Secondary Antibiotic Prophylaxis for Latent Rheumatic Heart Disease.

BACKGROUND: Rheumatic heart disease affects more than 40.5 million people worldwide and results in 306,000 deaths annually. Echocardiographic screening detects rheumatic heart disease at an early, latent stage. Whether secondary antibiotic prophylaxis is effective in preventing progression of latent rheumatic heart disease is unknown. METHODS: We conducted a randomized, controlled trial of secondary antibiotic prophylaxis in Ugandan children and adolescents 5 to 17 years of age with latent rheumatic heart disease. Participants were randomly assigned to receive either injections of penicillin G benzathine (also known as benzathine benzylpenicillin) every 4 weeks for 2 years or no prophylaxis. All the participants underwent echocardiography at baseline and at 2 years after randomization. Changes from baseline were adjudicated by a panel whose members were unaware of the trial-group assignments. The primary outcome was echocardiographic progression of latent rheumatic heart disease at 2 years. RESULTS: Among 102,200 children and adolescents who had screening echocardiograms, 3327 were initially assessed as having latent rheumatic heart disease, and 926 of the 3327 subsequently received a definitive diagnosis on the basis of confirmatory echocardiography and were determined to be eligible for the trial. Consent or assent for participation was provided for 916 persons, and all underwent randomization; 818 participants were included in the modified intention-to-treat analysis, and 799 (97.7%) completed the trial. A total of 3 participants (0.8%) in the prophylaxis group had echocardiographic progression at 2 years, as compared with 33 (8.2%) in the control group (risk difference, -7.5 percentage points; 95% confidence interval, -10.2 to -4.7; P<0.001). Two participants in the prophylaxis group had serious adverse events that were attributable to receipt of prophylaxis, including one episode of a mild anaphylactic reaction (representing <0.1% of all administered doses of prophylaxis). CONCLUSIONS: Among children and adolescents 5 to 17 years of age with latent rheumatic heart disease, secondary antibiotic prophylaxis reduced the risk of disease progression at 2 years. Further research is needed before the implementation of population-level screening can be recommended. (Funded by the Thrasher Research Fund and others; GOAL ClinicalTrials.gov number, NCT03346525.).

Web-CONEXS: an inroad to theoretical X-ray absorption spectroscopy.

Accurate analysis of the rich information contained within X-ray spectra usually calls for detailed electronic structure theory simulations. However, density functional theory (DFT), time-dependent DFT and many-body perturbation theory calculations increasingly require the use of advanced codes running on high-performance computing (HPC) facilities. Consequently, many researchers who would like to augment their experimental work with such simulations are hampered by the compounding of nontrivial knowledge requirements, specialist training and significant time investment. To this end, we present Web-CONEXS, an intuitive graphical web application for democratizing electronic structure theory simulations. Web-CONEXS generates and submits simulation workflows for theoretical X-ray absorption and X-ray emission spectroscopy to a remote computing cluster. In the present form, Web-CONEXS interfaces with three software packages: ORCA, FDMNES and Quantum ESPRESSO, and an extensive materials database courtesy of the Materials Project API. These software packages have been selected to model diverse materials and properties. Web-CONEXS has been conceived with the novice user in mind; job submission is limited to a subset of simulation parameters. This ensures that much of the simulation complexity is lifted and preliminary theoretical results are generated faster. Web-CONEXS can be leveraged to support beam time proposals and serve as a platform for preliminary analysis of experimental data.

Medical Treatment for Rheumatic Heart Disease: A Narrative Review.

