Background: Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure in older individuals. We sought to characterize the natural history of ATTR-CM and compare outcomes and quality of life among patients with acquired and hereditary forms of the disease. Methods: We studied 711 patients with wild-type ATTR-CM, 205 with hereditary ATTR-CM associated with the V122I variant (V122I-hATTR-CM), and 118 with non-V122I-hATTR-CM at the UK National Amyloidosis Center between 2000 and 2017. Patients underwent prospective protocolized evaluations comprising assessment of cardiac parameters, functional status by 6-minute walk test, quality of life according to the Kansas City Cardiomyopathy Questionnaire, and survival. Hospital service usage pre- and postdiagnosis was established using English central health records in a subset of patients. Results: There was substantial diagnostic delay, with patients using hospital services a median (interquartile range) of 17 (9–27) times during the 3 years before diagnosis, by which time quality of life was poor; diagnosis of wild-type ATTR-CM was delayed >4 years after presentation with cardiac symptoms in 42% of cases. Patients with V122I-hATTR-CM were more impaired functionally ( P <0.001) and had worse measures of cardiac disease ( P <0.001) at the time of diagnosis, a greater decline in quality of life, and poorer survival ( P <0.001) in comparison with the other subgroups. Conclusions: ATTR-CM is an inexorably progressive and eventually fatal cardiomyopathy associated with poor quality of life. Diagnosis is often delayed for many years after symptoms develop. Improved awareness and wider use of recently validated diagnostic imaging methods are urgently required for patients to benefit from recent therapeutic developments.
This work provides an insight into positron emission tomography (PET) joint image reconstruction/motion estimation (JRM) by maximization of the likelihood, where the probabilistic model accounts for warped attenuation. Our analysis shows that maximum-likelihood (ML) JRM returns the same reconstructed gates for any attenuation map (μ-map) that is a deformation of a given μ-map, regardless of its alignment with the PET gates. We derived a joint optimization algorithm accordingly, and applied it to simulated and patient gated PET data. We first evaluated the proposed algorithm on simulations of respiratory gated PET/CT data based on the XCAT phantom. Our results show that independently of which μ-map is used as input to JRM: (i) the warped μ-maps correspond to the gated μ-maps, (ii) JRM outperforms the traditional post-registration reconstruction and consolidation (PRRC) for hot lesion quantification and (iii) reconstructed gated PET images are similar to those obtained with gated μ-maps. This suggests that a breath-held μ-map can be used. We then applied JRM on patient data with a μ-map derived from a breath-held high resolution CT (HRCT), and compared the results with PRRC, where each reconstructed PET image was obtained with a corresponding cine-CT gated μ-map. Results show that JRM with breath-held HRCT achieves similar reconstruction to that using PRRC with cine-CT. This suggests a practical low-dose solution for implementation of motion-corrected respiratory gated PET/CT.
Positron range is one of the main physical effects limiting the spatial resolution true activity mean value of the hot regions. Moreover, in the case where a magnetic field is present, the correction accounts for the non-isotropy of the positron range effect, resulting in the recovery of resolution along the axial plane.
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