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.

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.

Rheumatic fever recurrences in New Zealand 2010-14.

AIM: To describe the epidemiology and clinical characteristics of recurrences of acute rheumatic fever (ARF) in New Zealand 2010-14. METHOD: Retrospective hospital chart review for ARF with repeat hospital admissions from 2010-14, to identify recurrences of ARF. Definitions of recurrence as per NZ Heart Foundation Guidelines. RESULTS: There were 65 episodes of recurrent ARF among 60 patients. Maori 51%, Pacific 49%. Arthritis and carditis were the most common major manifestations. Median age at recurrence 21.6 years, (8-42 years), with 83% patients over 15 years. There were 841 first episodes of ARF in New Zealand in 2010-4. Overall New Zealand ARF recurrence rate was 7.2% (CI 5.5-8.9%). The recurrence rate was 4% for those under 16 years, 16% for those aged 16-20 and 25% for those >20 years (p<0.05). Seventy-three percent of recurrences occurred in the Auckland region. Recurrences of ARF were strongly associated with RHD progression. CONCLUSION: The risk of recurrence of ARF in New Zealand is low for children. In contrast, recurrences of ARF in New Zealand occur predominantly after age 15, and disproportionately in the Auckland DHBs. Current medical systems and registers may not be meeting the needs of adolescents and adults requiring secondary prophylaxis.

Mismatches between health service delivery and community expectations in the provision of secondary prophylaxis for rheumatic fever in New Zealand.

OBJECTIVE: Rheumatic fever (RF) recurrence prevention requires secondary prophylaxis for at least ten years. However, recurrences of rheumatic fever (RRF) persist disproportionately affecting Maori and Pacific youth. Reasons for recurrence rates are not well understood and commonly attributed to patient non-adherence. This research explored Maori and Pacific family experiences of RRF to better understand barriers to accessing secondary prophylaxis to inform health service improvements. METHODS: Participants were Maori and Pacific patients who had RRF or unexpected rheumatic heart disease and their family; and health professionals working in RF contexts. Kaupapa Maori, Talanga and Kakala Pacific qualitative methodologies were employed. Data were thematically analysed using a general inductive approach. RESULTS: Data collection included 38 interviews with patients and families (n=80), six focus group interviews and nine interviews with health providers (n=33) from seven geographic regions. Three key themes were identified where mismatches occurred between services and community needs: 1. Model of delivery; 2. Interpersonal approaches to care; and 3. Adolescent care. Conclusions and Public health implications: Successful RRF prevention requires interventions to address structural causes of inequity, appropriate clinical guidelines and quality health services. Service-delivery models should provide regular prophylaxis in an accessible manner through culturally-safe, community-based, age-appropriate care.

Invasive group A streptococcal infection and vaccine implications, Auckland, New Zealand.

We aimed to assess the effect of invasive group A streptococcal (GAS) infection and the potential effects of a multivalent GAS vaccine in New Zealand. During January 2005-December 2006, we conducted prospective population-based laboratory surveillance of Auckland residents admitted to all public hospitals with isolation of GAS from normally sterile sites. Using emm typing, we identified 225 persons with confirmed invasive GAS infection (median 53 years of age; range 0-97 years). Overall incidence was 8.1 cases per 100,00 persons per year (20.4/100,000/year for Maori and Pacific Islanders; 24.4/100,000/year for persons >65 years of age; 33/100,000/year for infants <1 year of age). Nearly half (49%) of all cases occurred in Auckland's lowest socioeconomic quintile. Twenty-two persons died, for an overall case-fatality rate of 10% (63% for toxic shock syndrome). Seventy-four percent of patients who died had an underlying condition. To the population in our study, the proposed 26-valent vaccine would provide limited benefit.

The primary prevention of rheumatic fever.

Rheumatic fever (RF), caused by untreated group A streptococcal (GAS) pharyngitis, is a major cause of morbidity and mortality throughout much of the less developed world and disadvantaged populations (Indigenous and other) in the developed world. Through systematic literature searches, our group has identified potential risk factors for RF and possible interventions for its prevention. The causes can be divided into biological factors, socio-economic, and lifestyle factors and health-care systems and services. Currently, the most promising medical areas look to be improving access to health care and introducing community and school-based sore throat interventions (which aim to diagnose and treat GAS pharyngitis). We could find no convincing support for skin sepsis causing RF. Overall evidence suggests that measures that aim to alleviate poverty and crowding may also reduce the incidence of RF. In comparatively rich countries such as New Zealand and Australia, urgent measures based on available evidence should be undertaken to reduce the very striking health disparity seen with RF and its sequela, rheumatic heart disease in our at-risk populations.

Guidelines for sore throat management in New Zealand.

AIMS: Untreated group A streptococcal (GAS) sore throats can cause acute rheumatic fever (ARF), with permanent cardiac damage in 30%. An algorithm and guideline for appropriate screening and management of sore throats in the New Zealand setting was crafted to guide appropriate treatment in high risk rheumatic fever areas and reduce antibiotic use in low risk areas. METHODS: Three American sore throat guidelines were used as a framework, and searches were made of databases including Medline, Old Medline, Cochrane, DARE, Central, NHS EED, WHOLIS, (www.clinicalevidence.com), and (www.pubmed.gov). No European guidelines were available. RESULTS: A guideline for GAS pharyngitis was created, including an algorithm which stratifies patients according to their risk of ARF. Revised Centor criteria to determine the likelihood of GAS pharyngitis were used. Patients at medium or high risk for GAS pharyngitis should have throat swabs taken if possible. Recommended treatment tables are given. CONCLUSIONS: Patients presenting with sore throats need to have their risk of developing rheumatic fever assessed, and then the risk of GAS pharyngitis determined. Appropriate antibiotic therapy is instigated on the basis of proven or likely GAS pharyngitis. Ten days of oral penicillin V is the first-line treatment. Other household members are at high risk of infection. Unnecessary antibiotic use is a guiding principle in low risk patients.