Purpose Of Review: The purpose of this review is to highlight the increasing importance of cardiac magnetic resonance (CMR) imaging in diagnosing and managing cardiac amyloidosis, especially given the recent advancements in treatment options.
Recent Findings: This review emphasizes the crucial role of late gadolinium enhancement (LGE) with phase-sensitive inversion recovery (PSIR) techniques in both diagnosing and predicting patient outcomes in cardiac amyloidosis. The review also explores promising new techniques for diagnosing early-stage disease, such as native T1 mapping and ECV quantification. Additionally, it delves into experimental techniques like diffusion tensor imaging, MR elastography, and spectroscopy.
Summary: This review underscores CMR as a powerful tool for diagnosing cardiac amyloidosis, assessing risk factors, and monitoring treatment response. While LGE imaging remains the current best practice for diagnosis, emerging techniques such as T1 mapping and ECV quantification offer promise for improved detection, particularly in early stages of the disease. This has significant implications for patient management as newer therapeutic options become available for cardiac amyloidosis.
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http://dx.doi.org/10.1097/HCO.0000000000001166 | DOI Listing |
Eur J Nucl Med Mol Imaging
January 2025
Department of Biomedical Imaging and Image-guided Therapy, Division of Nuclear Medicine, Medical University of Vienna, Spitalgasse 23, Vienna, 1090, Austria.
Purpose: Advancements of deep learning in medical imaging are often constrained by the limited availability of large, annotated datasets, resulting in underperforming models when deployed under real-world conditions. This study investigated a generative artificial intelligence (AI) approach to create synthetic medical images taking the example of bone scintigraphy scans, to increase the data diversity of small-scale datasets for more effective model training and improved generalization.
Methods: We trained a generative model on Tc-bone scintigraphy scans from 9,170 patients in one center to generate high-quality and fully anonymized annotated scans of patients representing two distinct disease patterns: abnormal uptake indicative of (i) bone metastases and (ii) cardiac uptake indicative of cardiac amyloidosis.
R I Med J (2013)
February 2025
Department of Medicine, Division of Cardiology, Alpert Medical School of Brown University, Providence RI.
Cardiac amyloidosis (CA) is an infiltrative disease that results from the deposition of amyloid fibrils in the myocardium, resulting in restrictive cardiomyopathy. The amyloid fibrils are predominantly derived from two parent proteins, immunoglobulin light chain (AL) and transthyretin (ATTR), and ATTR is further classified into hereditary (ATTRv) and wild-type (ATTRwt) based on the presence or absence, respectively, of a mutation in the transthyretin gene. Once thought to be a rare entity, CA is increasingly recognized as a significant cause of heart failure due to improved clinical awareness and better diagnostic imaging.
View Article and Find Full Text PDFFuture Cardiol
January 2025
BridgeBio Pharma, Inc., San Francisco, CA, USA.
Introduction: The 6-minute walk test (6MWT) is used to assess submaximal exercise capacity in clinical trials. Conducting the 6MWT can be challenging when patients cannot visit the clinic due to physical/travel limitations. This pilot study assessed the feasibility of conducting the 6MWT using wearable sensors for patients with transthyretin amyloid cardiomyopathy.
View Article and Find Full Text PDFEur J Neurol
February 2025
Faculty of Medical & Health Sciences, Tel Aviv University, Tel Aviv, Israel.
Background: Hereditary transthyretin (ATTRv) amyloidosis is a rare, adult-onset autosomal-dominant disorder caused by pathogenic variants in the transthyretin (TTR) gene. Data about relevant variants in specific populations and typical initial manifestations may facilitate early diagnosis and treatment. We here describe the genetic landscape of ATTRv amyloidosis in Israel.
View Article and Find Full Text PDFEchocardiography
February 2025
Department of Cardiology, Loyola University Medical Center, Maywood, Illinois, USA.
The left atrium (LA) is pivotal in cardiac hemodynamics, serving as a dynamic indicator of left ventricular (LV) compliance and diastolic function. The LA undergoes structural and functional adaptations in response to hemodynamic stress, infiltrative processes, myocardial injury, and arrhythmic triggers. Remodeling of the LA in response to these stressors directly impacts pulmonary circulation, eventually leading to pulmonary capillary involvement, pulmonary artery hypertension, and eventually right ventricular failure.
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