2024 Australia-New Zealand Expert Consensus Statement on Cardiac Amyloidosis.

Over the past 5 years, early diagnosis of and new treatments for cardiac amyloidosis (CA) have emerged that hold promise for early intervention. These include non-invasive diagnostic tests and disease modifying therapies. Recently, CA has been one of the first types of cardiomyopathy to be treated with gene editing techniques. Although these therapies are not yet widely available to patients in Australia and New Zealand, this may change in the near future. Given the rapid pace with which this field is evolving, it is important to view these advances within the Australian and New Zealand context. This Consensus Statement aims to update the Australian and New Zealand general physician and cardiologist with regards to the diagnosis, investigations, and management of CA.

Cardiac "hypertrophy" phenotyping: differentiating aetiologies with increased left ventricular wall thickness on echocardiography.

Aims: Differentiating phenotypes of cardiac "hypertrophy" characterised by increased wall thickness on echocardiography is essential for management and prognostication. Transthoracic echocardiography is the most commonly used screening test for this purpose. We sought to identify echocardiographic markers that distinguish infiltrative and storage disorders that present with increased left ventricular (LV) wall thickness, namely, cardiac amyloidosis (CA) and Anderson-Fabry disease (AFD), from hypertensive heart disease (HHT). Methods: Patients were retrospectively recruited from Westmead Hospital, Sydney, and Princess Alexandra Hospital, Brisbane. LV structural, systolic, and diastolic function parameters, as well as global (LVGLS) and segmental longitudinal strains, were assessed. Previously reported echocardiographic parameters including relative apical sparing ratio (RAS), LV ejection fraction-to-strain ratio (EFSR), mass-to-strain ratio (MSR) and amyloidosis index (AMYLI) score (relative wall thickness × E/e') were evaluated. Results: A total of 209 patients {120 CA [58 transthyretin amyloidosis (ATTR) and 62 light-chain (AL) amyloidosis], 31 AFD and 58 HHT patients; mean age 64.1 ± 13.7 years, 75% male} comprised the study cohort. Echocardiographic measurements differed across the three groups, The LV mass index was higher in both CA {median 126.6 [interquartile range (IQR) 106.4-157.9 g/m2]} and AFD [median 134 (IQR 108.8-152.2 g/m2)] vs. HHT [median 92.7 (IQR 79.6-102.3 g/m2), p < 0.05]. LVGLS was lowest in CA [median 12.29 (IQR 10.33-15.56%)] followed by AFD [median 16.92 (IQR 14.14-18.78%)] then HHT [median 18.56 (IQR 17.51-19.97%), p < 0.05]. Diastolic function measurements including average e' and E/e' were most impaired in CA and least impaired in AFD. Indexed left atrial volume was highest in CA. EFSR and MSR differentiated secondary (CA + AFD) from HHT [receiver operating curve-area under the curve (ROC-AUC) of 0.80 and 0.91, respectively]. RAS and AMYLI score differentiated CA from AFD (ROC-AUC of 0.79 and 0.80, respectively). A linear discriminant analysis with stepwise variable selection using linear combinations of LV mass index, average e', LVGLS and basal strain correctly classified 79% of all cases. Conclusion: Simple echocardiographic parameters differentiate between different "hypertrophic" cardiac phenotypes. These have potential utility as a screening tool to guide further confirmatory testing.

Secular trends in the utility of SGLT-2 inhibitors in heart failure patients with type 2 diabetes mellitus across Metro South Health hospitals in South-East Queensland.

