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.

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.

Abbreviated Echocardiographic Screening for Rheumatic Heart Disease by Nonexperts with and without Offsite Expert Review: A Diagnostic Accuracy Study.

BACKGROUND: Early detection of rheumatic heart disease (RHD) through echocardiographic screening can facilitate early access to effective treatment, which reduces the risk for progression. Accurate, feasible approaches to echocardiographic screening that can be incorporated into routine health services are needed. The authors hypothesized that offsite expert review could improve the diagnostic accuracy of nonexpert-obtained echocardiographic images. METHODS: This prospective cross-sectional study was performed to evaluate the diagnostic accuracy of health worker-conducted single parasternal long-axis view with a sweep of the heart using hand-carried ultrasound for the detection of RHD in high-risk populations in Timor-Leste and Australia. In the primary analysis, the presence of any mitral or aortic regurgitation met the criteria for a positive screening result. Sensitivity and specificity were calculated for a screen-and-refer approach based on nonexpert practitioner assessment (approach 1) and for an approach using offsite expert review of nonexpert practitioner-obtained images to decide onward referral (approach 2). Each participant had a reference test performed by an expert echocardiographer on the same day as the index test. Diagnosis of RHD was determined by a panel of three experts, using 2012 World Heart Federation criteria. RESULTS: The prevalence of borderline or definite RHD among 3,329 participants was 4.0% (95% CI, 3.4%-4.7%). The sensitivity of approach 1 for borderline or definite RHD was 86.5% (95% CI, 79.5%-91.8%), and the specificity was 61.4% (95% CI, 59.7%-63.1%). Approach 2 achieved similar sensitivity (88.4%; 95% CI, 81.5%-93.3%) and improved specificity (77.1%; 95% CI, 75.6%-78.6%). CONCLUSION: Nonexpert practitioner-obtained single parasternal long-axis view with a sweep of the heart images, reviewed by an offsite expert, can detect borderline and definite RHD on screening with reasonable sensitivity and specificity. Brief training of nonexpert practitioners with ongoing support could be used as an effective strategy for scaling up echocardiographic screening for RHD in high-risk settings.

Short-term DAPT after coronary stenting has similar ischemic and bleeding outcomes as long-term DAPT: a 5-year population-based cohort study.

BACKGROUND: Optimal duration of dual antiplatelet therapy (DAPT) following percutaneous coronary intervention (PCI) remains controversial. AIM: We investigated the relationship between DAPT duration following PCI and long-term ischemic and bleeding outcomes under real-world conditions. METHODS: Patients aged ≥ 65 years who underwent PCI with stenting in Western Australian hospitals between 2003 and 2008 and survived 2 years were identified from linked hospital admissions data. The primary outcome was major adverse cardiovascular and cerebrovascular events (MACCE) defined as a composite of all-cause death and admissions for acute coronary syndrome (ACS), coronary artery revascularization procedure, stroke, and major bleeding. Secondary outcomes were ACS admissions, all-cause death, and major bleeding admissions. Patients were followed up for 5 years from initial PCI. RESULTS: A total of 3963 patients were included in the final analysis. The mean age of the cohort was 74.5 ± 6.1 years with 67.3% males. No significant difference was seen with 6-12, 12-18, or 18-24 months DAPT, compared to 0-6 months DAPT duration for MACCE and all secondary outcomes at 3- and 5-year post-PCI. CONCLUSION: There is no significant difference in both bleeding and ischemic outcomes in long-term DAPT as compared to short-term DAPT for first- and second-generation drug-eluting stents in a real-world population.

Inpatient cardiac care for acute coronary syndromes in the Top End of Australia.

