High burden of rheumatic heart disease confirmed by echocardiography among Pacific adults living in New Zealand.

BACKGROUND: Despite numerous echocardiographic screening studies of children in high incidence acute rheumatic fever (ARF)/rheumatic heart disease (RHD) communities, little is known about the prevalence of RHD in adults in these populations.We sought to determine the prevalence of RHD in an urban area of South Auckland, New Zealand, where previous studies had shown the prevalence of RHD in children to be around 2%. METHODS: A cross-sectional screening study was conducted between 2014 and 2016. Echocardiography clinics were conducted at an urban Pacific-led primary healthcare clinic in New Zealand. Eligible persons aged 16-40 years were recruited according to a stratified randomised approach. Echocardiograms were performed with a standardised image acquisition protocol and reported by cardiologists. RESULTS: There were 465 individuals who underwent echocardiograms. The overall prevalence of RHD (define and borderline) was 56 per 1000 (95% CI 36 to 78 per 1000). Definite RHD was found in 10 individuals (4 of whom were already under cardiology review at a hospital clinic) with a prevalence of 22 per 1000 (95% CI 9 to 36 per 1000). Non-rheumatic cardiac abnormalities were found in 29 individuals. CONCLUSIONS: There is a high burden of both rheumatic and non-rheumatic cardiac abnormalities in this population. Rates described in New Zealand are as high as lower-middle-income countries in Africa. Addressing knowledge gaps regarding the natural history of RHD detected by echocardiography in adults is a priority issue for the international RHD community.

Cohort profile: methodology and cohort characteristics of the Aotearoa New Zealand Rheumatic Heart Disease Registry.

PURPOSE: To create a cohort with high specificity for moderate and severe rheumatic heart disease (RHD) in New Zealand, not reliant on International Classification of Diseases discharge coding. To describe the demography and cardiac profile of this historical and contemporary cohort. DESIGN AND PARTICIPANTS: Retrospective identification of moderate or severe RHD with disease onset by 2019. Case identification from the following data sources: cardiac surgical databases, RHD case series, percutaneous balloon valvuloplasty databases, echocardiography databases, regional rheumatic fever registers and RHD clinic lists. The setting for this study was a high-income country with continued incidence of acute rheumatic fever (ARF). FINDINGS TO DATE: A Registry cohort of 4959 patients was established. The initial presentation was RHD without recognised prior ARF in 41%, and ARF in 59%. Ethnicity breakdown: Maori 38%, Pacific 33.5%, European 21.9%, other 6.7%. Ethnic disparities have changed significantly over time. Prior to 1960, RHD cases were 64.3% European, 25.3% Maori and 6.7% Pacific. However, in contrast, from 2010 to 2019, RHD cases were 10.7% European, 37.4% Maori and 47.2% Pacific.Follow-up showed 32% had changed region of residence within New Zealand from their initial presentation. At least one cardiac intervention (cardiac surgery, transcatheter balloon valvuloplasty) was undertaken in 64% of the cohort at a mean age of 40 years. 19.8% of the cohort had multiple cardiac interventions. At latest follow-up, 26.9% of the cohort died. Of those alive, the mean follow-up is 20.5+19.4 years. Maori and Pacific led governance groups have been established to provide data governance and oversight for the registry. FUTURE PLANS: Detailed mortality and morbidity of the registry cases will be defined by linkage to New Zealand national health data collections. The contemporary cohort of the registry will be available for future studies to improve clinical management and outcomes for the 3450 individuals living with chronic RHD.

Outcomes of Echocardiography-Detected Rheumatic Heart Disease: Validating a Simplified Score in Cohorts From Different Countries.

Background The natural history of latent rheumatic heart disease (RHD) detected by echocardiography remains unclear. We aimed to assess the accuracy of a simplified score based on the 2012 World Heart Federation criteria in predicting mid-term RHD echocardiography outcomes in children from 4 different countries. Methods and Results Patient-level baseline and follow-up data of children with latent RHD from 4 countries (Australia, n=62; Brazil, n=197; Malawi, n=40; New Zealand, n=94) were combined. A simplified echocardiographic scoring system previously developed from Brazilian and Ugandan cohorts, consisting of 5 point-based variables with respective weights, was applied: mitral valveanterior leaflet thickening (weight=3), excessive leaflettip motion (3), regurgitation jet length ≥2 cm (6), aortic valvefocal thickening (4), and any regurgitation (5). Unfavorable outcome was defined as worsening diagnostic category, persistent definite RHD or development/worsening of valve regurgitation/stenosis. The score model was updated using methods for recalibration. 393 patients (314 borderline, 79 definite RHD) with median follow-up of 36 (interquartile range, 25-48) months were included. Median age was 14 (interquartile range, 11-16) years and secondary prophylaxis was prescribed to 16%. The echocardiographic score model applied to this external population showed significant association with unfavorable outcome (hazard ratio, 1.10; 95% CI, 1.04-1.16; P=0.001). Unfavorable outcome rates in low (≤5 points), intermediate (6-9), and high-risk (≥10) children at 3-year follow-up were 14.3%, 20.8%, and 38.5% respectively (P<0.001). The updated score model showed good performance in predicting unfavorable outcome. Conclusions The echocardiographic score model for predicting RHD outcome was updated and validated for different latent RHD populations. It has potential utility in the clinical and screening setting for risk stratification of latent RHD.

