We report a previously healthy 73 years old woman, who was hospitalised with increasing dyspnea and signs of congestive heart failure. Echocardiography showed a normal left ventricular cavity with increased echogenicity of its walls and severe pulmonary hypertension. A lung ventilation/perfusion scintigraphy concluded that there was a low probability for pulmonary embolism. Coronary angiography was normal. A restrictive cardiomyopathy due to amyloid deposits was suspected. Myocardial pyrophosphate scintigraphy showed intense pyrophosphate uptake in the left ventricle wall. An abdominal fat tissue biopsy was positive for amyloid deposits.
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Eur J Nucl Med Mol Imaging
January 2025
Department of Internal Medicine, Division of Cardiology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan.
Objective: Tafamidis has shown potential in slowing disease progression in patients with transthyretin amyloid cardiomyopathy (ATTR-CM). This study aimed to evaluate serial changes on [Tc]Tc-pyrophosphate (PYP) scintigraphy during tafamidis treatment for hereditary ATTR-CM.
Methods: We retrospectively analyzed a prospectively collected cohort of Ala97Ser (A97S) hereditary ATTR-CM patients treated with tafamidis (61 mg/day) and a control group comprising A97S hereditary ATTR-CM patients who had not received disease-modifying medications.
Acta Cardiol Sin
January 2025
Division of Cardiology, Cardiovascular Medical Center, and Department of Nuclear Medicine, Far Eastern Memorial Hospital, New Taipei City.
This 2025 updated consensus outlines the diagnostic strategy for transthyretin amyloid cardiomyopathy (ATTR-CM). Given that ATTR-CM is a significant contributor to heart failure, this article emphasizes the importance of making an early and precise diagnosis, particularly as new therapeutic options become available. Highlighting the critical importance of an early and accurate diagnosis, particularly in light of emerging therapeutic modalities, this consensus underscores the central role of Tc-pyrophosphate (PYP) scintigraphy as a non-invasive diagnostic tool.
View Article and Find Full Text PDFSmall Methods
January 2025
Department of Biotechnology, College of Life Science and Biotechnology, Yonsei University, 50 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea.
Rolling circle amplification (RCA)-derived ultra-long DNA is highly attractive and versatile because of its diverse functionalities conferred by repeated DNA nanostructures. However, magnesium pyrophosphate (MgPPi) crystals, as byproducts of RCA, electrostatically interact with the DNA to form DNA microhybrids and hamper its broad bioapplications, as its large size is unfavorable for cellular uptake and decreases the density of functional DNA nanostructures. In this study, finely tuned synthesis strategies are developed to condense the microhybrids and replace non-functional MgPPi crystals with various functional metal nanostructures by reducing metal ions.
View Article and Find Full Text PDFMetabolites
November 2024
Molecular Microbiology and Structural Biochemistry, UMR 5086, CNRS, University Lyon, F-69367 Lyon, France.
Phosphatases are enzymes that catalyze the hydrolysis of phosphate esters. They play critical roles in diverse biological processes such as extracellular nucleotide homeostasis, transport of molecules across membranes, intracellular signaling pathways, or vertebrate mineralization. Among them, tissue-nonspecific alkaline phosphatase (TNAP) is today increasingly studied, due to its ubiquitous expression and its ability to dephosphorylate a very broad range of substrates and participate in several different biological functions.
View Article and Find Full Text PDFJ Card Fail
December 2024
Cardiology Division, Montefiore-Einstein Center for Heart and Vascular Care, Montefiore Medical Center, Albert Einstein College of Medicine. Bronx, NY, USA. Electronic address:
Background: Two diagnostic clinical scoring systems, the ATTR-CM Score and the T-AMYLO Score, have been proposed but not validated in diverse populations, despite Black race being an important risk factor for transthyretin amyloid cardiomyopathy (ATTR-CM). The aim of this study was to evaluate their performance in diagnosing ATTR-CM in a diverse patient cohort.
Methods: This retrospective single-center study analyzed patients who underwent a 99mTc-pyrophosphate single photon emission computed tomography scan (Tc-PYP) for workup of suspected ATTR-CM.
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