Background: Between 1964 and 1996, the 10-year survival of patients having valve replacement surgery for rheumatic heart disease (RHD) in the Northern Territory, Australia, was 68%. As medical care has evolved since then, this study aimed to determine whether there has been a corresponding improvement in the region’s RHD patients’ 10-year survival.Methods: A retrospective study of Aboriginal RHD patients in the Northern Territory, Australia, having their first valve surgery between 1997 and 2016. Survival was examined using Kaplan—Meier and Cox regression analysis.Findings: The cohort included 281 adults and 61 children. The median (interquartile range (IQR)) age at first surgery was 31 (18-42) years; 173/342 (51%) had a valve replacement. Of 281 adults, 204 (73%) had at least one preoperative comorbidity. There were 93/342 (27%) deaths during a median (IQR) follow-up of 8 (4-12) years. The overall 10-year survival was 70% [95% confidence interval (CI): 64-76], while it was 62% [95%CI (53-70)] in those having a valve replacement. Preoperative comorbidity was associated with earlier death, the risk of death increasing with each comorbidity (hazard ratio (HR): 1·3 (95%CI: 1·2-1·5), p<0·001). Preoperative chronic kidney disease (HR: 6·1 (95%CI: 2·8-13·3), p<0·001), coronary artery disease (HR: 2·3 (95%CI: 1·1-5·0), p=0·037) and pulmonary artery systolic pressure >50mmHg before surgery (HR: 2·1 (95%CI: 1·3-3·5), p=0·002) independently predicted death.Interpretation: Survival after RHD surgery in this region of Australia has not improved. Although the patients were young, many had multiple comorbidities which influenced long-term outcomes. The increasing prevalence of complex comorbidity in the region is a barrier to achieving optimal health outcomes.Funding Information: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Declaration of Interests: All co-authors declared no conflict of interest.Ethical Approval: The Human Research Ethics Committees of the Northern Territory Department of Health and Menzies School of Health Research (HREC reference number: 2016-25) and the Australian Institute of Health and Welfare (AIHW reference number: EO2018/5/410) provided ethical approval for the study. The committees waived the requirement for informed consent as the data were retrospective, de-identified at the time of analysis and presented in an aggregated manner.
These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.
Abstract Mitral regurgitation (MR) is the most common lesion in children with rheumatic heart disease (RHD). Progression of RHD results in the need for surgical intervention, the timing of which is dictated by left ventricular dilatation and the onset of heart failure symptoms. We sought to determine whether elevation in trans-mitral pressure gradient (TMPG) in those with moderate or severe rheumatic MR without significant mitral stenosis (MS) could predict the need for future surgical intervention. Echocardiographic studies were reviewed for 116 children and young people with moderate or severe rheumatic MR. Those with significant mitral stenosis or concurrent aortic valve disease were excluded. Trans-mitral pressure gradient was measured at baseline and details of mitral valve surgical intervention were retrieved from a registry database. Time to future surgery (up to six years) was compared between those with TMPG < 5 mmHg and TMPG ≥ 5 mmHg. Survival curves demonstrated an increased risk of surgery for those with TMPG ≥ 5 mmHg with Cox proportional regression analysis providing a hazard ratio of 5.8. The proportion free from mitral valve surgery at one year for the TMPG < 5 mmHg group was 0.94 (95% CI 0.86–0.97), compared to 0.62 (95% CI 0.42–0.77) in the ≥ 5 mmHg group. Trans-mitral pressure gradient is a strong predictor of future mitral valve surgery in children and young people with significant rheumatic MR without MS. This non-invasive measure could be used to signal the need for more aggressive monitoring in order to optimize the timing of intervention.
