The Spectrum, Severity and Outcomes of Rheumatic Mitral Valve Disease in Pregnant Women in Australia and New Zealand.

BACKGROUND: Rheumatic heart disease (RHD) poses significant perinatal risks. We aimed to describe the spectrum, severity and outcomes of rheumatic mitral valve disease in pregnancy in Australia and New Zealand. METHODS: A prospective, population-based cohort study of pregnant women with RHD recruited 2013-14 through the hospital-based Australasian Maternity Outcomes Surveillance System. Outcome measures included maternal and perinatal morbidity and mortality. Univariable and multivariable logistic regression analyses were undertaken to test for predictors of adverse maternal and perinatal outcomes. RESULTS: Of 274 pregnant women identified with RHD, 124 (45.3%) had mitral stenosis (MS) and 150 (54.7%) had isolated mitral regurgitation (MR). One woman with mild MS/moderate MR died. There were six (2.2%) stillbirths and two (0.7%) neonatal deaths. Babies born to women with MS were twice as likely to be small-for-gestational-age (22.7% vs 11.4%, p=0.013). In women with MS, use of cardiac medication (AOR 7.42) and having severe stenosis (AOR 16.35) were independently associated with adverse cardiac outcomes, while New York Heart Association (NYHA) class >1 (AOR 3.94) was an independent predictor of adverse perinatal events. In women with isolated MR, use of cardiac medications (AOR 7.03) and use of anticoagulants (AOR 6.05) were independently associated with adverse cardiac outcomes. CONCLUSIONS: Careful monitoring and specialist care for women with RHD in pregnancy is required, particularly for women with severe MS, those on cardiac medication, and those on anticoagulation, as these are associated with increased risk of adverse maternal cardiac outcomes. In the context of pregnancy, contraception and preconception planning are important for young women diagnosed with 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.

Bronchiectasis in indigenous and non-indigenous residents of Australia and New Zealand.

BACKGROUND AND OBJECTIVE: Bronchiectasis not associated with cystic fibrosis is an increasingly recognized chronic lung disease. In Oceania, indigenous populations experience a disproportionately high burden of disease. We aimed to describe the natural history of bronchiectasis and identify risk factors associated with premature mortality within a cohort of Aboriginal Australians, New Zealand Maori and Pacific Islanders, and non-indigenous Australians and New Zealanders. METHODS: This was a retrospective cohort study of bronchiectasis patients aged >15 years at three hospitals: Alice Springs Hospital and Monash Medical Centre in Australia, and Middlemore Hospital in New Zealand. Data included demographics, ethnicity, sputum microbiology, radiology, spirometry, hospitalization and survival over 5 years of follow-up. RESULTS: Aboriginal Australians were significantly younger and died at a significantly younger age than other groups. Age- and sex-adjusted all-cause mortality was higher for Aboriginal Australians (hazard ratio (HR): 3.9), and respiratory-related mortality was higher for both Aboriginal Australians (HR: 4.3) and Maori and Pacific Islander people (HR: 1.7). Hospitalization was common: Aboriginal Australians had 2.9 admissions/person-year and 16.9 days in hospital/person-year. Despite Aboriginal Australians having poorer prognosis, calculation of the FACED score suggested milder disease in this group. Sputum microbiology varied with Aspergillus fumigatus more often isolated from non-indigenous patients. Airflow obstruction was common (66.9%) but not invariable. CONCLUSIONS: Bronchiectasis is not one disease. It has a significant impact on healthcare utilization and survival. Differences between populations are likely to relate to differing aetiologies and understanding the drivers of bronchiectasis in disadvantaged populations will be key.

Better Indigenous Risk stratification for Cardiac Health study (BIRCH) protocol: rationale and design of a cross-sectional and prospective cohort study to identify novel cardiovascular risk indicators in Aboriginal Australian and Torres Strait Islander adults.

BACKGROUND: Of the estimated 10-11 year life expectancy gap between Indigenous (Aboriginal and Torres Strait Islander people) and non-Indigenous Australians, approximately one quarter is attributable to cardiovascular disease (CVD). Risk prediction of CVD is imperfect, but particularly limited for Indigenous Australians. The BIRCH (Better Indigenous Risk stratification for Cardiac Health) project aims to identify and assess existing and novel markers of early disease and risk in Indigenous Australians to optimise health outcomes in this disadvantaged population. It further aims to determine whether these markers are relevant in non-Indigenous Australians. METHODS/DESIGN: BIRCH is a cross-sectional and prospective cohort study of Indigenous and non-Indigenous Australian adults (≥ 18 years) living in remote, regional and urban locations. Participants will be assessed for CVD risk factors, left ventricular mass and strain via echocardiography, sleep disordered breathing and quality via home-based polysomnography or actigraphy respectively, and plasma lipidomic profiles via mass spectrometry. Outcome data will comprise CVD events and death over a period of five years. DISCUSSION: Results of BIRCH may increase understanding regarding the factors underlying the increased burden of CVD in Indigenous Australians in this setting. Further, it may identify novel markers of early disease and risk to inform the development of more accurate prediction equations. Better identification of at-risk individuals will promote more effective primary and secondary preventive initiatives to reduce Indigenous Australian health disadvantage.