BACKGROUND: Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are rare in high-income countries; however, in Aotearoa New Zealand ARF and RHD disproportionately affect Indigenous Maori and Pacific Peoples. This narrative review explores the evidence regarding non-surgical management of patients with clinically significant valve disease or heart failure due to RHD. METHODS: Medline, EMBASE and Scopus databases were searched, and additional publications were identified through cross-referencing. Included were 28 publications from 1980 onwards. RESULTS: Of the available interventions, improved anticoagulation management and a national RHD register could improve RHD outcomes in New Zealand. Where community pharmacy anticoagulant management services (CPAMS) are available good anticoagulation control can be achieved with a time in the therapeutic range (TTR) of more than 70%, which is above the internationally recommended level of 60%. The use of pharmacists in anticoagulation control is cost-effective, acceptable to patients, pharmacists, and primary care practitioners. There is a lack of local data available to fully assess other interventions; including optimal therapy for heart failure, equitable access to specialist RHD care, prevention, and management of endocarditis. CONCLUSION: As RHD continues to disproportionately affect Indigenous and minority groups, pro-equity tertiary prevention interventions should be fully evaluated to ensure they are reducing disease burden and improving outcomes in patients with RHD.

Rising Ethnic Inequalities in Acute Rheumatic Fever and Rheumatic Heart Disease, New Zealand, 2000-2018.

We describe trends in acute rheumatic fever (ARF), rheumatic heart disease (RHD), and RHD deaths among population groups in New Zealand. We analyzed initial primary ARF and RHD hospitalizations during 2000-2018 and RHD mortality rates during 2000-2016. We found elevated rates of initial ARF hospitalizations for persons of Maori (adjusted rate ratio [aRR] 11.8, 95% CI 10.0-14.0) and Pacific Islander (aRR 23.6, 95% CI 19.9-27.9) ethnicity compared with persons of European/other ethnicity. We also noted higher rates of initial RHD hospitalization for Maori (aRR 3.2, 95% CI 2.9-3.5) and Pacific Islander (aRR 4.6, 95% CI 4.2-5.1) groups and RHD deaths among these groups (Maori aRR 12.3, 95% CI 10.3-14.6, and Pacific Islanders aRR 11.2, 95% CI 9.1-13.8). Rates also were higher in socioeconomically disadvantaged neighborhoods. To curb high rates of ARF and RHD, New Zealand must address increasing social and ethnic inequalities.

Ethnically Disparate Disease Progression and Outcomes among Acute Rheumatic Fever Patients in New Zealand, 1989-2015.

We investigated outcomes for patients born after 1983 and hospitalized with initial acute rheumatic fever (ARF) in New Zealand during 1989-2012. We linked ARF progression outcome data (recurrent hospitalization for ARF, hospitalization for rheumatic heart disease [RHD], and death from circulatory causes) for 1989-2015. Retrospective analysis identified initial RHD patients <40 years of age who were hospitalized during 2010-2015 and previously hospitalized for ARF. Most (86.4%) of the 2,182 initial ARF patients did not experience disease progression by the end of 2015. Progression probability after 26.8 years of theoretical follow-up was 24.0%; probability of death, 1.0%. Progression was more rapid and ≈2 times more likely for indigenous Maori or Pacific Islander patients. Of 435 initial RHD patients, 82.2% had not been previously hospitalized for ARF. This young cohort demonstrated low mortality rates but considerable illness, especially among underserved populations. A national patient register could help monitor, prevent, and reduce ARF progression.

Early diagnosis of acute rheumatic fever and rheumatic heart disease as part of a secondary prevention strategy: Narrative review.

Acute rheumatic fever (ARF) and its sequela rheumatic heart disease (RHD) remain significant causes of morbidity and mortality. In New Zealand, ARF almost exclusively affects Indigenous Maori and Pacific children. This narrative review aims to present secondary interventions to improve early and accurate diagnosis of ARF and RHD, in order to minimise disease progression in New Zealand. Medline, EMBASE and Scopus databases were searched as well as other electronic publications. Included were 56 publications from 1980 onwards. Diagnosing ARF and RHD as early as possible is central to reducing disease progression. Recent identification of specific ARF biomarkers offer the opportunity to aid initial diagnosis and portable echocardiography has the potential to detect undiagnosed RHD in high-risk areas. However, further research into the benefits and risks to children with subclinical RHD is necessary, as well as an economic evaluation.

Systems immunology reveals a linked IgG3-C4 response in patients with acute rheumatic fever.