BACKGROUND: The 2018 Australian Heart Failure (HF) guidelines strongly recommended commencing sodium-glucose co-transporter-2 inhibitors (SGLT-2is) in HF patients with type 2 diabetes mellitus (T2DM). The uptake of SGLT-2is for HF patients with T2DM in our health service is unknown. AIMS: To determine the adoption of the 2018 HF guidelines by assessing the temporal trends of SGLT-2is' usage in HF patients with T2DM at Metro South Health (MSH) hospitals, in South-East Queensland. METHODS: Retrospective analysis of all HF patients (ejection fraction (EF) < 50%) with T2DM who were managed within MSH hospitals between June 2018 and June 2021. RESULTS: A total of 666 patients met the inclusion criteria with 918 HF encounters. Mean age was 72 years and 71% were male (473/666). Mean EF was 30% (SD ± 11%), and mean estimated glomerular filtration rate was 48 mL/min/1.73 m2 (SD ± 25). Fifty-four per cent (362/666) had contraindications to SGLT-2is. Among those without contraindications, there was a five-fold increase in the utility of SGLT-2is, 7% (2/29) before versus 38% (103/275) after implementation of the HF guidelines (P < 0.001). Patients on SGLT-2is were younger (64 years vs 69 years, P = 0.002) and had a lower number of HF hospitalisations (1.1 vs 2.1, P = 0.01). CONCLUSIONS: During the study period, 54% of our HF patients with T2DM were not on SGLT-2is due to prescribing guidelines/limitations in the Australian context. We observed a five-fold significant increase in the uptake of SGLT-2is before and after implementation of HF guidelines among patients without contraindications to SGLT-2is. There were significantly fewer HF hospitalisations among patients on SGLT-2is compared to those without.

Current approaches to the diagnosis and management of amyloidosis.

Amyloidosis is a collection of diseases caused by the misfolding of proteins that aggregate into insoluble amyloid fibrils and deposit in tissues. While these fibrils may aggregate to form insignificant localised deposits, they can also accumulate in multiple organs to the extent that amyloidosis can be an immediately life-threatening disease, requiring urgent treatment. Recent advances in diagnostic techniques and therapies are dramatically changing the disease landscape and patient prognosis. Delays in diagnosis and treatment remain the greatest challenge, necessitating physician awareness of the common clinical presentations that suggest amyloidosis. The most common types are transthyretin (ATTR) amyloidosis followed by immunoglobulin light-chain (AL) amyloidosis. While systemic AL amyloidosis was previously considered a death sentence with no effective therapies, significant improvement in patient survival has occurred over the past 2 decades, driven by greater understanding of the disease process, risk-adapted adoption of myeloma therapies such as proteosome inhibitors (bortezomib) and monoclonal antibodies (daratumumab) and improved supportive care. ATTR amyloidosis is an underdiagnosed cause of heart failure. Technetium scintigraphy has made noninvasive diagnosis much easier, and ATTR is now recognised as the most common type of amyloidosis because of the increased identification of age-related ATTR. There are emerging ATTR treatments that slow disease progression, decrease patient hospitalisations and improve patient quality of life and survival. This review aims to update physicians on recent developments in amyloidosis diagnosis and management and to provide a diagnostic and treatment framework to improve the management of patients with all forms of amyloidosis.

Transiently elevated estimated pulmonary pressures in a patient with complete heart block undergoing permanent pacing: a case report.

BACKGROUND: Complete heart block (CHB) is a frequent cause for acute admission in older patients with significant cardiac conduction disease. Common presenting symptoms are syncope and dyspnoea. Some patients may exhibit clinical and radiological signs of left ventricular (LV) decompensation, despite preserved LV ejection fraction on transthoracic echocardiography (TTE) and absent pre-existing LV dysfunction. CASE SUMMARY: In this clinical report, we present a case of CHB associated with transient but severe elevation in pulmonary artery systolic pressure, measured as the equivalent right ventricular systolic pressure (RVSP = 99 mmHg) by TTE in the absence of right ventricular outflow tract obstruction, that subsequently 'normalized' after implantation of a permanent pacemaker. After searching our echocardiogram database, we did find other cases with similar findings. DISCUSSION: There is limited literature describing transient acute elevation in estimated pulmonary pressures in the setting of new CHB that is subsequently reversed by permanent pacing. The true prevalence and mechanism of transient estimated pulmonary pressure as a result of CHB remains unknown. Based on our limited assessment, we postulate that the acute elevation in estimated pulmonary pressures is predominantly related to a compensatory augmentation of RV stroke volume and is caused by the underlying bradycardia and need to maintain forward cardiac output. This phenomenon may require further investigation and validation in future studies.