Sharing success - understanding barriers and enablers to secondary prophylaxis delivery for rheumatic fever and rheumatic heart disease.

BACKGROUND: Rheumatic fever (RF) and rheumatic heart disease (RHD) cause considerable morbidity and mortality amongst Australian Aboriginal and Torres Strait Islander populations. Secondary antibiotic prophylaxis in the form of 4-weekly benzathine penicillin injections is the mainstay of control programs. Evidence suggests, however, that delivery rates of such prophylaxis are poor. METHODS: This qualitative study used semi-structured interviews with patients, parents/care givers and health professionals, to explore the enablers of and barriers to the uptake of secondary prophylaxis. Data from participant interviews (with 11 patients/carers and 11 health practitioners) conducted in four far north Queensland sites were analyzed using the method of constant comparative analysis. RESULTS: Deficits in registration and recall systems and pain attributed to injections were identified as barriers to secondary prophylaxis uptake. There were also varying perceptions regarding responsibility for ensuring injection delivery. Enablers of secondary prophylaxis uptake included positive patient-healthcare provider relationships, supporting patient autonomy, education of patients, care givers and healthcare providers, and community-based service delivery. CONCLUSION: The study findings provide insights that may facilitate enhancement of secondary prophylaxis delivery systems and thereby improve uptake of secondary prophylaxis for RF/RHD.

Approaches to Improving Adherence to Secondary Prophylaxis for Rheumatic Fever and Rheumatic Heart Disease: A Literature Review with a Global Perspective.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune conditions resulting from infection with group A streptococcus. Current management of these conditions includes secondary antibiotic prevention. This comprises regular 3 to 4 weekly long-acting intramuscular benzathine penicillin injections. Secondary antibiotic prevention aims to protect individuals against reinfection with group A streptococcus, thereby preventing recurrent ARF and the risk of further damage to the heart valves. However, utilization of benzathine penicillin can be poor leaving patients at risk of avoidable and progressive heart damage. This review utilizes the Chronic Care Model as a framework to discuss initiatives to enhance the delivery of secondary antibiotic prophylaxis for ARF and RHD. Results from the search strategy utilized revealed that there is limited pertinent published evidence. The evidence that is available suggests that register/recall systems, dedicated health teams for delivery of secondary antibiotic prophylaxis, education about ARF and RHD, linkages with the community (particularly between health services and schools), and strong staff-patient relationships may be important. However, it is difficult to generalize findings from individual studies to other settings and high quality studies are lacking. Although secondary antibiotic prophylaxis is an effective treatment for those with ARF or RHD, the difficulties in implementing effective programs that reduce the burden of ARF and RHD demonstrates the importance of ongoing work in developing and evaluating research translation initiatives.

Echocardiographic screening for rheumatic heart disease-some answers, but questions remain.

Despite being preventable, rheumatic heart disease (RHD) remains a significant global cause of cardiovascular disease. Echocardiographic screening for early detection of RHD has the potential to enable timely commencement of treatment (secondary prophylaxis) to halt progression to severe valvular disease. However, a number of issues remain to be addressed regarding its feasibility. The natural history of Definite RHD without a prior history of acute rheumatic fever (ARF) and Borderline RHD are both unclear. Even if they are variants of RHD it is not known whether secondary antibiotic prophylaxis will prevent disease progression as it does in "traditionally" diagnosed RHD. False positives can also have a detrimental impact on individuals and their families as well as place substantial burdens on health care systems. Recent research suggests that handheld echocardiography (HAND) may offer a cheaper and more convenient alternative to standard portable echocardiography (STAND) in RHD screening. However, while HAND is sensitive for the detection of Definite RHD, it is less sensitive for Borderline RHD and is relatively poor at detecting mitral stenosis (MS). Given its attendant limited specificity, potential cases detected with HAND would require re-examination by standard echocardiography. For now, echocardiographic screening for RHD should remain a subject of research rather than routine health care.

Infective endocarditis and rheumatic heart disease in the north of Australia.

BACKGROUND: To prevent infective endocarditis (IE), Australian guidelines recommend providing prophylactic antibiotics to Indigenous patients with rheumatic heart disease (RHD) prior to procedures which may cause bacteremia. In northern Australia RHD remains prevalent. We aimed to determine whether RHD is associated with an increased risk of IE, which risk factors are associated with IE, and the incidence and aetiology of IE. METHODS: A retrospective review of IE patients who fulfilled modified Duke criteria at two tertiary centres in northern Australia. RESULTS: 89 patients were reviewed. The rate of IE was 6.5/100,000 person-years. IE was more common in people with RHD (relative risk (RR) 58), Indigenous Australians (RR 2.0) and men (RR 1.7). RHD-associated IE was not confined to Indigenous Australians with 42% being non-Indigenous. The commonest risk factors for IE were intracardiac prosthetic material, injecting drug use and previous IE. One in five patients died. CONCLUSIONS: In northern Australia the principle risk factor for IE is not RHD. Whilst RHD increased the risk of IE it was not restricted to Indigenous Australians. Current Australian recommendations of providing prophylactic antibiotics to Indigenous patients with RHD prior to procedures which may cause bacteremia may need to be broadened to include non-Indigenous patients.