Acute rheumatic fever (ARF) and chronic rheumatic heart disease (RHD) are autoimmune sequelae of a Group A streptococcal infection with significant global mortality and poorly understood pathogenesis. Immunoglobulin and complement deposition were observed in ARF/RHD valve tissue over 50 years ago, yet contemporary investigations have been lacking. This study applied systems immunology to investigate the relationships between the complement system and immunoglobulin in ARF. Patients were stratified by C-reactive protein (CRP) concentration into high (≥10 µg mL-1 ) and low (<10 µg mL-1 ) groups to distinguish those with clinically significant inflammatory processes from those with abating inflammation. The circulating concentrations of 17 complement factors and six immunoglobulin isotypes and subclasses were measured in ARF patients and highly matched healthy controls using multiplex bead-based immunoassays. An integrative statistical approach combining feature selection and principal component analysis revealed a linked IgG3-C4 response in ARF patients with high CRP that was absent in controls. Strikingly, both IgG3 and C4 were elevated above clinical reference ranges, suggesting these features are a marker of ARF-associated inflammation. Humoral immunity in response to M protein, an antigen implicated in ARF pathogenesis, was completely polarized to IgG3 in the patient group. Furthermore, the anti-M-protein IgG3 response was correlated with circulating IgG3 concentration, highlighting a potential role for this potent immunoglobulin subclass in disease. In conclusion, a linked IgG3-C4 response appears important in the initial, inflammatory stage of ARF and may have immediate utility as a clinical biomarker given the lack of specific diagnostic tests currently available.

Beyond first-degree heart block in the diagnosis of acute rheumatic fever.

OBJECTIVES: First-degree heart block is a minor manifestation of acute rheumatic fever. Second and third degree heart block and junctional rhythms occur less commonly. We report patients presenting with these latter three electrocardiographic abnormalities and investigate their diagnostic utility. DESIGN: Patients admitted to our centre meeting the 2014 New Zealand Rheumatic Fever Guideline Diagnostic Criteria for rheumatic fever over a 5-year period from January 2010 to December 2014 were identified. Clinical, haematologic, electrocardiographic, and echocardiographic records were reviewed. Electrocardiograms (ECG) were considered abnormal if there was second- or third-degree atrioventricular block or junctional rhythms. Comparative data from patients with advanced conduction abnormalities without a diagnosis of rheumatic fever during the same time period were reviewed. RESULTS: A total of 201 patients met inclusion criteria for rheumatic fever. Of these, 17 (8.5%) had transient abnormalities of atrioventricular conduction, 5 (2.5%) with second or third-degree atrioventricular block, and 12 (6%) junctional rhythms. The remaining 173 (86%) patients had evidence of rheumatic valvulitis at presentation. Only one patient without rheumatic fever was found to have advanced conduction abnormalities over the study period, from a total of 3702 ECG. CONCLUSIONS: This large contemporary cohort of acute rheumatic fever shows that 8.5% of cases had either advanced atrioventricular block or junctional rhythms both highly suggestive of the diagnosis in our population.

Infective endocarditis following Melody valve implantation: comparison with a surgical cohort.

BACKGROUND: Infective endocarditis has been reported post Melody percutaneous pulmonary valve implant; the incidence and risk factors, however, remain poorly defined. We identified four cases of endocarditis from our first 25 Melody implants. Our aim was to examine these cases in the context of postulated risk factors and directly compare endocarditis rates with local surgical valves. METHODS: We conducted a retrospective review of patients post Melody percutaneous pulmonary valve implant in New Zealand (October, 2009-May, 2015) and also reviewed the incidence of endocarditis in New Zealand among patients who have undergone surgical pulmonary valve implants. RESULTS: In total, 25 patients underwent Melody implantation at a median age of 18 years. At a median follow-up of 2.9 years, most were well with low valve gradient (median 27 mmHg) and only mild regurgitation. Two patients presented with life-threatening endocarditis and obstructive vegetations at 14 and 26 months post implant, respectively. Two additional patients presented with subacute endocarditis at 5.5 years post implant. From 2009 to May, 2015, 178 surgical pulmonic bioprostheses, largely Hancock valves and homografts, were used at our institution. At a median follow-up of 2.9 years, four patients (2%) had developed endocarditis in this group compared with 4/25 (16%) in the Melody group (p=0.0089). Three surgical valves have been replaced. CONCLUSIONS: The Melody valve offers a good alternative to surgical conduit replacement in selected patients. Many patients have excellent outcomes in the medium term. Endocarditis, however, can occur and if associated with obstruction can be life threatening. The risk for endocarditis in the Melody group was higher in comparison with that in a contemporaneous surgical pulmonary implant cohort.

