Comparison of Six-Month Outcomes in Patients with Cardiac Amyloidosis before and after the UNOS Allocation System Change

Advanced amyloid cardiomyopathy (ACM) patients have high waitlist (WL) mortality. Given the greater emphasis and clarity for status exceptions for ACM patients in the new allocation system, we sought to assess whether this change in the allocation policy would affect the WL and post-transplant outcomes in ACM pts.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (660) Source Type: research

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Abstract Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused by extracellular deposition of transthyretin, normally involved in the transportation of the hormone thyroxine and retinol-binding protein, in the myocardium. Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of advancement: Imaging techniques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial biopsies; observational studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proportion of patients with heart failure; an...
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
Contributors : Gian L Vita ; M'Hammed Aguennouz ; Francesca Polito ; Rosaria Oteri ; Massimo Russo ; Luca Gentile ; Cristina Barbagallo ; Marco Ragusa ; Carmelo Rodolico ; Rosa M Di Giorgio ; Antonio Toscano ; Giuseppe Vita ; Anna MazzeoSeries Type : Non-coding RNA profiling by arrayOrganism : Homo sapiensTransthyretin variant amyloidosis (ATTRv) is a rare autosomal dominant disease characterized by the accumulation of amyloid in many organs, mostly causing a sensory-motor neuropathy, cardiomyopathy, and dysautonomia. The aim of the study was to report microRNAs (miRNAs) expression profile identified in the blood of ATTRv ...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Non-coding RNA profiling by array Homo sapiens Source Type: research
Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized infiltrative cardiomyopathy in which conduction system disease is common. The aim of our study was to define the incidence and prevalence of high-grade atrioventricular (AV) block requiring pacemaker implantation in our quaternary referral center. This was a single-center retrospective cohort study of 369 consecutive patients with ATTR-CA who underwent 12-lead electrocardiogram (ECG) at the time of ATTR-CA diagnosis. During a mean follow-up of 28 months, serial ECGs and the electronic medical record were examined for the development of high-grade AV ...
Source: The American Journal of Cardiology - Category: Cardiology Authors: Source Type: research
1607Objectives: Quantitative uptake evaluation of 99mTechnetium-pyrophosphate (PYP) has been proved robust for the diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM). However, conventional quantitative methods were based on two-dimensional planar imaging and had some inherent limitations. The aim of this study was to evaluate the feasibility of quantitative PYP SPECT/CT for differentiating ATTR-CM patients from non-ATTR-CM patients. Methods: We retrospectively evaluated all patients who underwent PYP SPECT/CT at our hospital between October 2018 and December 2019. Among them, only 21 patients underwent endomyocard...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Clinical Science (Poster Session) Source Type: research
1366Objectives: CURRENT ROLE OF PET IMAGING IN CARDIAC AMYLOIDOSIS Cardiac amyloidosis (CA) is a relatively rare disease characterized by extracellular deposition of abnormal protein fibrils in the heart, resulting in restrictive cardiomyopathy and heart failure with preserved ejection fraction. Majority of CA occurs due to deposition of two types of amyloid protein: monoclonal immunoglobulin light chains (AL amyloidosis) or transthyretin (ATTR), either wild or mutant type. Differentiating the two most common types of amyloid deposition in the heart is of paramount importance in guiding therapy and prognostication. Diagnos...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Cardiovascular Sciences (Poster Session) Source Type: research
659Objectives: Understanding the characteristics of patients with a final diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CA) may help optimally triage patients for Tc-99m pyrophosphate (PYP) scanning. The study was aimed to investigate the prevalence as well as the clinical features along with the electrocardiographic and echocardiographic characteristics of patients with ATTR-CA, who were referred for PYP scan after being hospitalized for decompensated heart failure. Methods: We included all the patients who were referred for PYP scan after heart failure-related hospitalization at our center between 06/2015 and 0...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Diagnostic and Prognostic Advances in Cardiac Radionuclide and Hybrid Imaging Source Type: research
Abstract Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the import...
Source: Acta Haematologica - Category: Hematology Authors: Tags: Acta Haematol Source Type: research
A new study investigates echocardiographic phenotypes of transthyretin cardiac amyloidosis, the most common etiology of restrictive cardiomyopathy, identifying parameters predictive of survival.European Heart Journal
Source: Medscape Today Headlines - Category: Consumer Health News Tags: Cardiology Journal Article Source Type: news
This study evaluated the diagnostic value of the novel cardiovascular magnetic resonance (CMR) parameter “myocardial transit-time” (MyoTT) in distinguishing cardiac amyloidosis from other hypertrophic cardiomyopathies.MethodsN  = 20 patients with biopsy-proven cardiac amyloidosis (CA),N  = 20 patients with known hypertrophic cardiomyopathy (HCM), andN  = 20 control patients without relevant cardiac disease underwent dedicated CMR studies on a 1.5-T MR scanner. The CMR protocol comprised cine and late-gadolinium-enhancement (LGE) imaging as well as first-pass perfusion a...
Source: Clinical Research in Cardiology - Category: Cardiology Source Type: research
AbstractPurpose of ReviewTransthyretin amyloidosis is an increasingly recognized cause of restrictive cardiomyopathy related to amyloid fibril deposition in cardiac tissues. As treatment therapies have emerged for transthyretin amyloidosis (ATTR), so has interest in using biomarkers to identify disease prior to advanced presentation.Recent FindingsLower levels of transthyretin and retinol binding protein-4 have been demonstrated in patients with pathogenic mutations of transthyretin either with or without clinical disease. Levels associate with the severity of mutations as well as response to treatment with transthyretin s...
Source: Current Heart Failure Reports - Category: Cardiology Source Type: research
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