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Although transthyretin cardiac amyloidosis remains a cardiac disease, these extracardiac localizations can raise diagnostic interest and allow for early diagnosis, especially in the case of promising new therapeutic approaches that can slow or stop the disease progression. The presence of an old patient with or without clinical signs of cardiovascular disease, a complex sequence of musculoskeletal disorders, and unexpected and inexplicable series of musculoskeletal signs, can be a turning point for a patient's diagnosis and treatment.
Source link: https://doi.org/10.3390/biomedicines10123226
However, new studies indicate a significant presence in patients with degenerative aortic stenosis and heart failure with preserved ejection fractions. Case Description: An 85-year-old woman with a four-month history of suffering in the rib cage with a history of diffuse large B-cell lymphoma of the oral cavity, essential thrombocytosis, and dyslipidemia. With a preserving left ventricular ejection percentage of 70%, a transthoracic echocardiogram revealed degenerative aortic stenosis and normal systolic function. According to the Perugini scale, Bone-avid tracer cardiac scintigraphy with technetium-99m-labeled hydroxymethylene diphosphonate showed uptake in grade two myocardial blood test. The absence of mutations in the transthyretin gene, serum, and urine immunofixation electrophoresis negative for monoclonal protein and serum-free light chain assay with a normal kappa/lambda ratio was shown by laboratory experiments.
Source link: https://doi.org/10.2174/1573405618666220610091446
Methods of Measurement 75 patients with ATTR-CA underwent cardiac testing and CPET in a National Referral Center for cardiac amyloidosis as part of this research. ATR of the wild type ATTR was found in fifty-seven patients. In 19 patients, after a median follow-up of 25 months, the composite outcome of death or heart failure hospitalization was identified. %ppVO2 was a better predictor of the composite result than peak VO2 at univariate analysis than peak VO2. Conclusions CPET is a safe and useful diagnostic device in patients with ATTR-CA. CPET can help to identify patients with advanced disease with a chemotherapy approach.
Source link: https://doi.org/10.1093/eurheartjsupp/suac121.420
Abstract Aims The prognostic role of bone tracer uptake in transthyretin cardiac amyloidosis is disputed. Methods Consecutive ATTR-CA patients who under cardiac scintigraphy were treated with planar and single-photon emission computed tomography images from the National Amyloidosis Centre and four Italian centers were included. Result Among 1422 patients with ATTR-CA, Biv uptake was discovered in 85% of cases on planar scintigraphy, and in 100% of cases on SPECT images. During a median follow-up of 39 months, BiV uptake at planar scintigraphy was seen with a higher all-cause mortality than isolated LV uptake, whereas the Perugini scale was not compared. The addition of planar BiV uptake to the NAC stage resulted in increased precision of the model for prediction of all-cause death due to a time-dependent ROC curve review. Conclusions Planar RV uptake leading to BiV uptake found ATTR-CA patients with a poor outcome, possibly as a new prognostic marker.
Source link: https://doi.org/10.1093/eurheartjsupp/suac121.667
Abstract Purpose We investigated the prevalence and ECG causes of de novo atrial fibrillation in patients with wild-type transthyretin cardiac amyloidosis to drive customized arrhythmia screening. Background Information is limited on the incidence and causes of de novo AF in patients with ATTRwt CA patients. Patients with AF: patients with a nave AF u2019; patients in a U2018sinus rhythm u2019; and patients with no known AFu2019 during FU were admitted into the study, divided into three groups based on the presence of AF: patients with AF: patients with AF: patients with a u2018t-CA diagnosis between 2004 and 2020; patients with > 1-year follow-up were recruited in the trial; patients with AFu2019 AF: patients with AF; patients in AF/u2019 AFu2018sinus rhythm u2019 AFu2019 novo AFu2019 in AF: patients with AFu2019 AF: patients with AF; patients with AF: patients with AF: patients with AFu2018sinus rhythm AFu2018sinus rhythm AFu2019 AF; patients with >1-UA2018, AFu2019 AF; patients with AF; patients with AFu2019sinus The key findings were Incidence and factors associated with AF in patients with ATTRwt. Patients with at least two risk factors were associated with the onset of de novo AF in comparison to patients with no risk factors. In patients with ATTRwt vs. 20. 7%/year, there is an incidence of de novo AF.