BACKGROUND: Disparities in cardiovascular outcomes between Aboriginal and Torres Strait Islander Australians and non-Indigenous Australians persist. This has previously been attributed to a combination of differences in burden of cardiovascular disease risk factors, and inpatient access to guideline-recommended care. AIMS: To assess differences in inpatient access to guideline-recommended acute coronary syndrome (GR-ACS) treatment between Aboriginal and Torres Strait Islander and non-indigenous patients admitted to Royal Darwin Hospital (RDH) with index ACS event. METHODS: This retrospective study included index ACS admissions (n = 288) to RDH between January 2016 and June 2017. Outcomes included rates of coronary angiography, percutaneous coronary intervention (PCI), surgical revascularisation, GR-ACS medications prescribed on discharge and short-term outcomes (30-day mortality and ACS readmissions; 12-month all cardiac-related readmissions). RESULTS: Two hundred and eighty-eight patients, including 109 (37.85%) Aboriginal and Torres Strait Islander patients, were included. Compared with non-indigenous patients, they were younger (median age 48 years vs 60 years; P < 0.01), with a greater burden of comorbidities, including diabetes (39% vs 19%; P < 0.01), smoking (68% vs 35%; P < 0.01) and chronic kidney disease (29% vs 5%; P < 0.01). There were no differences in rates of coronary angiography (98% vs 96%; P = 0.24) or PCI (47% vs 57%; P = 0.12), although there was a trend towards surgical revascularisation in Aboriginal and Torres Strait Islander patients (16% vs 8%; P = 0.047). There were no differences in 30-day mortality (1.8% vs 1.7%; P = 0.72), 12-month ACS readmissions (7% vs 4%; P = 0.20) or 12-month cardiac-related readmissions (7% vs 13%; P = 0.11). CONCLUSIONS: Aboriginal and Torres Strait Islander patients received similar inpatient ACS care and secondary prevention medication at discharge, with similar short-term mortality outcomes as non-indigenous patients. While encouraging, these outcomes may not persist long term. Further outcomes research is required, with differences compelling consideration of other primary and secondary prevention contributors.

Early Australian experience with intravascular lithotripsy treatment of severe calcific coronary stenosis: IVL in acute/chronic coronary syndromes.

Aims: Calcified coronary stenoses are a serious impediment to optimal stent expansion and can lead to stent failure and catastrophic adverse outcomes. We hereby present early Australian experience with intravascular lithotripsy for the treatment of calcific lesions in acute and chronic coronary syndromes. Methods and results: This was a single-centre retrospective study of all patients treated with intravascular lithotripsy (IVL) between October 2019 and June 2021. Patient demographics, procedural variables, and treatment safety/efficacy outcomes were evaluated. During this period, there were 40 patients and 41 coronary lesions with IVL-assisted percutaneous coronary intervention (PCI) (70% male; mean age 72.8±9.5 years). Indications for PCI were acute coronary syndromes in 25 patients (62.5%), and stable angina in 15 patients (37.5%). Upfront IVL usage occurred in 5% of cases with the rest being bailout procedures due to suboptimal initial balloon predilatation or stent underexpansion. Angiographic success (<20% residual stenosis) occurred in 37 cases (92.5%), with mean residual stenosis of 8.25%±8.5%. Two patients experienced procedural complications (5%). Conclusions: IVL appears to be a safe and effective modality in modifying coronary calcium to achieve optimal stent expansion in real-world practice. This device obviates the need for more complex lesion preparation strategies such as rotational or orbital atherectomy.

Single-View Echocardiography by Nonexpert Practitioners to Detect Rheumatic Heart Disease: A Prospective Study of Diagnostic Accuracy.

BACKGROUND: Echocardiographic screening can detect asymptomatic cases of rheumatic heart disease (RHD), facilitating access to treatment. Barriers to implementation of echocardiographic screening include the requirement for expensive equipment and expert practitioners. We aimed to evaluate the diagnostic accuracy of an abbreviated echocardiographic screening protocol (single parasternal-long-axis view with a sweep of the heart) performed by briefly trained, nonexpert practitioners using handheld ultrasound devices. METHODS: Participants aged 5 to 20 years in Timor-Leste and the Northern Territory of Australia had 2 echocardiograms: one performed by an expert echocardiographer using a GE Vivid I or Vivid Q portable ultrasound device (reference test), and one performed by a nonexpert practitioner using a GE Vscan handheld ultrasound device (index test). The accuracy of the index test, compared with the reference test, for identifying cases with definite or borderline RHD was determined. RESULTS: There were 3111 enrolled participants; 2573 had both an index test and reference test. Median age was 12 years (interquartile range, 10-15); 58.2% were female. Proportion with definite or borderline RHD was 5.52% (95% CI, 4.70-6.47); proportion with definite RHD was 3.23% (95% CI, 2.61-3.98). Compared with the reference test, sensitivity of the index test for definite or borderline RHD was 70.4% (95% CI, 62.2-77.8), specificity was 78.1% (95% CI, 76.4-79.8). CONCLUSIONS: Nonexpert practitioners can be trained to perform single parasternal-long-axis view with a sweep of the heart echocardiography. However, the specificity and sensitivity are inadequate for echocardiographic screening. Improved training for nonexpert practitioners should be investigated.