Transcatheter closure of secundum atrial septal defects: has fear of device erosion altered outcomes?

BACKGROUND: Transcatheter device closure has become the established standard of care for suitable atrial septal defects. Device erosion has been a recent focus and has prompted changes in the Instructions for Users documentation released by device companies. We reviewed our entire local experience with atrial septal defect device closure, focussing on the evolution of this procedure in our centre and particularly on complications. METHODS: We carried out a retrospective review of 581 consecutive patients undergoing attempted transcatheter device closure of an atrial septal defect in Auckland from December 1997 to June 2014. We reviewed all complications recorded and compared our outcomes with the current literature. We sought to understand the impact of the evolution in recommendations and clinical practice on patient outcomes in our programme. RESULTS: There were a total of 24 complications (4.1%), including 10 device embolisations (1.7%), nine arrhythmias (1.5%), two significant vascular access-related complications (0.3%), one device erosion (0.2%), one malposed device (0.2%), and one probable wire perforation of the left atrial appendage (0.2%). There was one mortality related to device embolisation. All device embolisations occurred following the change in Instructions for Users after publication of the first device erosion report in 2004. This increase in embolisation rate was statistically significant (p-value 0.015). CONCLUSIONS: In our series, the incidence of device embolisation was higher than that anticipated, with a significant increase following changes to the Instructions for Users. This highlights the need for ongoing data collection on complication incidence and for ongoing review of the impact of changes in clinical practice on complication rates.

Association study involving polymorphisms in IL-6, IL-1RA, and CTLA4 genes and rheumatic heart disease in New Zealand population of Maori and Pacific ancestry.

INTRODUCTION: Rheumatic fever (RF) incidence among New Zealand (NZ) individuals of Polynesian (Maori and Pacific) ancestry remains among the highest in the world. Polymorphisms in the IL-6, IL1RN, and CTLA4 genes have been associated with RF, and their products are modulated by new medications. Confirmation of these previous associations could help guide clinical approaches. We aimed to test IL-6, IL-1RA (IL1RN), and CTLA4 functional SNPs in 204 rheumatic heart disease (RHD) patients and 116 controls of Maori and Pacific ancestry. MATERIAL AND METHOD: Self-reported ancestry of the eight great-grandparents defined ancestry of participants. Severity of carditis was classified according to the 2012 World Heart Federation guideline for the echocardiographic diagnosis of RHD. The IL-6 promoter rs1800797, IL1RN rs447713 and CTLA4 rs3087243 SNPs were genotyped by Taqman. Correlations were assessed by logistic regression analysis adjusting for gender and ancestry. RESULTS: The IL-6 rs1800797 variant was significantly associated with RHD with carriers of the GG genotype 6.09 (CI 1.23; 30.23) times more likely to develop RHD than the carriers of the AA genotype (P=0.027). No significant associations with RHD were found for the IL1RN rs447713 and CTLA4 rs3087243 SNPs. Patients carrying the G allele (GG plus AG genotype) for the IL1RN rs447713 SNP had 2.36 times (CI 1.00; 5.56) more severe carditis than those without this allele (the AA genotype) (P=0.049). CONCLUSION: The IL-6 promoter rs1800797 (-597G/A) SNP may influence susceptibility to RHD of people of Maori and Pacific ancestry living in NZ. The IL1RN rs447713 SNP may influence the severity of carditis in this population.

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

BACKGROUND: Echocardiographic 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. METHODS: Seven 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. RESULTS: The 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). CONCLUSIONS: After 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.

Family acceptability of school-based echocardiographic screening for rheumatic heart disease in a high-risk population in New Zealand.