Amyloid Cardiomyopathy.

Amyloid cardiomyopathy is emerging as an important and under-recognised cause of heart failure and cardiac arrhythmias, especially in older adults. This disorder is characterised by extracellular deposition of amyloid fibrils that form due to misfolding of secreted light chains (AL) or transthyretin protein (ATTR). In ATTR, amyloid aggregates typically result from excessive accumulation of wild-type transthyretin (ATTRwt) or from protein structural defects caused by TTR gene variants (ATTRv). Amyloid fibril deposition may predominantly affect the heart or show multi-system involvement. Previously considered to be rare and inexorably progressive with no specific therapy, there has been enormous recent interest in ATTR cardiomyopathy due to upwardly-revised estimates of disease prevalence together with development of disease-modifying interventions. Because of this, there is a clinical imperative to have a high index of suspicion to identify potential cases and to be aware of contemporary diagnostic methods and treatment options. Genetic testing should be offered to all patients with proven ATTR to access the benefits of new therapies specific to ATTRv and allow predictive testing of family members. With heightened awareness of amyloid cardiomyopathy and expanded use of genetic testing, a substantial rise in the numbers of asymptomatic individuals who are carriers of pathogenic variants is expected, and optimal strategies for monitoring and treatment of these individuals at risk need to be determined. Pre-emptive administration of fibril-modifying therapies provides an unprecedented opportunity for disease prevention and promises to change amyloid cardiomyopathy from being a fatal to a treatable disorder.

Cost-Utility Analysis of Home-Based Telerehabilitation Compared With Centre-Based Rehabilitation in Patients With Heart Failure.

BACKGROUND: Whilst home-based telerehabilitation has been shown non-inferior to traditional centre-based rehabilitation in patients with chronic heart failure, its economic sustainability remains unknown. This study aimed to investigate the cost-utility of a home-based telerehabilitation program. METHODS: A comparative, trial-based, incremental cost-utility analysis was conducted from a health care provider's perspective. We collected data as part of a multi-centre, two-arm, non-inferiority, randomised controlled trial with 6 months follow-up. There were 53 participants randomised to either a telerehabilitation program (consisting of 12 weeks of group-based exercise and education delivered into the home via online videoconferencing) or a traditional centre-based program. Health care costs (including personnel, equipment and hospital readmissions due to heart failure) were extracted from health system records, and calculated in Australian dollars using 2013 as the base year. Health utilities were measured using the EuroQol five-dimensional (EQ-5D) questionnaire. Estimates were presented as means and 95% confidence intervals (CIs) based on bootstrapping. Costs and utility differences were plotted on a cost-effectiveness plane. RESULTS: Total health care costs per participant were significantly lower in the telerehabilitation group (-$1,590, 95% CI: -2,822, -359) during the 6 months. No significant differences in quality-adjusted life years (0, 95% CI: -0.06, 0.05) were seen between the two groups. CONCLUSIONS: Heart failure telerehabilitation appears to be less costly and as effective for the health care provider as traditional centre-based rehabilitation.

Patient preferences for the delivery of cardiac rehabilitation.

OBJECTIVE: To elicit patients' preferences for cardiac rehabilitation(CR). METHODS: A Discrete Choice Experiment was used to quantify patients' preferences for the delivery of CR. This survey-based method elicited the relative importance of different characteristics of a program. RESULTS: 200 in-patients eligible to attend CR completed the survey. Over half of the patients strongly preferred a centre-based compared to a home-based program. Many but not all preferred a program starting within two rather than six weeks of discharge and exercise delivered in a group rather than individual setting, with exercise via the internet using telehealth strongly disliked. Some respondents preferred lifestyle information delivered one-to-one by a health professional, and there was an overall preference against delivery by smart phone Apps. Some preferred a program out of rather than within working hours and a shorter program (four weeks compared to eight weeks). CONCLUSIONS: This study provides further insight into patient preferences for a CR program. Although the strongest preferences were for centre-based programs with healthcare professionals facilitating exercise classes and one-on-one education, it is important to offer flexible delivery as one approach will not suit everyone. PRACTICE IMPLICATIONS: There is the potential to improve CR programs by focusing on patient preferences.