The use of cardiac valve procedures for rheumatic heart disease in Australia; a cross-sectional study 2002-2017.

BACKGROUND: Australia, although a high income economy, carries a significant burden of rheumatic heart disease (RHD). Acute rheumatic fever (ARF) and RHD are endemic in the Indigenous population. Immigrants from low/lower-income countries ('non-Indigenous high-risk') are also at increased risk compared with 'non-Indigenous low-risk' Australians. This study describes the utilisation of surgical and percutaneous procedures for RHD-related valve disease among patients aged less than 50 years, from 2002 to 2017. METHODS: A descriptive study using data from the 'End RHD in Australia: Study of Epidemiology (ERASE) Project' linking RHD Registers and hospital inpatient data from five states/territories, and two surgical databases. Trends across three-year periods were determined and post-procedural all-cause 30-day mortality calculated. RESULTS: A total of 3900 valves interventions were undertaken in 3028 procedural episodes among 2487 patients. Over 50% of patients were in the 35-49 years group, and 64% were female. Over 60% of procedures for 3-24 year-olds were for Indigenous patients. There were few significant changes across the study period other than downward trends in the number and proportion of procedures for young Indigenous patients (3-24 years) and 'non-Indigenous/low risk' patients aged ≥35 years. Mitral valve procedures predominated, and multi-valve interventions increased, including on the tricuspid valve. The majority of replacement prostheses were mechanical, although bioprosthetic valve use increased overall, being highest among females <35 years and Indigenous Australians. All-cause mortality (n = 42) at 30-days was 1.4% overall (range 1.1-1.7), but 2.0% for Indigenous patients. CONCLUSIONS: The frequency of cardiac valve procedures, and 30-day mortality remained steady across 15 years. Some changes in the distribution of procedures in population groups were evident. Replacement procedures, the use of bioprosthetic valves, and multiple-valve interventions increased. The challenge for Australian public health officials is to reduce the incidence, and improve the early detection and management of ARF/RHD in high-risk populations within Australia.

Hyperendemic rheumatic heart disease in a remote Australian town identified by echocardiographic screening.

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.

The Utility of Whole Body 18F-FDG PET-CT in Diagnosing Isolated Cardiac Sarcoidosis: The Western Australian Cardiac Sarcoid Study.

BACKGROUND: It is reported that up to 29-52% of patients with cardiac sarcoidosis (CS) may have isolated cardiac sarcoidosis (ICS). The wide variation in prevalence may be related to the diagnostic methods for assessing extracardiac involvement. Whole-body 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) imaging is a useful and increasingly used technique for screening for extracardiac involvement in cases of suspected ICS. This study aims to determine the rate of isolated cardiac involvement with clinically manifest CS using cardiac 18F-FDG PET-CT. METHODS: We performed a retrospective analysis of data in the West Australian Cardiac Sarcoid (WACaS) Database. After cardiologist review and workup, all cases of proven or probable CS, based on either current Heart Rhythm Society criteria for the diagnosis of CS or local expert consensus were included. Only patients who underwent whole body 18F-FDG PET-CT were included in the final analysis. RESULTS: Fifty-two (52) cases of CS were identified. Data on symptoms, imaging findings, treatment and outcomes were collected. Of the 42 patients who underwent diagnostic 18F-FDG PET-CT, 32 demonstrated changes consistent with CS. Of the 32, 69% were male, mean age 50 years at diagnosis. Only 3/32 (9.4%) patients had ICS. Pulmonary involvement occurred in 91% with varied involvement in other organs. The mean number of extracardiac sites at diagnosis was 2.2. CONCLUSIONS: This study demonstrates the utility of 18F-FDG PET-CT in diagnosing extracardiac organ involvement in cases of CS. With the use of this modality, ICS may be rarer than previously reported.