Source link: https://doi.org/10.1093/eurheartjsupp/suac121.618
Abstract Background and Aspirations The misfolding, aggregation, and tissue deposition of native TTR, leading to cardiac structural remodeling, contributes to cardiac remodeling. We aimed to determine circulating TTR levels in plasma samples of ATTRwt-CA patients before and after treatment with tafamidis by a native electrophoretic technique. Methods Plasma samples from six male patients with ATTRwt amyloidosis, collected before and during tafamidis therapy, were collected before and during tafamidis therapy, and six healthy controls were obtained. Following a steadyizing action on TTR-RBP complexes, all ATTRwt-CA patients showed a gradual rise in the intensity of the band corresponding to TTR-RBP complexes following tafamidis therapy. The evaluation of TTR isoform in patients with ATTRwt-CA may be a valuable biomarker for determining responses to therapy.
Source link: https://doi.org/10.1093/eurheartjsupp/suac121.596
In the cardiac subpopulation, 18 months of vutrisiran therapy significantly raised NT-ProBNP levels vs. the external placebo, as well as a trend toward improvement in echocardiographic parameters relative to the external placebo. Of the 122 vutrisiran-treated patients, the 99mTc scintigraphy report was obtained for 64 vutrisiran-treated patients at baseline, 35 of whom had a Perugini grade u22652 cardiac uptake of 99mTc. In 64. 6% and 68 percent respectively at 18 months, respectively, among patients with evaluable scintigraphy parameters repeated at 18 months, heart-to-contralateral lung ratio, and normalised LV total uptake on scintigraphy increased in 66% and 68. 1%, respectively. 76. 9% and 100% respectively for evaluable patients with baseline Perugini grade u22652, the percentage with rise in heart-to-contralateral lung ratio and normalized LV total uptake was 76. 9% and 100% respectively. Vutrisiran therapy decreased heart uptake of 99mTc, possibly reducing cardiac amyloid reductions, although the clinical relevance of this is not yet clear.
Source link: https://doi.org/10.1093/eurheartjsupp/suac121.654
- Background Hereditary Transthyretin Amyloidosis is a rare, genetically diverse, and phenotypically heterogeneous disease characterized by the deposition of misfolded transthyretin fibrils in various organs and tissues. As disease-modifying treatment options improve, timely diagnosis and treatment are crucial to preventing rapid disease progression. Case presentation Here's a case study from a 73-year-old patient who was first diagnosed with cardiac wild-type ATTR amyloidosis by endomyocardial biopsy. Molecular genetic analysis revealed a novel TTR sequence variant that is highly likely to be amyloidogenic in light of previously reported TTR mutations and the patient's clinical presentation and family history.
Source link: https://doi.org/10.1186/s12883-022-02952-3
According to NI301A, selectively with high affinity to the disease-associated ATTR aggregates of either wild-type or variant ATTR related to sporadic or hereditary disease. The NI301A removes ATTR deposits ex vivo from patient-derived myocardium by macrophages, as well as in vivo from mice transplanted with patient-derived ATTR fibrils in a dose- and time-dependent manner. ATTR removal causes antibody-mediated activation of phagocytic immune cells, including macrophages, in an animal's biological function. These findings support the investigation of NI301A's safety and tolerability in an ongoing phase 1 clinical trial in patients with ATTR cardiomyopathy.
Source link: https://doi.org/10.1038/s41467-021-23274-x
In 60 and 21 patients, respectively, were diagnosed with ATTR and AL, and concomitant AL and ATTR were present in one patient. The specificity and positive predictive value of Perugini score u2265 2 for ATTR CA were 95% and 96%, respectively. For ATTR CA, the NPV of Perugini was 79%, with a % score of zero detectable monoclonal proteins from reports excluding all patients with detectable monoclonal proteins from reports. Conclusively, ATTR CA can be diagnosed non-invasively in the case of a Perugini score u22652 2 and unremarkable FLC report.
Source link: https://doi.org/10.3390/biomedicines10123052
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