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.

Background: Methamphetamine-associated cardiomyopathy (MAC) is an increasingly diagnosed condition with poor prognosis, and there is a paucity of literature, including how MAC differs from other cardiomyopathies. This study compared the characteristics and outcomes of MAC patients with non-ischaemic cardiomyopathy controls at the current centre. Methods: Clinical profile, management, and outcomes were prospectively assessed in consecutive patients with MAC at Middlemore Hospital from 2006–2018. They were compared with randomly chosen controls with non-ischaemic dilated cardiomyopathy of a similar age group (20–65 years). Results: Both groups had 62 patients who were followed for 3.0 ± 2.9 years. The MAC patients were younger, with a higher proportion of Maori ethnicity, unemployment and cardiogenic shock during index admission, and a lower proportion of Pacific ethnicity, cardiovascular risk factors, and atrial fibrillation. The MAC patients also had higher peak N-terminal pro-B-type natriuretic peptide (NT-proBNP), lower ejection fraction, and lower attendance rate to outpatient appointments. There was no index admission mortality in either group. The MAC patients had higher mortality and a trend to higher heart failure re-admissions rates during follow-up. Amongst MAC patients, baseline left ventricular end diastolic diameter and failure of improvement in right ventricular systolic function by one category during follow-up were independent predictors of mortality, while failure of improvement of left ventricular ejection fraction by one category predicted heart failure readmission. Conclusions: The MAC patients were younger but sicker on presentation, with higher mortality and trend towards higher heart failure readmission rates during medium-term follow-up than controls. Adherence to therapy and attendance to appointments may improve cardiac systolic function over time, and reduce adverse clinical endpoints.

BACKGROUND: Methamphetamine-associated cardiomyopathy (MAC) is increasingly recognised as a serious consequence of chronic metamphetamine use. Evidence to guide management and prognostication of patients with MAC compared to other cardiomyopathies remain limited. METHODS: Clinical characteristics, in-hospital and post-discharge outcomes were collected in consecutive MAC patients at Middlemore Hospital from 2006-2018, and compared with a 1:1 age-range matched cohort with non-ischaemic cardiomyopathy (NCM). RESULTS: Sixty-two patients (eight females, median age 41 years) with MAC were included. MAC patients were younger than the NCM cohort, and the majority were of indigenous Maori ethnicity. MAC patients had higher peak N-terminal pro B-type natriuretic peptide (NT-proBNP) and lower left ventricular (LV) ejection fraction at presentation. No patients died during index admission. However, there were more MAC patients (10 versus two, P=0.030) with cardiogenic shock at presentation. There were 15 deaths in the MAC patients and seven deaths in the NCM patients during follow-up. MAC patients were at increased mortality risk (HR 2.7, 95% confidence interval 1.1-6.2, P=0.029), and had a trend to more heart failure re-admissions. (HR 1.6, 95% CI 1.0-2.8, P=0.075) compared to NCM patients. Baseline LV end diastolic diameter and failure of improvement in right ventricular systolic function during follow-up were independent predictors of mortality, while failure of improvement in LV ejection fraction predicted heart failure readmission in MAC patients. CONCLUSIONS: MAC patients were more likely to be younger, male, of Maori ethnicity and have a worse prognosis when compared to patients with other non-ischaemic cardiomyopathies.

A 50-year-old woman presented with progressive right heart failure. A chest x-ray showed a right-sided pleural effusion and pericardial calcification. Echocardiography revealed constrictive physiology with preserved biventricular systolic function. There was a small pericardial effusion with an echogenic pericardium. A non-contrast cardiac computed tomography (CT) revealed partial calcification of the visceral and parietal pericardium with a hyperdense pericardial effusion (Hounsfield units (HU) – 150) suggestive of "milk of calcium" pericardial effusion (Fig. 1A & B). Right heart catheterization confirmed constriction with late diastolic equalization of pressures in all cardiac chambers. A large amount of viscous muddy-coloured pericardial fluid (Fig. 1C) was identified during pericardiectomy. Analysis of the pericardial fluid using flame atomic absorption showed a very high calcium content of 830 mmol/L.