Echocardiography in Indigenous Populations and Resource Poor Settings.

The majority of global cardiovascular disease burden occurs in low- and middle-income countries (LMIC) and indigenous populations. Although common diseases, such as ischaemic heart disease, cause significant burden, there are also neglected diseases. Forgotten by many, these diseases-including rheumatic heart disease, endomyocardial fibrosis and Chagas cardiomyopathy-continue to take a tremendous toll on a large proportion of the world's population. Whilst the technology of echocardiography continues to evolve in many high-income countries, low resource countries are working out how to make this vital tool available and affordable for the most remote and poorest populations. This paper aims to highlight the neglected cardiovascular diseases and their echocardiographic features. It also highlights the latest research in relation to portable echocardiography, task shifting and disease screening. The authors make recommendations in relation to future directions, including making echocardiography an affordable and accessible tool for all.

Corynebacterium diphtheriae-infective endocarditis in a patient with an atrial septal defect closure device.

An 18-year-old woman presented to our institution with fever, bilateral flank pain, headache and photophobia. She had a previous atrial septal defect (ASD) closure device inserted at the age of 9 years. Blood cultures on admission were positive for Corynebacterium diphtheriae, and transoesophageal echocardiogram (TOE) revealed an echodensity associated with the ASD closure device, most consistent with a vegetation. She was treated for infective endocarditis with 6 weeks of intravenous benzylpenicillin, and follow-up TOE showed resolution of the echodensity. To our knowledge, no cases of C. diphtheriaeendocarditis of an ASD closure device have previously been reported.

Heart failure following oncological treatment.

PURPOSE OF REVIEW: The aim of this review is to give the reader an up-to-date overview of the progress made in the burgeoning field of cardio-oncology, encompassing oncological treatments conferring risk, prediction strategies to identify patients at risk, imaging and biomarker monitoring for emergent or subclinical toxicity and prevention in primary and secondary settings with a focus on heart failure. RECENT FINDINGS: The rapid recent advances in cancer management, particularly with the expansion of targeted and immunotherapies, have led to substantial improvements in outcome, but have also added to the potential causes of cardiac toxicity, which can lead to heart failure. Against this, there has been progression in the field of imaging for cardiac toxicity, identification of at-risk individuals and the clarification of the role of therapy for prevention and treatment of cardiac toxicity. SUMMARY: The findings described in this review provide guidance to clinicians in order to direct monitoring strategy and therapy choice, both in the individual with preexisting cardiac comorbidities and in those predicted to be at the highest risk of cardiac toxicity wherever therapy elements carrying cardiac risk are considered oncologically appropriate.

Current attitudes on cardiac devices in heart failure: a review.

PURPOSE: Despite significant advances in optimizing drug therapy, heart failure-related mortality and morbidity remain high. There has been great progression with regard to device therapy in heart failure, and device use continues to increase. The aims of this review were to critically re-examine the evidence base and to highlight recent refinements in device therapy in heart failure. METHODS: Significant contemporary clinical trials and registries of device therapy in heart failure were examined and critically reviewed to draw conclusions on the clinical applications of implantable cardioverter-defibrillators, cardiac resynchronization therapy, remote monitoring of devices, and hemodynamic monitoring. FINDINGS: Advances regarding patient selection, technology, and implementation for the use of devices in heart failure have significantly improved outcomes. IMPLICATIONS: This review article provides a contemporary guide to the current attitudes toward the use of devices in heart failure. Device therapy is an important adjuvant to optimal pharmacologic therapy. The role of devices continues to increase, and devices have a positive impact on patients' quality of life and survival.