Cardiac Sarcoidosis, Left Ventricular Impairment and Chronic Right Ventricular Pacing: Pacing or Pathology?

BACKGROUND: Cardiac sarcoidosis (CS) is an uncommon and under-recognised disease which most frequently presents with atrioventricular (AV) block and may also present with ventricular arrhythmias and left ventricular (LV) systolic dysfunction. Because of its protean clinical manifestations, confirming a diagnosis of CS is often challenging. METHODS: We report two cases where patients presented with atrioventricular (AV) block without evidence of underlying myocardial disease, underwent chronic dual-chamber pacing, and presented several years later with severe LV systolic dysfunction. RESULTS: Both patients were referred for assessment of pacing-induced cardiomyopathy with a view to upgrading their device to cardiac resynchronisation therapy (CRT). Subsequent investigation revealed features consistent with CS and appropriate immunosuppressive therapy resulted in improvement in LV function avoiding the requirement for CRT. CONCLUSION: We present a review of the diagnosis of cardiac sarcoidosis, the importance of imaging modalities and current treatment recommendations.

Exploring patient experiences and perspectives of a heart failure telerehabilitation program: A mixed methods approach.

OBJECTIVES: To describe patient experiences and perspectives of a group-based heart failure (HF) telerehabilitation program delivered to the homes via online video-conferencing. BACKGROUND: Limited information currently exists on patient experiences of telerehabilitation for HF. Patient feedback and end-user perspectives provide important information regarding the acceptability of this new delivery model which may have a substantial impact on future uptake. METHODS: We used mixed-methods design with purposive sampling of patients with HF. We used self-report surveys and semi-structured interviews to measure patient experiences and perspectives following a 12-week telerehabilitation program. The telerehabilitation program encompassed group-based exercise and education, and were delivered in real-time via videoconferencing. Interviews were transcribed and coded, with thematic analysis undertaken. RESULTS: Seventeen participants with HF (mean age [SD] of 69 [12] years and 88% males) were recruited. Participants reported high visual clarity and ease of use for the monitoring equipment. Major themes included motivating and inhibiting influences related to telerehabilitation and improvement suggestions. Participants liked the health benefits, access to care and social support. Participants highlighted a need for improved audio clarity and connectivity as well computer training for those with limited computer experience. The majority of participants preferred a combined face-to-face and online delivery model. CONCLUSION: Participants in this study reported high visual clarity and ease-of-use, but provided suggestions for further improvements in group-based video telerehabilitation for HF.

The Utility of 99mTc-DPD Scintigraphy in the Diagnosis of Cardiac Amyloidosis: An Australian Experience.