Background— Echocardiographic screening for rheumatic heart disease (RHD) is becoming more widespread, but screening studies to date have used different echocardiographic definitions. The World Heart Federation has recently published new criteria for the echocardiographic diagnosis of RHD. We aimed to establish the prevalence of RHD in high-risk Indigenous Australian children using these criteria and to compare the findings with a group of Australian children at low risk for RHD. Methods and Results— Portable echocardiography was performed on high-risk Indigenous children aged 5 to15 years living in remote communities of northern Australia. A comparison group of low-risk, non-Indigenous children living in urban centers was also screened. Echocardiograms were reported in a standardized, blinded fashion. Of 3946 high-risk children, 34 met World Heart Federation criteria for definite RHD (prevalence, 8.6 per 1000 [95% confidence interval, 6.0–12.0]) and 66 for borderline RHD (prevalence, 16.7 per 1000 [95% confidence interval, 13.0–21.2]). Of 1053 low-risk children, none met the criteria for definite RHD, and 5 met the criteria for borderline RHD. High-risk children were more likely to have definite or borderline RHD than low-risk children (adjusted odds ratio, 5.7 [95% confidence interval, 2.3–14.1]; P <0.001). Conclusions— The prevalence of definite RHD in high-risk Indigenous Australian children approximates what we expected in our population, and no definite RHD was identified in the low-risk group. This study suggests that definite RHD, as defined by the World Heart Federation criteria, is likely to represent true disease. Borderline RHD was identified in children at both low and high risk, highlighting the need for longitudinal studies to evaluate the clinical significance of this finding.
Abstract Introduction Echocardiographic screening can detect rheumatic heart disease (RHD) in high-risk populations,but is limited by reliance on highly-trained experts and equipment. We sought to determine the diagnostic utility of an ultra-abbreviated single parasternal-long-axis-sweep of the heart (SPLASH) echocardiography protocol to detect RHD, performed by briefly-trained health workers. Methods In Timor-Leste and Northern Australia, individuals aged 5–20y were offered school-based echocardiographic screening. Health workers completed online modules followed by one-week of practical training, logging 50 echocardiograms prior to study. The index test was SPLASH, performed and reported by health workers using handheld GE V-scan devices. The index test was abnormal if any mitral or aortic regurgitation was detected. The reference test was a comprehensive echocardiogram, performed by an echocardiographers or cardiologist on a GE Vivid-Q, reported according to World Heart Federation criteria. The diagnostic accuracy of the index test was determined. Results 2590 subjects underwent index and reference tests. Prevalence of definite RHD was 3.2% (83/2590). Sensitivity and specificity of index test were 0.75 (95% CI 0.64–0.83) and 0.77 (95% CI 0.75–0.78) respectively for detection of any definite RHD, and 0.91 (95% CI 0.74–0.98) and 0.76 (95% CI 0.74–0.78) respectively for detection of moderate or severe RHD. Conclusion Health workers using SPLASH detected the vast majority of moderate and severe RHD cases, but lacked sensitivity for detection of mild RHD. Further analysis is underway to evaluate the learning curve and other performance indicators of health workers performing and interpreting echocardiograms. This will allow refinement of SPLASH protocol and augmentation of health worker training to increase detection rates and accuracy for future population screening initiatives. Acknowledgement/Funding Heart Foundation Australia Vanguard Grant, Menzies HOT-NORTH pilot project grant, Snow Foundation, Rotary, Bawinanga Aboriginal Corporation, Mala'la
Objectives Using echocardiographic screening, to estimate the prevalence of rheumatic heart disease (RHD) in a remote Northern Territory town. Design Prospective, cross-sectional echocardiographic screening study; results compared with data from the NT rheumatic heart disease register. Setting, participants People aged 5–20 years living in Maningrida, West Arnhem Land (population, 2610, including 2366 Indigenous Australians), March 2018 and November 2018. Intervention Echocardiographic screening for RHD by an expert cardiologist or cardiac sonographer. Main outcome measures Definite or borderline RHD, based on World Heart Federation criteria; history of acute rheumatic fever (ARF), based on Australian guidelines for diagnosing ARF. Results The screening participation rate was 72%. The median age of the 613 participants was 11 years (interquartile range, 8–14 years); 298 (49%) were girls or women, and 592 (97%) were Aboriginal Australians. Definite RHD was detected in 32 screened participants (5.2%), including 20 not previously diagnosed with RHD; in five new cases, RHD was classified as severe, and three of the participants involved required cardiac surgery. Borderline RHD was diagnosed in 17 participants (2.8%). According to NT RHD register data at the end of the study period, 88 of 849 people in Maningrida and the surrounding homelands aged 5–20 years (10%) were receiving secondary prophylaxis following diagnoses of definite RHD or definite or probable ARF. Conclusion Passive case finding for ARF and RHD is inadequate in some remote Australian communities with a very high burden of RHD, placing children and young people with undetected RHD at great risk of poor health outcomes. Active case finding by regular echocardiographic screening is required in such areas.
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