Prognostic value of technetium-pyrophosphate single-photon emission computed tomography visual grade in patients with transthyretin cardiac amyloidosis on stabilization therapy.

Background: The aim of our study was to evaluate the prognostic value of amyloid burden estimated by technetium-pyrophosphate single-photon emission computed tomography (Tc-PYP-SPECT) visual grade in patients with transthyretin (ATTR) amyloid cardiomyopathy (ATTR-CM) receiving TTR stabilization therapy.

Methods: Our retrospective cohort study included 344 ATTR-CM participants receiving TTR stabilization therapy. Tc-PYP-SPECT myocardial uptake was graded as 0, 1, 2, or 3, based on myocardial uptake that was absent, less than, equal to, or greater than rib uptake, respectively.

Cardiac Amyloidosis in Older Adults With a Focus on Frailty: JACC: Advances Expert Consensus.

Amyloidosis, which is caused by misfolded proteins that form amyloid fibrils, is predominantly diagnosed in older adults. Although previously considered a rare disease, increased awareness and noninvasive diagnostic methods have resulted in a rise in diagnoses. As a multisystem disease that affects multiple organ systems (cardiac, gastrointestinal, renal, and neurological), there is significant overlap with both geriatric conditions and common conditions in heart failure.

Echocardiographic Characterization of Myocardial Stiffness in Healthy Volunteers, Cardiac Amyloidosis, and Hypertrophic Cardiomyopathy: A Case-Control Study Using Multimodality Imaging.

Background: Noninvasive tools to measure myocardial stiffness are limited. Intrinsic cardiac elastography in echocardiography relates to myocardial stiffness by measuring the propagation of the myocardial stretch generated by atrial contraction. The aims of the present study were (1) to evaluate myocardial stiffness using intrinsic cardiac elastography in healthy volunteers versus those with myocardial diseases (ie, cardiac amyloidosis [CA] and hypertrophic cardiomyopathy) and (2) to identify key factors that affect myocardial stiffness.

Development of Imaging Endpoints for Clinical Trials in AL and ATTR Amyloidosis: Proceedings of the Amyloidosis Forum.

Light chain amyloidosis and transthyretin amyloidosis are rare protein misfolding disorders characterized by amyloid deposition in organs, varied clinical manifestations, and poor outcomes. Amyloid fibrils trigger various signaling pathways that initiate cellular, metabolic, structural, and functional changes in the heart and other organs. Imaging modalities have advanced to enable detection of amyloid deposits in involved organs and to assess organ dysfunction, disease stage, prognosis, and treatment response.

Functional Status and Quality of Life in Light-Chain Amyloidosis: Advanced Imaging, Longitudinal Changes, and Outcomes.

Background: In light-chain (AL) amyloidosis, whether functional status and heart failure-related quality of life (HF-QOL) correlate with cardiomyopathy severity, improve with therapy, and are associated with major adverse cardiac events (MACE) beyond validated scores is not well-known.

Objectives: The authors aimed to: 1) correlate functional status and HF-QOL with cardiomyopathy severity; 2) analyze their longitudinal changes; and 3) assess their independent associations with MACE.

Methods: This study included 106 participants with AL amyloidosis, with 81% having AL cardiomyopathy.

Recent Advances in Positron Emission Tomography Radiotracers to Image Cardiac Amyloidosis.

Cardiac amyloidosis includes a group of protein-misfolding diseases characterized by fibril accumulation within the extracellular space of the myocardium and cardiac dysfunction. Cardiac amyloidosis has high mortality. Emerging radionuclide techniques have helped us to better understand disease pathogenesis, prognostication, and treatment response in cardiac amyloidosis.

Quantitative Tc-pyrophosphate myocardial uptake: Changes on transthyretin stabilization therapy.

Background: Quantitative technetium-99m-pyrophosphate cardiac single-photon emission computed tomography (Tc-PYP SPECT/CT) is an emerging method for estimating myocardial burden of transthyretin cardiac amyloidosis (ATTR-CA), but its efficacy in monitoring longitudinal changes remains uncertain. We aimed to investigate longitudinal changes in cardiac ATTR amyloid burden following transthyretin stabilization therapy using visual and quantitative Tc-PYP SPECT/CT and to relate these with changes in cardiac biomarkers and function.