BACKGROUND: The uptake of bone-seeking radiotracers in the amyloid heart is well recognised. 99mTc-DPD has been shown to be highly sensitive for cardiac transthyretin (ATTR) amyloid in an overseas population, but is not registered for use in Australia. We explored its utility as a diagnostic tool within our population. METHODS: Patients diagnosed with AL and ATTR (wild-type and inherited) cardiac amyloidosis were prospectively recruited from the Princess Alexandra Hospital Amyloidosis Centre. Patients underwent injection with 99mTc-DPD then planar whole body imaging was performed at 5 minutes post-injection (soft tissue phase) and 3 hours (bone phase). A myocardial SPECT and low amperage CT were acquired after the late whole-body scan. Scans were analysed by two nuclear imaging specialists. Intensity of cardiac 99mTc-DPD uptake was graded as 0 to 3 in accordance with previous criteria, and semiquantitative analysis was performed using a heart to whole body ratio (H:WB) on the 3-hour scan. Patients also underwent electrocardiography and transthoracic echocardiography, and blood samples were taken for troponin I and brain natriuretic peptide levels, to assess for any correlation with DPD uptake. RESULTS: Twenty-one patients (8 AL and 13 ATTR) completed the study. Median age was 58 and 70 years for AL and ATTR patients respectively, and 19 (90.5%) were male. 99mTc-DPD scintigraphy was positive in 2 (25%) of AL, and 13 (100%) of ATTR patients. Grade of cardiac uptake, and mean H:WB (0.1249 v. 0.0794) was greater in the ATTR cohort (p-value<0.001 and 0.001 respectively). No statistically significant correlation was identified between H:WB and echocardiographic parameters. There was a significant positive correlation between H:WB and the PR interval on ECG (p=0.026). CONCLUSIONS: 99mTc-DPD scintigraphy is highly sensitive for the diagnosis of cardiac ATTR amyloid, but less so for AL amyloid.

The Self-care Educational Intervention for Patients With Heart Failure: A Study Protocol.

BACKGROUND: A variety of educational interventions have been implemented to assist patients with heart failure (HF) to maintain their own health, develop self-care behaviors, and decrease readmissions. The most effective approach to education has yet to be established. OBJECTIVE: The aim of this study is to determine the effectiveness of a multimedia educational intervention for patients with HF in reducing hospital readmissions. Secondary outcomes include changes in knowledge and self-care behaviors. METHODS: A randomized controlled trial in a large tertiary referral hospital in Australia has recruited 200 patients and will follow them for 12 months. Patients diagnosed with HF have been randomly allocated 1:1 to either usual education or a multimedia educational intervention. Framed by the principles of adult learning, this individualized intervention was delivered face to face by a specialized HF nurse, with a targeted educational assessment and subsequent development of an educational plan. The multimedia approach combined viewing a DVD and verbal discussion supported by a written manual. The teach-back strategy at the conclusion of the intervention evaluated the patient's learning through 5 key questions about self-management of HF. Readmissions are assessed at 28 days, 3 months, and 12 months. Knowledge and self-care behavior are assessed at baseline, 3 months, and 12 months. CONCLUSIONS: This study evaluates the effectiveness of a targeted multimedia educational intervention. Study results may inform the design of in-hospital education for HF patients.

Assessing functional exercise capacity using telehealth: Is it valid and reliable in patients with chronic heart failure?

Introduction This study aimed to determine the validity and reliability of video-based telerehabilitation assessments in patients with heart failure. Methods Seventeen consecutive participants (mean age 69 years, SD 12 years and 88% males) undertook assessments of three functional tests via both telerehabilitation and face-to-face approaches, on the same day. The assessment order was randomised and conducted by independent assessors. Outcome measures included functional tests: timed up and go (time), six-minute walk (distance), grip strength (kilograms); system usability scale to rate participant experience with telerehabilitation assessment; and number of technical issues encountered. Validity and inter- and intra-rater reliability of telerehabilitation assessments were examined using limits of agreement, intra-class correlation coefficients (ICC), and paired t-tests. Results The limits of agreement for telerehabilitation assessments were within the clinically acceptable limits for timed up and go and grip strength. Telerehabilitation assessments for all functional tests were strongly associated with face-to-face assessments, with ICCs of between 0.85 and 0.96. Inter- and intra-rater reliability of telerehabilitation assessments for all functional tests were excellent (all ICC > 0.95). The mean (SD) system usability scale score was 85 (15)/100. Some incidences of Internet drop-outs, video freezing and auditory fading occurred. Discussion The use of telehealth for the assessment of functional exercise capacity appears to be valid and reliable in patients with heart failure.

Characterisation and Comparison of Acute Haemodynamic, Cardiac Biochemical and Hormonal Response to Different Ventricular Pacing Sites in the Normal Heart.