BACKGROUND:Valvular heart disease is an important healthcare issue and its impacts are increasing. Following valve surgery, traditional models of care involve medical personnel, however, significant gaps in guideline adherence and delays in follow-up have been reported. Internationally, there is increasing evidence that specialist nurses can function in a variety of clinical settings and improve patient management.METHODS:In 2009, a nurse practitioner clinic to support patients following valve surgery was established. We used a retrospective clinical audit and clinical review with descriptive statistics to describe the development of the clinic and to provide guidance for other services for model of care development.RESULTS:Over four years, 462 patients have been reviewed at least once, with over half having multiple assessments, 37% had rheumatic heart disease. These patients were 20 years younger and more likely to be women, non-European, current smokers and have atrial fibrillation. All patients received a focussed lifestyle, rheumatic, thromboembolic and endocarditis risk and symptom review with tailored support, advice and referral where appropriate. Four percent were referred back to a cardiologist for early evidence of valve dysfunction and a further 1.5% required urgent admission for unstable symptoms.CONCLUSION:The nurse practitioner clinic offers a systematic approach to promoting guideline adherence post valvular surgery. Important clinical symptoms and differences in health needs were identified and were actioned appropriately.

HomeCirculation: Heart FailureVol. 3, No. 6Isolated Primary Cardiac Sarcoidosis Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBIsolated Primary Cardiac SarcoidosisMRI Diagnosis and Monitoring of Treatment Response With Cardiac Enzymes Jonathon White, MB ChB, Tim Sutton, MB ChB, FRACP and Andrew Kerr, MB ChB, FRACP Jonathon WhiteJonathon White From the Department of Cardiology (J.W., T.S., A.K.), Middlemore Hospital, Auckland, New Zealand; and the University of Auckland (A.K.), Auckland, New Zealand. , Tim SuttonTim Sutton From the Department of Cardiology (J.W., T.S., A.K.), Middlemore Hospital, Auckland, New Zealand; and the University of Auckland (A.K.), Auckland, New Zealand. and Andrew KerrAndrew Kerr From the Department of Cardiology (J.W., T.S., A.K.), Middlemore Hospital, Auckland, New Zealand; and the University of Auckland (A.K.), Auckland, New Zealand. Originally published1 Nov 2010https://doi.org/10.1161/CIRCHEARTFAILURE.110.939686Circulation: Heart Failure. 2010;3:e28–e29IntroductionA 48-year-old woman without known cardiovascular disease presented with progressive dyspnea. The ECG showed first-degree heart block. Plasma Troponin I and T were persistently elevated. Chest x-ray was normal. Echocardiography demonstrated asymmetrical left ventricular (LV) hypertrophy with septal predominance and moderately impaired LV systolic function. Coronary angiography demonstrated angiographically normal coronary arteries.Although the echocardiographic features were consistent with a diagnosis of hypertrophic cardiomyopathy, the ECG evidence of conduction abnormality and persistent elevation of cardiac enzymes were more suggestive of a myocarditic process. A cardiac MRI (CMR) was requested. This demonstrated asymmetrical septal hypertrophy with thickening of the basal-mid septum extending to the apex (Figure 1). On delayed-enhancement imaging, multiple discrete areas of hyperenhancement were noted throughout the LV myocardium that deformed the endocardial and epicardial