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The Accuracy of Technetium-99 m Pyrophosphate Imaging in Diagnosing Transthyretin Cardiac Amyloidosis and Its Impact on Patient Management.
Curr Cardiol Rep
January 2025
Department of Cardiac Sciences, University of Calgary and Libin Cardiovascular Institute, Calgary, AB, Canada.
Purpose Of Review: This review evaluates recent advancements in Technetium-99 m pyrophosphate (99mTc-PYP) imaging for transthyretin amyloid cardiomyopathy (ATTR-CM). We summarize the advantages of single-photon emission computed tomography (SPECT) over planar imaging, the potential impact of quantitative methods, and emerging data for quantifying response to therapy.
Recent Findings: The current literature demonstrates the superior diagnostic accuracy of SPECT compared with planar imaging in 99mTc-PYP studies.
Cardiac amyloidosis: when to suspect and how to confirm.
J Cardiovasc Med (Hagerstown)
February 2025
Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste.
Diagnosing cardiac amyloidosis (CA) is challenging because of its phenotypic heterogeneity, multiorgan involvement requiring interaction among experts in different specialties and subspecialties, lack of a single noninvasive diagnostic tool, and still limited awareness in the medical community. Missing or delaying the diagnosis of CA may profoundly impact on patients' outcomes, as potentially life-saving treatments may be omitted or delayed. The suspicion of CA should arise when "red flags" for this condition are present, together with increased left ventricular wall thickness.
View Article and Find Full Text PDFEchocardiographic index of left ventricular performance for prognostication in transthyretin cardiac amyloidosis: the central role of stroke volume index.
J Cardiovasc Med (Hagerstown)
February 2025
Cardiology Unit, Azienda Ospedaliera Universitaria di Ferrara, Cona, Ferrara, Italy.
Introduction: Cardiac amyloidosis typically causes restrictive cardiomyopathy, in which the impairment of diastolic function is dominant. Echocardiography provides prognostic information through some important parameters: left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS). However, LVEF often remains preserved despite disease progression, and GLS is not routinely performed as it is limited by suboptimal image quality.
View Article and Find Full Text PDFTransthyretin Cardiac Amyloidosis: Current and Emerging Therapies.
Curr Cardiol Rep
January 2025
The Pauley Heart Center, Virginia Commonwealth University, 1200 East Broad Street West Hospital, 8th Floor, West Wing, Richmond, VA, 23231, USA.
Purpose Of Review: In this article, we describe current and newer TTR stabilizers, TTR silencers which include small interfering RNA agents (siRNA), antisense oligonucleotides (ASO) and CRISPR-Cas9 gene editing, and TTR depleters, which investigates the use of monoclonal antibodies to remove amyloid fibril deposits for patients with advanced disease.
Recent Findings: Once thought to be a rare and fatal condition, increased recognition, improved non-invasive diagnostic tools, and the explosive development of novel therapies, has transformed the landscape of transthyretin amyloid cardiomyopathy (ATTR-CM). Advances in cardiac imaging with respect to echocardiography, cardiac magnetic resonance imaging (CMR), and radionuclide bone scintigraphy has increased the diagnosis of ATTR-CM over the last twenty years.
Epidemiology of transthyretin (ATTR) amyloidosis: a systematic literature review.
Orphanet J Rare Dis
January 2025
Cardiovascular, Renal and Metabolism (CVRM) Evidence, BioPharmaceuticals Medical, AstraZeneca, Gothenburg, Sweden.
Introduction: Significant advances in the treatment of transthyretin (ATTR) amyloidosis has led to an evolving understanding of the epidemiology of this condition. This systematic literature review (SLR) aims to synthesize current evidence on epidemiology and mortality outcomes in ATTR amyloidosis, addressing the need for a comprehensive understanding of its current global impact.
Methods: An SLR of the literature from January 2018 to April 2023 was conducted using the Medline and Embase databases.
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