Methods: This prospective longitudinal cohort study investigated changes in Tc-PYP SPECT/CT in 23 participants with ATTR-CA on transthyretin stabilization therapy (median: 2.

Myocardial Characteristics, Cardiac Structure, and Cardiac Function in Systemic Light-Chain Amyloidosis.

Background: In systemic light-chain (AL) amyloidosis, cardiac involvement portends poor outcomes.

Objectives: The authors' objectives were to detect early myocardial alterations, to analyze longitudinal changes with therapy, and to predict major adverse cardiac events (MACE) in participants with AL amyloidosis using cardiac magnetic resonance imaging (MRI).

Methods: Recently diagnosed participants were prospectively enrolled.

Prognostic Value of Left Ventricular F-Florbetapir Uptake in Systemic Light-Chain Amyloidosis.

Background: Positron emission tomography/computed tomography (PET/CT) with F-florbetapir, a novel amyloid-targeting radiotracer, can quantify left ventricular (LV) amyloid burden in systemic light-chain (AL) amyloidosis. However, its prognostic value is not known.

Objectives: The authors' aim was to evaluate the prognostic value of LV amyloid burden quantified by F-florbetapir PET/CT, and to identify mechanistic pathways mediating its association with outcomes.

Mechanisms of left ventricular systolic dysfunction in light chain amyloidosis: a multiparametric cardiac MRI study.

Background: Cardiac systolic dysfunction is a poor prognostic marker in light-chain (AL) cardiomyopathy, a primary interstitial disorder; however, its pathogenesis is poorly understood.

Purpose: This study aims to analyze the effects of extracellular volume (ECV) expansion, a surrogate marker of amyloid burden on myocardial blood flow (MBF), myocardial work efficiency (MWE), and left ventricular (LV) systolic dysfunction in AL amyloidosis.

Methods: Subjects with biopsy-proven AL amyloidosis were prospectively enrolled (April 2016-June 2021; Clinicaltrials.

Quantification of right ventricular amyloid burden with 18F-florbetapir positron emission tomography/computed tomography and its association with right ventricular dysfunction and outcomes in light-chain amyloidosis.

Aims: In systemic light-chain (AL) amyloidosis, quantification of right ventricular (RV) amyloid burden has been limited and the pathogenesis of RV dysfunction is poorly understood. Using 18F-florbetapir positron emission tomography/computed tomography (PET/CT), we aimed to quantify RV amyloid; correlate RV amyloid with RV structure and function; determine the independent contributions of RV, left ventricular (LV), and lung amyloid to RV function; and associate RV amyloid with major adverse cardiac events (MACE: death, heart failure hospitalization, cardiac transplantation).

Methods And Results: We prospectively enrolled 106 participants with AL amyloidosis (median age 62 years, 55% males) who underwent 18F-florbetapir PET/CT, magnetic resonance imaging, and echocardiography.

Myocardial Characterization for Early Diagnosis, Treatment Response Monitoring, and Risk Assessment in Systemic Light-Chain Amyloidosis.

Aims: In systemic light-chain (AL) amyloidosis, cardiac involvement portends poor prognosis. Using myocardial characteristics on magnetic resonance imaging (MRI), this study aimed to detect early myocardial alterations, to analyze temporal changes with plasma cell therapy, and to predict risk of major adverse cardiac events (MACE) in AL amyloidosis.

Methods And Results: Participants with recently diagnosed AL amyloidosis were prospectively enrolled.

Cardiac magnetic resonance biomarkers as surrogate endpoints in cardiovascular trials for myocardial diseases.

Cardiac magnetic resonance offers multiple facets in the diagnosis, risk stratification, and management of patients with myocardial diseases. Particularly, its feature to precisely monitor disease activity lends itself to quantify response to novel therapeutics. This review critically appraises the value of cardiac magnetic resonance imaging biomarkers as surrogate endpoints for prospective clinical trials.

Cardiac Amyloid Quantification Using I-Evuzamitide (I-P5+14) Versus F-Florbetapir: A Pilot PET/CT Study.

Background: Cardiac amyloid quantification could advance early diagnosis of amyloid cardiomyopathy (CMP) and treatment monitoring. However, current imaging tools are based on indirect measurements. I-evuzamitide is a novel pan-amyloid radiotracer binding to amyloid deposits from multiple amyloidogenic proteins.