The current status of quantitative SPECT/CT in the assessment of transthyretin cardiac amyloidosis

AbstractNuclear medicine bone scans differentiate ATTR cardiomyopathy (ATTR-CM) from light chain cardiac amyloidosis and other myocardial disorders, helping to make the diagnosis without biopsy. Standard bone scans are not absolutely quantitative, so are assessed by comparing the heart to other tissues. The standard visual scoring system compares heart to bone. This accurately diagnoses ATTR-CM and has been validated in a multicenter study, but has limitations. Semiquantitative techniques including heart/contralateral thorax (H/CL) and heart/whole body ratio (H/WB) improve on visual scoring but still rely on extracardiac sites as comparators. Absolute quantitation of myocardial uptake using quantitative SPECT should help overcome these shortcomings. In ATTR-CM, this technique is practical, accurately makes the diagnosis and provides information that is not identical to visual scores. However, more work needs to be done. The reproducibility in ATTR-CM must be tested. Larger studies need to be undertaken to determine whether quantitative SPECT measurements can assess prognosis, disease progression or treatment response. As ATTR-CM is relatively uncommon multicenter trials will help recruit enough subjects to answer these questions. Accurate measurement techniques are needed in ATTR-CM to enable appropriate use of proven therapy and to conduct trials of new therapeutic agents. Quantitative bone scans offer a promising avenue.
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research

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ont C Abstract Surgery of the cervical spine under conscious sedation has been rarely reported in the literature. The main indications are the lack of neurophysiological monitoring and surgery in patients with high cardiovascular risk. To date, no reports of awake C1-2 instrumentation have been published in the English-language literature. The authors present the case of a 76-year-old patient with multiple myeloma and severe cardiomyopathy associated with primary amyloidosis who experienced severe myelopathy from a C2 pseudotumor associated with an odontoid fracture. Due to his high cardiovascular risk, the patien...
Source: Journal of Neurosurgery.Spine - Category: Neurosurgery Authors: Tags: J Neurosurg Spine Source Type: research
Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an underappreciated cause of heart failure that results from misfolded TTR (prealbumin) protein. Diflunisal is an approved non-steroidal anti-inflammatory drug (NSAID) that stabilizes TTR, with limited data available regarding effects on cardiac structure and function.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Source Type: research
ConclusionsThe present study showed excellent interobserver reproducibility and intraobserver repeatability of18F-florbetapir PET retention index in patients with cardiac AL amyloidosis.
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
Conclusion: Tafamidis is an effective and safe oral medication for the treatment of the cardiomyopathy of transthyretin-mediated amyloidosis in adults to reduce cardiovascular mortality and cardiovascular-related hospitalization. PMID: 31735059 [PubMed - as supplied by publisher]
Source: The Annals of Pharmacotherapy - Category: Drugs & Pharmacology Authors: Tags: Ann Pharmacother Source Type: research
Abstract Transthyretin cardiac amyloidosis is a progressive and life-threating disease that is significantly underdiagnosed, and the actual number of patients with the disease is presently unknown. Accumulation of wild-type transthyretin-derived amyloid in the heart is a common finding in very elderly patients. Recent clinical trials demonstrated that tafamidis reduced all-cause death and the number of cardiovascular hospitalizations when compared with placebo. The Japanese Ministry of Health, Labour and Welfare approved tafamidis (Vyndaqel®, Pfizer Inc.) for the treatment of cardiomyopathy caused by both wild...
Source: Circulation Journal - Category: Cardiology Authors: Tags: Circ J Source Type: research
In this issue of the International Journal of Cardiology, Dr. Maurizi and colleagues report on the higher than expected prevalence of cardiac amyloidosis in patients undergoing evaluation for hypertrophic cardiomyopathy (HCM) in a tertiary referral center [1]. This carefully performed analysis highlights the clinical overlap of cardiac amyloidosis in a substantial percentage (a total of 9%) of patients who display a phenotype of hypertrophic cardiomyopathy and emphasizes the need to consider cardiac amyloidosis in any patient with ventricular hypertrophy without obvious inciting factor or known hypertrophic mutation.
Source: International Journal of Cardiology - Category: Cardiology Authors: Tags: Editorial Source Type: research
Condition:   Transthyretin Amyloidosis (ATTR) With Cardiomyopathy Interventions:   Drug: Vutrisiran;   Drug: Sterile Normal Saline (0.9% NaCl) Sponsor:   Alnylam Pharmaceuticals Not yet recruiting
Source: - Category: Research Source Type: clinical trials
AbstractCardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. The use of iodine-123 labelled metaiodobenzylguanidine ([I-123]MIBG), a chemical modified analogue of norepinephrine, is well established in...
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
35Objectives: The current gold standard for diagnosis of amyloid cardiomyopathy (A-CM) is endomyocardial biopsy (EMB). Bone radiotracer based scintigraphy has re-emerged as an effective non-invasive procedure for diagnosing A-CM, specifically transthyretin amyloid cardiomyopathy (ATTR-CM). The purpose of our study was to evaluate the diagnostic accuracy of a new combined single-photon emission computed tomography and computed tomography (SPECT-CT) (3-D imaging) based quantitative method for diagnosing ATTR-CM and compare it with the current standard planar scintigraphy (2-D imaging) method, the heart-to-contralateral side ...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Cardiovascular Young Investigator Award Symposium Source Type: research
This study was to evaluate the relationship of myocardial(MYO) and extra-myocardial (EXT) active inflammation in patients with CS.Methods Consecutive patients with known or suspect of CS according to Japanese guideline were enrolled. Patients with other cardiac disease (ischemic heart disease, valvular disease, and other cardiomyopathy, etc.) or under corticosteroid therapy were excluded. Patients underwent cardiac and whole body FDG scan with high-fat, low-carbohydrate diet and intravenous pre-administration of heparin (50UI/kg). Maximum of standardized uptake value (SUVmax) was measured for myocardium, and other organs i...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Cardiovascular Clinical Science: Myocardial Amyloidosis and Sarcoidosis Source Type: research
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