AIM: Echocardiographic screening for rheumatic heart disease has been piloted in high-risk areas in New Zealand and internationally, and fulfils most of the criteria for a targeted screening programme. The question of acceptability of rheumatic heart disease screening has not been assessed, and the aim of our study was to assess parental acceptability of a school-based echocardiographic screening programme in a high-risk population in New Zealand. METHODS: A post-screening questionnaire was developed to survey parents of children who underwent echocardiographic screening. The families of 34 children with abnormal scan results and a sample of 80 children with normal scan results were surveyed by phone within 4 months of screening. RESULTS: Positive results were seen in all survey questions in both normal and abnormal scan groups. All families were supportive of an ongoing screening programme. Of children with abnormal results, 62% of their parents reported that they would treat their child differently; however, all responses were positive health-promoting outcomes. CONCLUSION: The study showed strong positive support for school-based echocardiographic screening by a community with high acute rheumatic fever incidence. The study did not detect any short-term negative effects in those with abnormal results. The survey result shows family and community support for the establishment of echocardiographic screening programmes in high acute rheumatic fever areas provided there is adequate infrastructural support.

Mitral valve geometry in paediatric rheumatic mitral regurgitation.

Rheumatic mitral valve disease is associated with valvulitis and valvular regurgitation; however, the effect of the rheumatic process on the mitral valve geometry and function is not well understood. To assess mitral valve annulus remodelling in rheumatic mitral valve disease, 16 subjects aged 6-15 years with rheumatic mitral valve regurgitation [MR] [6 mild or moderate (Group 1), 10 severe (Group 2)] and 7 age- and body size-matched normal controls with adequate trans-thoracic echocardiograms were recruited. None of the patients had undergone surgical intervention and none had more than mild aortic regurgitation. None of the patients had mitral stenosis. 3D mitral valve geometry was assessed using a Tomtec system. The non-planar angle was increased in all subjects during early (control 147° ± 10, Group 1 168° ± 9, Group 2 166° ± 10; p < 0.05) and late systole (control 149° ± 12, Group 1 162° ± 10, Group 2 164° ± 6; p < 0.05), indicating loss of saddle shape. 2D annular area increased in Group 2 (control 397 ± 48 mm(2)/m(2) vs Group 2 739 ± 207, p < 0.05) with no significant change in annular perimeter indicating leaflet effacement. Mitral valve area correlated with left ventricular size (p < 0.001, r (2) = 0.74). There was no significant change in valve area and perimeter between early and late systole. Remodelling of the mitral valve apparatus in childhood rheumatic heart disease results in abnormal annular geometry. The mitral valve loses its saddle shape regardless of the severity of MR. This may be a consequence of inflammation on the fibrous trigones and surrounding annulus. In contrast, annular area enlargement occurs as a result of leaflet effacement and relates to MR severity and LV size.

Two cases of warfarin-induced tracheobronchial calcification after Fontan surgery.

This study identified tracheobronchial cartilage calcification in children with congenital heart disease. Calcification of the tracheobronchial airways has been found previously in adults receiving warfarin and in children receiving warfarin after mitral valve replacement. A 9-year-old girl who had received a Fontan repair 6 years previously underwent a cardiac computed tomography (CT) scan to evaluate pulmonary artery size. The result was an incidental finding of extensive tracheobronchial cartilage calcification. A retrospective review of all pediatric Fontan patients who had undergone cardiac CT was conducted to search for calcification of the tracheobronchial cartilage. The study investigated ten pediatric Fontan patients who had undergone cardiac CT scanning. Two patients with extensive calcification of the tracheobronchial airways were identified. The index case had hypoplastic left heart syndrome, and the patient had undergone a staged repair with the Fontan at the age of 3 years. A 16-year-old boy with tricuspid atresia had undergone staged repair and Fontan at the age of 3.5 years. These two patients had received continuous warfarin therapy for 6 and 13 years, respectively. Other common causes of airway calcification were excluded from the study. This report describes warfarin-induced tracheobronchial calcification in patients after the Fontan procedure. This finding has possible implications for airway growth and vascular calcification.

