Bo Remenyi

Screening for rheumatic heart disease: current approaches and controversies.

Rheumatic heart disease (RHD) is a leading cause of cardiac disease among children in developing nations, and in indigenous populations of some industrialized countries. In endemic areas, RHD has long been a target of screening programmes that, historically, have relied on cardiac auscultation. The evolution of portable echocardiographic equipment has changed the face of screening for RHD over the past 5 years, with greatly improved sensitivity. However, concerns have been raised about the specificity of echocardiography, and the interpretation of minor abnormalities poses new challenges. The natural history of RHD in children with subclinical abnormalities detected by echocardiographic screening remains unknown, and long-term follow-up studies are needed to evaluate the significance of detecting these changes at an early stage. For a disease to be deemed suitable for screening from a public health perspective, it needs to fulfil a number of criteria. RHD meets some, but not all, of these criteria. If screening programmes are to identify additional cases of RHD, parallel improvements in the systems that deliver secondary prophylaxis are essential.

Echocardiographic Screening for Rheumatic Heart Disease in High and Low Risk Australian Children

Background— 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 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. Methods and Results— 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 World Heart Federation criteria for definite RHD (prevalence, 8.6 per 1000 [95% confidence interval, 6.0–12.0]) and 66 for borderline RHD (prevalence, 16.7 per 1000 [95% confidence interval, 13.0–21.2]). Of 1053 low-risk children, none met the criteria for definite RHD, and 5 met the 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% confidence interval, 2.3–14.1]; P<0.001). Conclusions— 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 the low-risk group. This study suggests that definite RHD, as defined by the World Heart Federation criteria, is likely to represent true disease. Borderline RHD was identified in children at both low and high risk, highlighting the need for longitudinal studies to evaluate the clinical significance of this finding.

Echocardiographic screening in a resource poor setting: Borderline rheumatic heart disease could be a normal variant

OBJECTIVE To estimate the echocardiography confirmed prevalence of rheumatic heart disease (RHD) in school children in Fiji. DESIGN Cross-sectional observational study. SETTING Ten primary schools in Fiji. PATIENTS School children aged 5-14 years. INTERVENTIONS 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. MAIN OUTCOME MEASURES 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. RESULTS 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%). CONCLUSION 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.

Improved Long-Term Survival for Rheumatic Mitral Valve Repair Compared to Replacement in the Young

Background: Mitral valve (MV) repair offers potential advantages over replacement in patients with rheumatic heart disease (RHD). We present the first long-term study that compares MV repair with replacement in children with RHD. Methods and Results: Single institute retrospective review of patients with RHD under 20 years of age, who underwent their first isolated MV surgery between 1990 and 2006. Of the 81 patients, 98% were Māori or Pacific Islander. The median age was 12.7 (3-19) years. The MV was repaired in 59%, a mechanical valve replacement (MVR) took place in 35% and bioprosthetic valve replacement in 6% of the patients. Follow-up data were available for 91.4% of the patients with mean follow-up of 7.6 years (range 0-19.4 years), a total of 620 patient years. Actuarial survival at 10 and 14 years for patients with MVR was 79% and 44%, compared to 90% and 90% for patients who underwent repair (P = .06). Actuarial freedom from late reoperation at 10 and 14 years for patients with MVR was 88% and 73%, compared to 76% and 76% for patients with repair (P = .52). Actuarial freedom from thrombotic, embolic, and hemorrhagic events at 10 and 14 years for patients with MVR was 63% and 45%, compared to 100% and 100% for patients with repair P < .01). Conclusion: This study shows that MV repair is superior to replacement for RHD in the young with follow-up to 19 years. Repair offers a survival advantage, greater freedom from valve-related morbidity, and long-term durability that equals that of MVR.

Focused cardiac ultrasound screening for rheumatic heart disease by briefly trained health workers: A study of diagnostic accuracy

