.5 cases per 1000 (95% CI, 16.8 to 26.2) and 30.4 cases per 1000 (95% CI, 23.2 to 37.6), respectively. The mitral valve was involved in the great majority of cases (87.3% in Cambodia and 98.4% in Mozambique).
Systematic screening with echocardiography, as compared with clinical screening, reveals a much higher prevalence of rheumatic heart disease (approximately 10 times as great). Since rheumatic heart disease frequently has devastating clinical consequences and secondary prevention may be effective after accurate identification of early cases, these results have important public health implications.
Rheumatic heart disease (RHD) affects at least 32.9 million people worldwide and ranks as a leading cause of death and disability in low-income and middle-income countries (LMICs). Echocardiographic screening has been demonstrated to be a powerful tool for early RHD detection, and holds potential for global RHD control. However, national screening programmes have not emerged. Major barriers to implementation include the lack of human and financial resources in LMICs. Here, we focus on recent research advances that could make echocardiographic screening more practical and affordable, including handheld echocardiography devices, simplified screening protocols and task shifting of echocardiographic screening to non-experts. Additionally, we highlight some important remaining questions before echocardiographic screening can be widely recommended, including demonstration of cost-effectiveness, assessment of the impact of screening on children and communities, and determining the importance of latent RHD. While a single strategy for echocardiographic screening in all high-prevalence areas is unlikely, we believe recent advancements are bringing the public health community closer to developing sustainable programmes for echocardiographic screening.
The medical profession should play a central role in evaluating evidence related to drugs, devices, and procedures for detection, management, and prevention of disease. When properly applied, expert analysis of available data on the benefits and risks of these therapies and procedures can improve
To estimate the echocardiography confirmed prevalence of rheumatic heart disease (RHD) in school children in Fiji.
Cross-sectional observational study.
Ten primary schools in Fiji.
School children aged 5-14years.
Each child had an echocardiogram performed by an echocardiographic technician subsequently read by a paediatric cardiologist not involved with field screening, and auscultation performed by a paediatrician.
Echocardiographic criteria for RHD diagnosis were based on those previously published by the National Institutes of Health (NIH) and World Health Organization (WHO), and data were also analyzed using the new World Heart Federation (WHF) criteria. Prevalence figures were calculated with binomial 95% confidence intervals.
Using the modified NIH/WHO criteria the prevalence of definite RHD prevalence was 7.2 cases per 1000 (95% CI 3.7-12.5), and the prevalence of probable RHD 28.2 cases per 1000 (95% CI 20.8-37.3). By applying the WHF criteria the prevalence of definite and borderline RHD was 8.4 cases per 1000 (95% CI 4.6-14.1) and 10.8 cases per 1000 (95% CI 6.4-17.0) respectively. Definite RHD was more common in females (OR 5.1, 95% CI 1.1-48.3) and in children who attended school in a rural location (OR 2.3, 95% CI 0.6-13.50). Auscultation was poorly sensitive compared to echocardiography (30%).
There is a high burden of undiagnosed RHD in Fiji. Auscultation is poorly sensitive when compared to echocardiography in the detection of asymptomatic RHD. The results of this study highlight the importance of the use of highly sensitive and specific diagnostic criteria for echocardiography diagnosis of RHD.
Echocardiographic screening for rheumatic heart disease (RHD) is becoming more widespread, but screening studies to date have used different echocardiographic definitions. The World Heart Federation (WHF) has recently published new criteria for the echocardiographic diagnosis of RHD. We aimed to establish the prevalence of RHD in high-risk Indigenous Australian children using these criteria, and to compare the findings with a group of Australian children at low risk for RHD.
Portable echocardiography was performed on high-risk Indigenous children aged 5 to15 years living in remote communities of northern Australia. A comparison group of low-risk, non-Indigenous children living in urban centers was also screened. Echocardiograms were reported in a standardized, blinded fashion. Of 3946 high-risk children, 34 met WHF criteria for Definite RHD (prevalence 8.6 per 1000; 95% CI 6.0-12.0) and 66 for Borderline RHD (prevalence 16.7 per 1000; 95% CI 13.0-21.2). Of 1053 low-risk children, none met criteria for Definite RHD, and 5 met criteria for Borderline RHD. High-risk children were more likely to have Definite or Borderline RHD than low-risk children (adjusted odds ratio 5.7, 95% CI 2.3-14.1, p<0.001).
