Imaging Options in Cardiac Amyloidosis: Differentiating AL from ATTR

AbstractPurpose of ReviewCardiac amyloidosis (CA) results from the myocardial deposition of abnormally folded endogenous proteins leading to significant morbidity and mortality. Accurate and timely non-invasive diagnosis of cardiac amyloidosis and the correct identification of amyloid type are critical for choosing appropriate therapy. Disease type is defined by the precursor protein and most typically involves light chain (AL) or transthyretin (ATTR). Traditionally, cardiac amyloidosis has been suspected on the basis of characteristic echocardiographic features and confirmed with endomyocardial biopsy. Recent advances in echocardiography, cardiovascular magnetic resonance (CMR), and nuclear imaging now permit the non-invasive diagnosis of CA with a high degree of confidence and, in some cases, assignment of amyloid types.Recent FindingsCMR techniques including late gadolinium enhancement and new protocols such as T1 mapping and extra-cellular volume (ECV) quantification have afforded insight into the diagnosis and pathophysiology of cardiac amyloidosis, while bone-avid nuclear tracers can identify ATTR with a high degree of accuracy.SummaryWe highlight the recent advances in imaging techniques that can identify cardiac amyloidosis with a special emphasis on nuclear modalities used for differentiating light chain from transthyretin disease.
Source: Current Cardiovascular Imaging Reports - Category: Radiology Source Type: research

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ConclusionQuantitative99mTc-DPD SPECT/CT in suspected ATTR CA patients is feasible and allows for a more accurate assessment of myocardial uptake.
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
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Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
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Source: Amyloid: The Journal of Protein Folding Disorders - Category: Biochemistry Authors: Source Type: research
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Source: Amyloid: The Journal of Protein Folding Disorders - Category: Biochemistry Authors: Source Type: research
We report a 58-year-old Japanese woman who presented with nephrotic syndrome. Steroid therapy and cyclosporine A administration were initiated, but hematological remission and renal response were not achieved. Renal biopsy revealed amyloid deposits in the mesangial region and the small arteries. Proteomic analysis based on laser microdissection and mass spectrometry showed that the amyloid deposits were composed of the constant region of the lambda light chain. She received vincristine, adriamycin, and dexamethasone therapy followed by high-dose melphalan and autologous stem cell transplantation, resulting in hematological...
Source: CEN Case Reports - Category: Urology & Nephrology Source Type: research
Publication date: Available online 12 September 2019Source: Journal de Mycologie MédicaleAuthor(s): Dharmesh Patel, Ritesh Agarwal, Sahajal Dhooria, Umapati Hedge, Hardik Patel, Inderpaul Singh SehgalAbstractSecondary amyloidosis results from the deposition of abnormally folded proteins in body organs due to chronic inflammatory disorders. Kidneys are the most commonly affected organ and manifest as nephrotic syndrome with or without renal failure. Chronic pulmonary aspergillosis (CPA) is a chronic infection of lung parenchyma affecting those with an underlying structural lung disease. Herein, we present a case of C...
Source: Journal of Medical Mycology - Category: Biology Source Type: research
AbstractPurpose of ReviewThe review ’s main focus centers on the genetics of hereditary cardiac amyloidosis, highlighting the opportunities and challenges posed by the widespread availability of genetic screening and diagnostic cardiac imaging.Recent FindingsAdvancements in cardiac imaging, heightened awareness of the ATTR amyloidosis diagnosis, and greater access to genetic testing have all led to an increased appreciation of the prevalence of ATTR cardiac amyloidosis. Elucidation of the TTR molecular structure and effect of mutations on TTR function have allowed for novel TTR therapy development leading to clinical...
Source: Current Heart Failure Reports - Category: Cardiology Source Type: research
ConclusionOur findings reveal that ATTR p.A97S is a cardiomyopathy as well as a polyneuropathic syndrome. Routine use of more contemporary echocardiographic techniques are recommended to identify cardiac amyloidosis and provide prognostic information.
Source: Journal of the Formosan Medical Association - Category: General Medicine Source Type: research
AbstractBackgroundThe aim of our study was to investigate outcomes of patients with ATTR CA and implantable devices with respect to left ventricular ejection fraction (LVEF), mitral regurgitation (MR), New York Heart Association (NYHA) functional class and mortality.MethodsThis was a retrospective observational cohort study of 78 patients with ATTR CA and implantable devices. During a mean follow ‐up of 42 months we investigated the impact of RV pacing burden and BiV pacing on LVEF, MR severity, NYHA functional class and mortality.ResultsWorsening MR occurred in 11% of patients with a RV pacing % 40% (p=0.002). Similarly...
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: ORIGINAL ‐ CLINICAL Source Type: research
CONCLUSION: Our findings reveal that ATTR p.A97S is a cardiomyopathy as well as a polyneuropathic syndrome. Routine use of more contemporary echocardiographic techniques are recommended to identify cardiac amyloidosis and provide prognostic information. PMID: 31521469 [PubMed - as supplied by publisher]
Source: J Formos Med Assoc - Category: General Medicine Authors: Tags: J Formos Med Assoc Source Type: research
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