Echocardiography for latent rheumatic heart disease in first degree relatives of children with acute rheumatic fever: Implications for active case finding in family members.

BACKGROUND: Individuals with Acute Rheumatic Fever (ARF) often report a family history of ARF or Rheumatic Heart Disease (RHD) however the degree of familial susceptibility to RHD is poorly defined. This study aimed to determine RHD prevalence among first degree relatives of ARF patients using echocardiography. METHODS: Children with ARF were recruited from Auckland, New Zealand. Parents and siblings ≥ 4years were offered echocardiography. Echocardiograms were reported according to World Heart Federation 2012 criteria. RHD prevalence in first degree relatives was compared to previously established population rates in the region. FINDINGS: In total, 70 index cases with ARF were recruited. Echocardiography was performed in 94 parents and 132 siblings. There were 3 siblings with definite RHD and 9 with borderline RHD. There were 4 parents with definite RHD. Overall prevalence of RHD (definite and borderline) in siblings was 90/1,000 (95% CI 45-143/1,000) compared to 36/1,000 (95% CI 30-42/1,000) in New Zealand children from high ARF incidence populations (p 0.001). Prevalence of definite RHD in parents was 42/1,000 (95% CI 7-87/1,000) compared to 22/1,000 (95% CI 9-36/1,000) in adults from a high ARF incidence New Zealand population (p 0.249). INTERPRETATION: RHD prevalence in siblings and parents of ARF cases is significantly greater than in comparable background populations. The contribution of hereditary versus environmental risk factors remains uncertain. We recommend targeted echocardiographic case-finding among siblings and parents of ARF/RHD cases in order to detect previously unrecognized latent RHD.

The World Heart Federation criteria raise the threshold of diagnosis for mild rheumatic heart disease: Three reviewers are better than one.

BACKGROUND: The World Heart Federation (WHF) criteria, published in 2012, provided an evidence-based guideline for the minimal diagnosis of echocardiographically-detected RHD. Primary aim of the study was to determine whether use of the WHF criteria altered the threshold for the diagnosis of echocardiographically-detected RHD compared with the previous WHO/NIH criteria. A secondary aim was to explore the utility of a three reviewer reporting system compared to a single or two reviewer reporting structure. METHODS: 144 de-identified echocardiograms (RHD, congenital valvar abnormality, physiological valvar regurgitation) were independently reported using the WHF criteria by two reviewers blinded to the previous WHO/NIH diagnosis. If there was discordance between the two reviewers, a third cardiologist independently performed a tie-breaker review. RESULTS: There was a 21% reduction of cases classified as RHD using the WHF criteria compared to the modified WHO/NIH criteria (68 cases compared to 86, p = 0.04). There was a 60% consensus across the different diagnostic categories with 2 reviewers, 89% majority agreement with 3 reviewers. 11% required an open label discussion. There was moderate agreement between 2 reviewers for any RHD, kappa 0.57 (CI 0.44-0.70), with no significant difference in agreement between the different categories. CONCLUSION: The WHF criteria have raised the threshold for the diagnosis of RHD compared to the WHO/NIH criteria. However, inter-reporter variability of the WHF criteria is high. A three reviewer system is likely more accurate than a single or two reporter system for the diagnosis of mild RHD. This has resource implications for echocardiographic screening programmes.

Adequate adherence to benzathine penicillin secondary prophylaxis following the diagnosis of rheumatic heart disease by echocardiographic screening.

AIMS: The primary aim of this study was to determine adherence to benzathine penicillin (BPG) for individuals diagnosed with rheumatic heart disease (RHD) by echocardiographic screening between 2007-2012. METHODS: BPG records were obtained for 57 patients, median age 12 at time of diagnosis. A 'days at risk' analysis was undertaken. Annual adherence was calculated for each individual. A comparison with the Wellington region's Rheumatic Fever 2013 adherence data was undertaken. RESULTS: Adherence to BPG was good with a median follow-up time of 5.8 years. Days at risk analysis: median 0% at year one and 2.7% at year five. The median adherence for the entire cohort over the entire follow-up period was 92%, range 0-100%. There was no difference of proportions of late doses compared to the Wellington region. Median adherence was higher for register based (94%, n=48) compared to primary health care penicillin delivery (37%, n=7), p<0.005. During follow-up, 30% of the cohort moved between regions or overseas. CONCLUSIONS: Good adherence rates are achievable for secondary prophylaxis when RHD is diagnosed by echocardiographic screening. This likely reflects the benefit of rheumatic fever registers and community nursing services rather than the pathway of the diagnosis for RHD.

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.