BACKGROUND Echocardiographic screening for rheumatic heart disease (RHD) can identify individuals with subclinical disease who could benefit from antibiotic prophylaxis. However, most settings have inadequate resources to implement conventional echocardiography and require a feasible, accurate screening method. We aimed to investigate the accuracy of screening by non-expert operators using focused cardiac ultrasound (FoCUS). METHODS In this prospective study of diagnostic accuracy, we recruited schoolchildren aged 5 to 15 years in Fiji to undergo two blinded tests. The index test was a FoCUS assessment of mitral and aortic regurgitation, performed by nurses after an 8-week training programme. The reference standard was the diagnosis of RHD by a paediatric cardiologist, based on a standard echocardiogram performed by a skilled echocardiographer. The primary outcome was the accuracy of the index test with use of the most sensitive criteria (any regurgitation). FINDINGS We included 2004 children in the study. The index tests were done between September, 2012, and September, 2013, by seven nurses in eight schools in Fiji. The diagnostic accuracy of the screening test (area under receiver operator characteristic curve) was 0·89 (95% CI 0·83-0·94). When the primary cut-off point (any regurgitation) was used for analysis, sensitivity was 84·2% (72·1-92·5) and specificity was 85·6% (83·9-87·1). The sensitivity of individual nurses ranged from 66·7% to 100% and specificity 74·0% to 93·7%. INTERPRETATION Screening by briefly trained nurses using FoCUS was accurate for the diagnosis of RHD. Refinements to training and screening test methods should be studied in a range of settings, and in parallel with investigations of the long-term clinical and cost-effectiveness of screening for RHD. FUNDING Cure Kids, New Zealand; the Fiji Water Foundation provided funding for portable ultrasound equipment; see acknowledgments for further details of funders.

Congenital mitral valve lesions : Correlation between morphology and imaging

Congenital malformations of the mitral valve are often complex and affect multiple segments of the valve apparatus. They may occur in isolation or in association with other congenital heart defects. The majority of mitral valve malformations are not simply classified, and descriptive terms with historical significance (parachute, mitral, or arcade) often lack the specificity that cardiac surgeons demand as part of preoperative echocardiographic morphological assessment. This paper examines the strengths and limitations of commonly used descriptions and classification systems of congenitally malformed mitral valves. It correlates pathological, surgical, and echocardiographic findings. Finally, it makes recommendations for the systematic evaluation of the congenitally malformed mitral valve using segmental echocardiographic analysis to assist precise communication and optimal surgical management.

Teaching focused echocardiography for rheumatic heart disease screening

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.

Screening for rheumatic heart disease: quality and agreement of focused cardiac ultrasound by briefly trained health workers

BackgroundEchocardiographic screening for rheumatic heart disease (RHD) has the potential to detect subclinical cases for secondary prevention, but is constrained by inadequate human resources in most settings. Training non-expert health workers to perform focused cardiac ultrasound (FoCUS) may enable screening at a population-level. We aimed to evaluate the quality and agreement of FoCUS for valvular regurgitation by briefly trained health workers.MethodsSeven nurses participated in an eight week training program in Fiji. Nurses performed FoCUS on 2018 children aged five to 15 years, and assessed any valvular regurgitation. An experienced pediatric cardiologist assessed the quality of ultrasound images and measured any recorded regurgitation. The assessment of the presence of regurgitation and measurement of the longest jet by the nurse and cardiologist was compared, using the Bland-Altman method.ResultsThe quality of FoCUS overall was adequate for diagnosis in 96.6 %. There was substantial agreement between the cardiologist and the nurses overall on the presence of mitral regurgitation (κ = 0.75) and aortic regurgitation (κ = 0.61) seen in two views. Measurements of mitral regurgitation by nurses and the cardiologist were similar (mean bias 0.01 cm; 95 % limits of agreement −0.64 to 0.66 cm).ConclusionsAfter brief training, health workers with no prior experience in echocardiography can obtain adequate quality images and make a reliable assessment on the presence and extent of valvular regurgitation. Further evaluation of the imaging performance and accuracy of screening by non-expert operators is warranted, as a potential population-level screening strategy in high prevalence settings.

Rheumatic heart disease in Indigenous children in northern Australia: differences in prevalence and the challenges of screening

Objectives: To compare regional differences in the prevalence of rheumatic heart disease (RHD) detected by echocardiographic screening in high‐risk Indigenous Australian children, and to describe the logistical and other practical challenges of RHD screening.

The Second Rheumatic Heart Disease Forum Report

The second rheumatic heart disease (RHD) forum was held on February 18, 2013, at the Sixth World Congress of Pediatric Cardiology and Cardiac Surgery in Cape Town, South Africa, to focus attention on key areas in global RHD control, management, and prevention. Building on the foundation of the first RHD forum, over 150 interested participants met to discuss critical issues on the RHD landscape. Unique to this meeting was a mixture of diverse backgrounds and disciplines, all crucially important to the conversation around RHD control and prevention. Some clear priorities have emerged for RHD activities in the next era: the necessity for political intervention and policy change; increasing the health workforce by incorporating teaching, training, and task-shifting; revitalizing the research agenda by merging basic, clinical, and translational research; and obtaining universal access to high-quality penicillin. There was also an urgent request for new resources; for existing resources to be further developed, improved, and shared across platforms; and for resources to be supported in the nonmedical arena. Finally, the necessity of involving the patient community in the ongoing discussion was highlighted. The participants of both the first and second RHD forum represent a new, thriving, and growing community of RHD activists who should usher in a new era of significant improvements in RHD control and prevention.