or transthyretin (ATTR) 2-4 types in the vast majority of cases. ATTR amyloidosis may be acquired, associated with wild-type Background-Cardiac transthyretin (ATTR) amyloidosis is a progressive and fatal cardiomyopathy for which several promising therapies are in development. The diagnosis is frequently delayed or missed because of the limited specificity of echocardiography and the traditional requirement for histological confirmation. It has long been recognized that technetium-labeled bone scintigraphy tracers can localize to myocardial amyloid deposits, and use of this imaging modality for the diagnosis of cardiac ATTR amyloidosis has lately been revisited. We conducted a multicenter study to ascertain the diagnostic value of bone scintigraphy in this disease. Methods and Results-Results of bone scintigraphy and biochemical investigations were analyzed from 1217 patients with suspected cardiac amyloidosis referred for evaluation in specialist centers. Of 857 patients with histologically proven amyloid (374 with endomyocardial biopsies) and 360 patients subsequently confirmed to have nonamyloid cardiomyopathies, myocardial radiotracer uptake on bone scintigraphy was >99% sensitive and 86% specific for cardiac ATTR amyloid, with false positives almost exclusively from uptake in patients with cardiac AL amyloidosis. Importantly, the combined findings of grade 2 or 3 myocardial radiotracer uptake on bone scintigraphy and the absence of a monoclonal protein in serum or urine had a specificity and positive predictive value for cardiac ATTR amyloidosis of 100% (positive predictive value confidence interval, 98.0-100). Conclusions-Bone scintigraphy enables the diagnosis of cardiac ATTR amyloidosis to be made reliably without the need for histology in patients who do not have a monoclonal gammopathy. We propose noninvasive diagnostic criteria for cardiac ATTR amyloidosis that are applicable to the majority of patients with this disease.
Clinical Perspective on p 2412Autopsy studies have shown the presence of cardiac ATTR amyloid deposits in up to 25% of individuals >80 years of age, although in many of these hearts the amount of amyloid was small.8 Nevertheless, among patients with heart failure and preserved ejection fraction, postmortem examination indicates that cardiac amyloid deposition is commoner than in an age-matched autopsy group without heart failure. The majority of patients with cardiac amyloid on postmortem in these studies had not had amyloidosis diagnosed during their lifetime.9 Echocardiography, although a valuable and widely accessible tool for investigating heart failure, is neither sensitive nor specific for cardiac amyloidosis.10 Typical findings on echocardiography include thickening of ventricular walls, restrictive filling, abnormal left and right ventricular longitudinal strain, and atrial septal thickening.11 Cardiac magnetic resonance imaging (CMR) has much greater diagnostic value in cardiac amyloidosis, but false-positive and false-negative CMRs are not infrequent.12 Typical findings ...
DEDICATIONAfter studying medicine at Hanover Medical School and philosophy and social psychology at the Leibniz University Hanover, Omke E. Teebken joined the Christian Albrechts University in Kiel at the end of the 1990s as a research fellow at the Clinic for Cardiovascular Surgery headed by Professor Dr Axel Haverich, whom Omke E. Teebken later followed back to Hanover.In Hanover, besides working as a clinician, Omke E.Teebken was particularly active scientifically, contributing to the establishment of the then newly founded Leibniz Laboratories for Biotechnology and Artificial Organs (LEBAO). His work focused on regenerative medicine and tissue engineering, and subsequently he wrote his habilitation thesis in in this field. After basic training in cardiac surgery, he specialised clinically in vascular surgery and played a pioneering role in the development of this field. Before being appointed director of the Clinic for Vascular Surgery e Endovascular Surgery at the Peine Clinic in 2016, Omke E. Teebken headed the Vascular Surgery e Endovascular Surgery Division of the Department of Cardiothoracic, Thoracic, Transplantation and Vascular Surgery at Hanover Medical School. Professor Teebken was a highly appreciated, committed, and competent colleague and teacher.On 8 April 2019, Professor Teebken passed away after a short and severe illness. He was member and author of the ESVS guideline writing committee, an esteemed colleague, and friend.We will always honor his memory.
accurate recognition at earlier stages. Imaging provides non-invasive tools for follow-up of disease remission/progression complementing clinical evaluation. Additional areas not defined include appropriate clinical indications for imaging, optimal imaging utilization by clinical presentation, accepted imaging methods, accurate image interpretation, and comprehensive and clear reporting. Prospective randomized clinical trial data for the diagnosis of amyloidosis and for imaging-based strategies for treatment are not available. A consensus of expert opinion is greatly needed to guide the appropriate clinical utilization of imaging in cardiac amyloidosis.
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