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Objective: To estimate the burden of transthyretin cardiac amyloidosis (ATTR-CA) through a cross- sectional 'snapshot' of Australian Amyloidosis Network (AAN) and New Zealand (NZ) specialist amyloidosis clinics., Design, Setting & Participants: A prospective survey was performed of seven AAN/ specialist amyloidosis clinics across Australia and NZ. All centres were invited to contribute data; participating centres provided clinical and demographic data for patients with ATTR-CA reviewed in the 2022 calendar year. Patients with new or previously confirmed ATTR-CA reviewed in the 2022 calendar year were included. Diagnosis was established through a positive cardiac scintigraphy scan in the absence of a monoclonal gammopathy or through a cardiac biopsy staining positive with transthyretin (TTR)., Results: A total of 515 patients were reviewed across seven sites. A total of 302/515 (59%) were wild type TTR (ATTRwt), 63/515 (12%) were variant ATTR (ATTRv) and the remaining 150 (29%) had not undergone genetic testing at the time of data collection. A total of 455/515 (88%) patients were male. Compared to ATTRwt, patients with ATTRv had smaller left ventricular (LV) wall thickness (IVSd 14±3 mm vs 16±3mm, p<0.001), and better LV systolic function (LVGLS -15.4±5% vs -11.7±3%, p<0.001). Most patients, 387/515 (75%) were on at least one ATTR specific treatment, including EGCG (157), diflunisal (139), doxycycline (68) and tafamidis (78), acoramidis (33) and gene silencer therapies or monoclonal antibodies (23)., Conclusion: A significant number of patients with ATTR-CA are seen in specialist amyloidosis clinics across Australia and NZ. Most patients received specific amyloidosis therapy, thorough enrollment in clinical trials. With increased recognition of amyloidosis and newer therapies becoming available, the volume of patients seen in these clinics is likely to increase., Competing Interests: Declaration of Competing Interests J.B., N.D., D.K., F.K., K.Y.P., and T.S. declare no relevant disclosures. N.B receives funding from the Ramaciotti Foundations and Fullbright Commission, is a member of the advisory board for Bristol Myers Squibb, and engages in research funded by Novartis and Pfizer. P.G is supported by The National Heart Foundation of Australia PhD Scholarship. S.G receives honoria for speaking engagements with Pfizer, BridgeBio, Alexion/AstraZeneca, Ionis, Intellia, and Attralus, is a member of the advisory board for steering committee payments from Pfizer, and has involvement in clinical trials with Pfizer, Alexion. J.H is a member of the advisory board for Pfizer and has involvement in clinical trials with Pfizer. L.T is a member of the advisory boards for Boehringer Ingelheim, Novartis, Sanofi, and receives research funding from Janssen, Pfizer, Bayer, and Medical Research Future Fund., (Copyright © 2024 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

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., Competing Interests: Conflicts of Interest NB: Novartis, Pfizer—research, Bristol Myers Squibb—advisory board. JLH: Pfizer—advisory board, clinical trials involvement. SDJG: Pfizer, BridgBio, Alexion/Astrazeneca, Ionis, Intellia, Attralus—honoria for speaking engagements, advisory board or steering committee payments, clinical trials involvement. PM: Jansen, Pfizer—research funding/clinical trials involvement. LT: Boehringer Ingelheim, Novartis, Sanofi Genzyme—advisory board, Jansen, Pfizer, Bayer—research funding. No relevant conflicts of interest to declare: DF, JG, DK, FK, KL, DR, HS, TS., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Objectives: A hypertensive response to submaximal exercise is associated with cardiovascular disease but this relationship is influenced by functional capacity. Spironolactone improves functional capacity, which could mask treatment effects on exercise blood pressure. This study sought to examine this hypothesis., Design: Retrospective analysis of a randomized clinical trial., Methods: 102 participants (54 ± 9 years; 52% male) with a hypertensive response to maximal exercise (systolic BP ≥210 mm Hg men; ≥190 mm Hg women) were randomized to 3-month spironolactone 25 mg daily (n = 53) or placebo (n = 49). Submaximal exercise blood pressure was measured during low-intensity cycling (50, 60 or 70% age-predicted maximal heart rate). Functional capacity was measured as maximal oxygen capacity obtained during a maximal treadmill exercise test, and (resting) aortic stiffness by carotid-to-femoral pulse wave velocity., Results: Spironolactone improved submaximal exercise systolic blood pressure vs. placebo (-4 ± 16 vs. 2 ± 15 mm Hg, p = 0.045, Cohen's d = 0.42), and had a small (but non-statistically significant) improvement in functional capacity (0.64 ± 5.10 vs. -1.43 ± 5.04 ml/kg/min, p = 0.06, Cohen's d = 0.4). When treatment effects were expressed as the change in submaximal exercise systolic blood pressure relative to the change in functional capacity, a larger effect size was observed (-0.3 ± 1.1 vs. 0.3 ± 1.1 mm Hg/ml·kg·min -1 , p = 0.01, Cohen's d = 0.58), but was not explained by improved aortic stiffness., Conclusions: Spironolactone reduces submaximal exercise blood pressure, but this treatment effect may be hidden by improved functional capacity and a non-fixed workload. This highlights the most clinically relevant exercise blood pressure is at a low intensity and fixed workload where the influence of fitness on exercise blood pressure is removed, and the effects of therapy can be appreciated., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Patients presenting with a syndrome of chest pain, elevated cardiac enzyme levels with or without electrocardiogram changes are a common diagnostic and management problem in cardiology. Most commonly, this is due to ischaemic myocardial infarction secondary to coronary artery disease. However, when coronary angiography does not demonstrate any obstructive coronary artery lesion, the diagnosis of myocarditis should be considered. Cardiac magnetic resonance imaging is helpful towards making this diagnosis. Here, we describe the first reported Australian cases of recurrent myocarditis presenting with ischaemic chest pain and elevated cardiac enzyme levels. These cases serve as an important reminder to clinicians that myocarditis is an important mimic of ischaemic myocardial infarction., (Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)