2023 World Heart Federation guidelines for the echocardiographic diagnosis of rheumatic heart disease.

Rheumatic heart disease (RHD) is an important and preventable cause of morbidity and mortality among children and young adults in low-income and middle-income countries, as well as among certain at-risk populations living in high-income countries. The 2012 World Heart Federation echocardiographic criteria provided a standardized approach for the identification of RHD and facilitated an improvement in early case detection. The 2012 criteria were used to define disease burden in numerous epidemiological studies, but researchers and clinicians have since highlighted limitations that have prompted a revision. In this updated version of the guidelines, we incorporate evidence from a scoping review, an expert panel and end-user feedback and present an approach for active case finding for RHD, including the use of screening and confirmatory criteria. These guidelines also introduce a new stage-based classification for RHD to identify the risk of disease progression. They describe the latest evidence and recommendations on population-based echocardiographic active case finding and risk stratification. Secondary antibiotic prophylaxis, echocardiography equipment and task sharing for RHD active case finding are also discussed. These World Heart Federation 2023 guidelines provide a concise and updated resource for clinical and research applications in RHD-endemic regions.

The Use of Artificial Intelligence Guidance for Rheumatic Heart Disease Screening by Novices.

INTRODUCTION: A novel technology utilizing artificial intelligence (AI) to provide real-time image-acquisition guidance, enabling novices to obtain diagnostic echocardiographic images, holds promise to expand the reach of echo screening for rheumatic heart disease (RHD). We evaluated the ability of nonexperts to obtain diagnostic-quality images in patients with RHD using AI guidance with color Doppler. METHODS: Novice providers without prior ultrasound experience underwent a 1-day training curriculum to complete a 7-view screening protocol using AI guidance in Kampala, Uganda. All trainees then scanned 8 to 10 volunteer patients using AI guidance, half RHD and half normal. The same patients were scanned by 2 expert sonographers without the use of AI guidance. Images were evaluated by expert blinded cardiologists to assess (1) diagnostic quality to determine presence/absence of RHD and (2) valvular function and (3) to assign an American College of Emergency Physicians score of 1 to 5 for each view. RESULTS: Thirty-six novice participants scanned a total of 50 patients, resulting in a total of 462 echocardiogram studies, 362 obtained by nonexperts using AI guidance and 100 obtained by expert sonographers without AI guidance. Novice images enabled diagnostic interpretation in >90% of studies for presence/absence of RHD, abnormal MV morphology, and mitral regurgitation (vs 99% by experts, P ≤ .001). Images were less diagnostic for aortic valve disease (79% for aortic regurgitation, 50% for aortic stenosis, vs 99% and 91% by experts, P < .001). The American College of Emergency Physicians scores of nonexpert images were highest in the parasternal long-axis images (mean, 3.45; 81% ≥ 3) compared with lower scores for apical 4-chamber (mean, 3.20; 74% ≥ 3) and apical 5-chamber images (mean, 2.43; 38% ≥ 3). CONCLUSIONS: Artificial intelligence guidance with color Doppler is feasible to enable RHD screening by nonexperts, performing significantly better for assessment of the mitral than aortic valve. Further refinement is needed to optimize acquisition of color Doppler apical views.

Circumflex aorta with double aortic arch in an infant.

Circumflex aorta and double aortic arch are two forms of rare vascular rings. We present a case of an infant who was diagnosed with circumflex aorta and double aortic arch, and describe the surgical management of this rare anomaly.

Task shifting to clinical officer-led echocardiography screening for detecting rheumatic heart disease in Malawi, Africa.

BACKGROUND: 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. Hypothesis With short-course training, clinical officers can accurately screen for rheumatic heart disease using focussed echocardiography. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

School and Community Screening Shows Malawi, Africa, to Have a High Prevalence of Latent Rheumatic Heart Disease.

Rheumatic heart disease (RHD) is the largest cardiac cause of morbidity and mortality in the world's youth. Early detection of RHD through echocardiographic screening in asymptomatic children may identify an early stage of disease, when secondary prophylaxis has the greatest chance of stopping disease progression. Latent RHD signifies echocardiographic evidence of RHD with no known history of acute rheumatic fever and no clinical symptoms. OBJECTIVE: Determine the prevalence of latent RHD among children ages 5-16 in Lilongwe, Malawi. DESIGN: This is a cross-sectional study in which children ages 5 through 16 were screened for RHD using echocardiography. SETTING: Screening was conducted in 3 schools and surrounding communities in the Lilongwe district of Malawi between February and April 2014. OUTCOME MEASURES: Children were diagnosed as having no, borderline, or definite RHD as defined by World Heart Federation criteria. The primary reader completed offline reads of all studies. A second reader reviewed all of the studies diagnosed as RHD, plus a selection of normal studies. A third reader served as tiebreaker for discordant diagnoses. The distribution of results was compared between gender, location, and age categories using Fisher's exact test. RESULTS: The prevalence of latent RHD was 3.4% (95% CI = 2.45, 4.31), with 0.7% definite RHD and 2.7% borderline RHD. There was no significant differences in prevalence between gender (P = .44), site (P = .6), urban vs. peri-urban (P = .75), or age (P = .79). Of those with definite RHD, all were diagnosed because of pathologic mitral regurgitation (MR) and 2 morphologic features of the mitral valve. Of those with borderline RHD, most met the criteria by having pathological MR (92.3%). CONCLUSION: Malawi has a high rate of latent RHD, which is consistent with other results from sub-Saharan Africa. This study strongly supports the need for a RHD prevention and control program in Malawi.