The prevalence of Definite RHD in high-risk Indigenous Australian children approximates what we expected in our population, and no Definite RHD was identified in low risk children. This study suggests that Definite RHD, as defined by the WHF criteria, is likely to represent true disease. Borderline RHD was identified in both low- and high-risk children, highlighting the need for longitudinal studies to evaluate the clinical significance of this finding.
Rheumatic heart disease (RHD) remains endemic in most of the developing world. Echocardiography has proved highly sensitive for early detection of RHD, but it remains too costly for most low-income settings. Handheld ultrasound machines used to perform handheld echocardiography (HAND) are both less expensive and more portable, possibly making them ideal screening tools. HAND has never been tested for the early diagnosis of RHD. The aim of this study was to evaluate the performance of focused HAND compared with focused standard portable echocardiography for the diagnosis of subclinical RHD.
HAND and standard portable echocardiography were performed on 125 Ugandan children, 41 with borderline or definite RHD, and 84 healthy controls. Images were blindly reviewed according to the 2012 World Heart Federation guidelines.
HAND was highly sensitive (90.2%) and specific (92.9%) for distinguishing between normal patients and those with RHD, but it performed best with definite RHD. HAND overestimated mitral valve morphologic valve abnormalities, being only 66.7% specific for anterior leaflet thickness > 3 mm and 79.0% specific for restricted leaflet motion. False-negative results (n = 4) were due primarily to underestimation of mitral regurgitation length.
In this population, HAND was highly sensitive and specific for early detection of RHD. HAND functions best as a screening tool with confirmation of positive screening results by fully functional echocardiography machines. Technical advances may enable one-step RHD screening using HAND. The performance of HAND should be studied across diverse populations and in field tests before recommending it for widespread screening.
It is fairly easy to detect advanced valve lesions of established rheumatic heart disease by echocardiography in the clinically identified cases of rheumatic heart disease. However, to diagnose a subclinical case of rheumatic heart disease, no uniform set of echocardiographic criteria exist. Moderate thickening of valve leaflets is considered an indicator of established rheumatic heart disease. World Health Organization criteria for diagnosing probable rheumatic heart disease are more sensitive and are based on the detection of significant regurgitation of mitral and/or aortic valves by color Doppler. We attempted diagnosing RHD in school children in Bikaner city by cardiac ultrasound.
The stratified cluster sampling technique was employed to identify 31 random clusters in the coeducational schools of Bikaner city. We selected 1059 school children aged 6-15 years from these schools. An experienced operator did careful cardiac auscultation and echocardiographic study. A second expert confirmed the echocardiographic findings.
The prevalence of lesions suggestive of rheumatic heart disease by echocardiography was 51 per 1,000 (denominator = 1059; 95% CI: 38-64 per 1,000). We were able to clinically diagnose RHD in one child. None of these children or their parents having echocardiographic evidence of RHD could provide a positive history of acute rheumatic fever.
By echocardiographic screening, we found a high prevalence of rheumatic heart disease in the surveyed population. Clinical auscultation had much lower diagnostic efficacy.
Early case detection is vital in rheumatic heart disease (RHD) in children to minimize the risk of advanced valvular heart disease by preventive measures. The currently utilized World Health Organization (WHO) criteria for echocardiographic diagnosis of subclinical RHD emphasize the presence of pathological valve regurgitation but do not include valves with morphological features of RHD without pathological regurgitation. We hypothesized that adding morphological features to diagnostic criteria might have significant consequences in terms of case detection rates.
We screened 2170 randomly selected school children aged 6 to 17 years in Maputo, Mozambique, clinically and by a portable ultrasound system. Two different echocardiographic sets of criteria for RHD were assessed: "WHO" (exclusively Doppler-based) and "combined" (Doppler and morphology-based) criteria. Independent investigators reviewed all suspected RHD cases using a higher-resolution, nonportable ultrasound system. On-site echocardiography identified 18 and 124 children with suspected RHD according to WHO and combined criteria, respectively. After consensus review, 17 were finally considered to have definite RHD according to WHO criteria, and 66 had definite RHD according to combined criteria, giving prevalence rates of 7.8 (95% confidence interval, 4.6 to 12.5) and 30.4 (95% confidence interval, 23.6 to 38.5) per 1000 children, respectively (P<0.0001, exact McNemar test).