BACKGROUND: Acute cardiac response to right ventricular pacing is unknown. We aimed to assess the acute haemodynamic, biochemical and hormonal response to asynchronous right ventricular pacing and investigate whether there is a difference between an apical and outflow tract site. METHODS: In 21 patients with normal cardiac function, haemodynamics, brain natriuretic peptide and high sensitive troponin T were measured in response to 10minutes of pacing at each site in a randomised crossover fashion and compared. RESULTS: Pacing both sites there were significant increases in pulmonary capillary wedge pressures (p<0.001) and QRS width (p< 0.01). In comparison to baseline, apical pacing demonstrated significant (p<0.05) increases in arterial peptide and troponin levels and venous peptide levels. Outflow tract pacing compared to baseline demonstrated significant (p<0.05) increases in arterial peptide and venous, arterial and coronary sinus troponin. There were no significant differences in responses between sites. CONCLUSION: Asynchronous right ventricular pacing demonstrated significant increases in filling pressures, cardiac hormonal and biochemical response above baseline with very short durations of pacing (10minutes). There was no difference in response between sites. These findings imply that even very short periods of right ventricular based pacing are potentially deleterious.

A systematic approach to chronic heart failure care: a consensus statement.

The National Heart Foundation of Australia assembled an expert panel to provide guidance on policy and system changes to improve the quality of care for people with chronic heart failure (CHF). The recommendations have the potential to reduce emergency presentations, hospitalisations and premature death among patients with CHF. Best-practice management of CHF involves evidence-based, multidisciplinary, patient-centred care, which leads to better health outcomes. A CHF care model is required to achieve this. Although CHF management programs exist, ensuring access for everyone remains a challenge. This is particularly so for Aboriginal and Torres Strait Islander peoples, those from non-metropolitan areas and lower socioeconomic backgrounds, and culturally and linguistically diverse populations. Lack of data and inadequate identification of people with CHF prevents efficient patient monitoring, limiting information to improve or optimise care. This leads to ineffectiveness in measuring outcomes and evaluating the CHF care provided. Expanding current cardiac registries to include patients with CHF and developing mechanisms to promote data linkage across care transitions are essential. As the prevalence of CHF rises, the demand for multidisciplinary workforce support will increase. Workforce planning should provide access to services outside of large cities, one of the main challenges it is currently facing. To enhance community-based management of CHF, general practitioners should be empowered to lead care. Incentive arrangements should favour provision of care for Aboriginal and Torres Strait Islander peoples, those from lower socioeconomic backgrounds and rural areas, and culturally and linguistically diverse populations. Ongoing research is vital to improving systems of care for people with CHF. Future research activity needs to ensure the translation of valuable knowledge and high-quality evidence into practice.

Repeat six-minute walk tests in patients with chronic heart failure: are they clinically necessary?

BACKGROUND: Owing to a reported learning effect in patients with chronic cardiopulmonary disease, performance of at least two six-minute walk tests (6MWT) are recommended as standard practice. Patients with chronic heart failure (CHF) are typically elderly and frail and it is unknown whether current guidelines are practical in a clinical setting. AIMS: The aim of this study was to determine whether repeat performance of 6MWTs in patients with CHF is related to between-test interval or baseline performance. METHODS: This was a multisite observational study enrolling participants entering into heart failure rehabilitation programmes. Participants performed two 6MWTs with randomly allocated inter-test intervals between 15 and 90 minutes. Distance walked in the second test was compared with the first test using a paired t test. RESULTS: Eighty-eight participants (45 females, age 65 ± 14 years) with stable CHF were enrolled. Mean distance walked increased from 301 metres in test 1 to 313 metres in test 2 (p < 0.001). No significant change was recorded between test 1 and test 2 for those whose baseline distance was <300 metres. The interval between tests had no significant effect on the distance walked. CONCLUSION: The change in 6MWT distance was significantly associated with better baseline performance but not with the interval between tests.