The Echocardiographic Diagnosis of Rheumatic Heart Disease: A Review of the Performance of the World Heart Federation Criteria 2012-2023.

Background: The World Heart Federation (WHF) published the first evidence-based guidelines on the echocardiographic diagnosis of rheumatic heart disease (RHD) in 2012. These guidelines have since been applied internationally in research and clinical practice. Substantial research has assessed the utility of the 2012 WHF criteria, including its applicability in low-resource settings. This article summarises the evidence regarding the performance of the guidelines. Methods: A scoping review assessing the performance of the guidelines was performed. Cochrane, Embase, Medline, PubMed Lilacs, Sielo, and Portal BVS databases were searched for studies on the performance of the guidelines between January 2012-March 2023, and 4047 manuscripts met the search criteria, of which 34 were included. This included papers assessing the specificity, inter-rater reliability, application using hand-carried ultrasound, and modification of the criteria for simplicity. The review followed the PRISMA Extension for Scoping Reviews guideline. Results: The WHF 2012 criteria were 100% specific for definite RHD when applied in low-prevalence populations. The criteria demonstrated substantial and moderate inter-rater reliability for detecting definite and borderline RHD, respectively. The inter-rater reliability for morphological features was lower than for valvular regurgitation. When applied to hand-carried ultrasound performed by an expert, modified versions of the criteria demonstrated a sensitivity and specificity range of 79-90% and 87-93% respectively for detecting any RHD, performing best for definite RHD. The sensitivity and the specificity were reduced when performed in task-sharing but remains moderately accurate. Conclusion: The WHF 2012 criteria provide clear guidance for the echocardiographic diagnosis of RHD that is reproducible and applicable to a range of echocardiographic technology. Furthermore, the criteria are highly specific and particularly accurate for detecting definite RHD. There are limitations in applying all aspects of the criteria in specific settings, including task-sharing. This summary of evidence can inform the updated version of the WHF guidelines to ensure improved applicability in all RHD endemic regions.

An acute rheumatic fever immune signature comprising inflammatory markers, IgG3, and Streptococcus pyogenes-specific antibodies.

Understanding the immune profile of acute rheumatic fever (ARF), a serious post-infectious sequelae of Streptococcal pyogenes (group A Streptococcus [GAS]), could inform disease pathogenesis and management. Circulating cytokines, immunoglobulins, and complement were analyzed in participants with first-episode ARF, swab-positive GAS pharyngitis and matched healthy controls. A striking elevation of total IgG3 was observed in ARF (90% > clinical reference range for normal). ARF was also associated with an inflammatory triad with significant correlations between interleukin-6, C-reactive protein, and complement C4 absent in controls. Quantification of GAS-specific antibody responses revealed that subclass polarization was remarkably consistent across the disease spectrum; conserved protein antigens polarized to IgG1, while M-protein responses polarized to IgG3 in all groups. However, the magnitude of responses was significantly higher in ARF. Taken together, these findings emphasize the association of exaggerated GAS antibody responses, IgG3, and inflammatory cytokines in ARF and suggest IgG3 testing could beneficially augment clinical diagnosis.

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.

Rheumatic fever in New Zealand.

Acute rheumatic fever and its sequel rheumatic heart disease remain major unsolved problems in New Zealand, causing significant morbidity and premature death. The disease burden affects predominantly indigenous Maori and Pacific Island children and young adults. In the past decade these ethnic disparities are even widening. Secondary prophylaxis using 28-day intramuscular penicillin has been the mainstay of disease control. In the greater Auckland region, audit shows community nurse-led penicillin delivery rates of 95% and recurrence rates of less than 5%. The true penicillin failure rate of 0.07 per 100 patient years supports 4 weekly penicillin rather than more frequent dose regimens. Landmark primary prevention research has been undertaken supporting sore throat primary prevention programmes in regions with very high rheumatic fever rates. Echocardiographic screening found 2.4% previously undiagnosed rheumatic heart disease in socially disadvantaged children. Combined with secondary prevention, echocardiography screening has the potential to reduce the prevalence of severe rheumatic heart disease.