Important consideration should be given to echocardiographic criteria for detecting subclinical RHD because the number of cases detected may differ importantly according to the diagnostic criteria utilized. Currently recommended WHO criteria risk missing up to three quarters of cases of subclinically affected and therefore potentially treatable children with RHD.
This paper presents a general statistical methodology for the analysis of multivariate categorical data arising from observer reliability studies. The procedure essentially involves the construction of functions of the observed proportions which are directed at the extent to which the observers agree among themselves and the construction of test statistics for hypotheses involving these functions. Tests for interobserver bias are presented in terms of first-order marginal homogeneity and measures of interobserver agreement are developed as generalized kappa-type statistics. These procedures are illustrated with a clinical diagnosis example from the epidemiological literature.
The prognosis for 115 rheumatic fever patients subsequently receiving regular intramuscular penicillin prophylaxis for at least 5 years was studied. Average follow-up was 9.3 years, and 57 patients were followed for 10 years or longer. Of the 79 patients with acute mitral regurgitation, 70% lost their murmur from 4 days to 8.5 years after it was first heard. This is in contrast to those with acute aortic regurgitation which persisted in 73%. No patient developed mitral or aortic stenosis: this suggests that regular prophylaxis may prevent the development of stenosis. Congestive failure, cardiomegaly, and arrhythmia correlated closely with persistence of murmurs, but increased P-R interval, acute QRS or T-voltage variation or both, and ST-T abnormalities did not.
The clinical disappearance of the murmur of rheumatic mitral regurgitation after period of time has been documented by many researchers. However no studies have related the disappearance of the murmur with the functional or anatomical state of the mitral valve. This study was done to elucidate the mitral valve status using doppler and color coded echocardiography among those children who have lost their apical pansystolic murmur on auscultation following a documented attack of rheumatic fever. The study sample consisted of 51 patients including 31 patients in whom the murmur has disappeared (group I), and 20 patients with persistent isolated mitral regurgitation (group II). Patients of group I had significantly lower grades of murmur intensity, lower incidence of cardiomegaly, and had no heart failure in the initial attack. They were more compliant with prophylaxis and had less recurrences than patients of group II. The murmur disappeared in patients of group I from 1/2 to 14 years after the initial attack. Echocardiography revealed that such patients had a normal mitral valve apparatus, and a normal heart size and function. Only 5 patients of this group had a significant regurgitant jet demonstrated by colour doppler. We concluded that recovery of the mitral valve and return of cardiac functions to normal is possible in patients who had mitral regurgitation following rheumatic fever. Some of them may still have an inaudible mild regurgitation. Patients who have lost their murmur may be allowed to exercise freely, yet penicillin prophylaxis should not be discontinued.
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Rheumatic heart disease (RHD) is an important problem in developing countries; however, many cases are detected only when the disease has progressed to cardiac failure. Screening can detect cases earlier, but there are no screening guidelines.
We performed a cross-sectional screening study in Tonga among 5,053 primary school children, in whom auscultation followed by echocardiography of those with heart murmurs were used to identify RHD. We also analyzed whether a three-stage screening protocol of auscultation performed by a medical student to detect any heart murmur, second-stage auscultation performed by a local pediatrician to differentiate pathological from innocent murmurs and echocardiography of those with pathological murmurs altered outcomes.
The prevalence of definite RHD was 33.2 per 1,000. The prevalence of RHD increased significantly with age, peaking at 42.6 per 1,000 in children aged 10-12 years. Most valve lesions (91 [54%] of 169) were mild. Auscultation to detect pathological murmurs was poorly sensitive (46.4%), and the finding of any murmur on auscultation did not affect the likelihood of detecting pathology on echocardiography. The finding of a pathological murmur did significantly increase the likelihood of detecting pathology on echocardiography, but still missed 54% of those with pathology (mainly RHD) detected on echocardiography.
Screening is a useful method for detecting asymptomatic RHD in regions of high prevalence and we report a high echocardiographically confirmed prevalence. The most appropriate screening strategy remains to be confirmed, however, and implementation will depend on the availability of echocardiography and trained staff.
Focused cardiac ultrasound screening for rheumatic heart disease by briefly trained health workers: a study of diagnostic accuracy
- Enelman
Rheumatic heart disease echocardiographic screening: approaching practical and affordable solutions
- Nascimenti