borders (Figure 2). The LV ejection fraction was 31%. The clinical presentation and CMR appearances were suggestive of sarcoidosis; however, high-resolution CT of the thorax to look for confirmatory evidence of pulmonary sarcoidosis showed no evidence of lymphadenopathy or other pulmonary involvement. She therefore proceeded to cardiac biopsy to establish a diagnosis. This showed focal granulomas composed of multinucleated giant cells with central necrosis and associated lymphocytes. Tuberculous and fungal infections were excluded by negative Ziehl-Neelsen and periodic acid-Schiff staining. A diagnosis of isolated primary cardiac sarcoidosis (PCS) was made.Download figureDownload PowerPointFigure 1. Asymmetrical LV hypertrophy shown in CMR steady-state–free precession images in LV short-axis and 3-chamber views.Download figureDownload PowerPointFigure 2. Late gadolinium enhancement (arrows) shown in CMR delayed-enhancement images in 2-, 4-, and 3-chamber projections.The clinical course was complicated by complete heart block. She received an implantable cardiac defibrillator. Prednisone was commenced at 40 mg per day for treatment of PCS, with a rapid response of dyspnea and normalization of the Troponin I within 1 week, remaining suppressed over 6 months of follow-up. Repeat echocardiography at 1 month showed no change in LV function.Cardiac involvement has been reported in up to 30% of patients with sarcoidosis, but the prevalence of isolated PCS is not well defined.1,2 Nonspecific CMR features of sarcoidosis may include segmental wall motion abnormalities in nonvascular distributions and focal wall thickening,3,4 changes that may mimic hypertrophic cardiomyopathy. CMR has been shown to be of greater utility than echocardiography and nuclear imaging modalities (other than PET) in the diagnosis of PCS.4 Although not absolutely contraindicated, the utility of CMR may be limited in patients with pacemakers.4 Cardiac enzymes may be helpful in guiding treatment in patients such as this woman in whom serial CMR evaluations are contraindicated by insertion of pacing devices.AcknowledgmentsWe thank Darren Brown, Biomedical Imaging, Middlemore Hospital.DisclosuresNone.FootnotesCorrespondence to Jonathon White, MB ChB, 17 Norfolk St, Ponsonby, Auckland 1021, New Zealand. E-mail jonathon.[email protected]co.nzReferences1. Butany J, Bahl N, Morales K, Thangaroopan M, Ross H, Rao V, Leong S. The intricacies of cardiac sarcoidosis: a case report involving the coronary arteries and a review of the literature. Cardiovasc Pathol. 2006; 15:222–227CrossrefMedlineGoogle Scholar2. Ayyala US, Nair AP, Padilla ML. Cardiac sarcoidosis. Clin Chest Med. 2008; 29:493–508CrossrefMedlineGoogle Scholar3. Manins V, Habersberger J, Pfluger H, Taylor AJ. Cardiac magnetic resonance imaging in the evaluation of cardiac sarcoidosis: an Australian single-centre experience. Int Med J. 2009; 39:77–82CrossrefMedlineGoogle Scholar4. Smedema J-P, Snoep G, van Kroonenburgh MPG, van Geuns R-J, Dassen WRM, Gorgels APM, Crijns HJGM. Evaluation of the accuracy of gadolinium-enhanced cardiovascular magnetic resonance in the diagnosis of cardiac sarcoidosis. J Am Coll Cardiol. 2005; 45:1683–1690CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Takaya Y, Nakamura K, Nishii N and Ito H (2021) Clinical outcomes of patients with isolated cardiac sarcoidosis confirmed by clinical diagnostic criteria, International Journal of Cardiology, 10.1016/j.ijcard.2021.10.150, 345, (49-53), Online publication date: 1-Dec-2021. Okada D, Bravo P, Vita T, Agarwal V, Osborne M, Taqueti V, Skali H, Chareonthaitawee P, Dorbala S, Stewart G, Di Carli M and Blankstein R (2016) Isolated cardiac sarcoidosis: A focused review of an under-recognized entity, Journal of Nuclear Cardiology, 10.1007/s12350-016-0658-1, 25:4, (1136-1146), Online publication date: 1-Aug-2018. Thangam M, Nathan S, Kar B, Petrovic M, Patel M, Loyalka P, Buja L and Gregoric I (2016) Primary Cardiac Sarcoidosis with Syncope and Refractory Atrial Arrhythmia: A Case Report and Review of the Literature, Texas Heart Institute Journal, 10.14503/THIJ-14-4792, 43:3, (236-240), Online publication date: 1-Jun-2016. Wicks E, Menezes L and Elliott P (2015) Improving the diagnostic accuracy for detecting cardiac sarcoidosis, Expert Review of Cardiovascular Therapy, 10.1586/14779072.2015.1001367, 13:2, (223-236), Online publication date: 1-Feb-2015. Isobe M (2015) Clinical Features of Isolated Cardiac Sarcoidosis, Nihon Naika Gakkai Zasshi, 10.2169/naika.104.120, 104:1, (120-127), . Isobe M and Tezuka D (2015) Isolated cardiac sarcoidosis: Clinical characteristics, diagnosis and treatment, International Journal of Cardiology, 10.1016/j.ijcard.2014.12.056, 182, (132-140), Online publication date: 1-Mar-2015. Kron J, Sauer W, Mueller G, Schuller J, Bogun F, Sarsam S, Rosenfeld L, Mitiku T, Cooper J, Mehta D, Greenspon A, Ortman M, Delurgio D, Valadri R, Narasimhan C, Swapna N, Singh J, Danik S, Markowitz S, Almquist A, Krahn A, Wolfe L, Feinstein S, Ellenbogen K and Crawford T (2015) Outcomes of patients with definite and suspected isolated cardiac sarcoidosis treated with an implantable cardiac defibrillator, Journal of Interventional Cardiac Electrophysiology, 10.1007/s10840-015-9978-3, 43:1, (55-64), Online publication date: 1-Jun-2015. Şentürk A, Maraş Y, Argüder E, Karalezli A, Hasanoğlu H, Öğüt T, Baştuğ S and Karabekir E (2014) What type of different clinical manifestations can cardiac sarcoidosis present?, Rheumatology International, 10.1007/s00296-014-3183-z, 35:6, (1103-1106), Online publication date: 1-Jun-2015. Takaya Y, Kusano K, Nakamura K and Ito H (2015) Comparison of Outcomes in Patients With Probable Versus Definite Cardiac Sarcoidosis, The American Journal of Cardiology, 10.1016/j.amjcard.2015.01.562, 115:9, (1293-1297), Online publication date: 1-May-2015. Sugiyama E, Takenaka T, Kato M, Minoshima A, Muto H, Noriyasu K, Fujita M, Sato M, Betsuyaku T, Inoue H, Okamoto H, Kunishige H, Ishibashi Y, Tsukamoto E, Nakamura H and Hasebe N (2014) Isolated cardiac sarcoidosis requiring open-chest myocardial biopsy for differentiation from malignant lymphoma, Journal of Cardiology Cases, 10.1016/j.jccase.2014.03.006, 9:6, (239-242), Online publication date: 1-Jun-2014. Zacek P, Omran N, Chek J, Krbal L, Vojacek J and Harrer J (2013) Cardiac Sarcoidosis, Journal of Cardiac Surgery, 10.1111/jocs.12163, 28:5, (525-528), Online publication date: 1-Sep-2013. Bogaert J and Taylor A (2011) Heart Muscle Diseases Clinical Cardiac MRI, 10.1007/174_2011_358, (275-353), . Kuwabara M, Ishimura R, Ishiwata S and Ohno M (2018) Isolated Cardiac Sarcoidosis Presenting with Stroke, Korean Circulation Journal, 10.4070/kcj.2017.0133, 48:3, (236) November 2010Vol 3, Issue 6 Advertisement Article InformationMetrics © 2010 American Heart Association, Inc.https://doi.org/10.1161/CIRCHEARTFAILURE.110.939686PMID: 21081735 Manuscript receivedJanuary 20, 2010Manuscript acceptedMay 13, 2010Originally publishedNovember 1, 2010 Keywordstroponinimagingsarcoidosisheart failurePDF download Advertisement SubjectsComputerized Tomography (CT)Heart FailureImaging