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Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure. A total of 3-4% of individuals of African descent carry a TTR gene mutation encoding the p.(V142I) variant, a powerful risk factor for development of variant ATTR-CM (ATTRv-CM); this equates to 1.6 million carriers in the United States. We undertook deep phenotyping of p.(V142I)-ATTRv-CM and comparison with wild-type ATTR-CM (ATTRwt-CM)., Methods and Results: A retrospective study of 413 patients with p.(V142I) ATTRv-CM who attended the UK National Amyloidosis Centre (NAC) was conducted. Patients underwent evaluation at time of diagnosis, including clinical, echocardiography, and biomarker analysis; a subgroup had cardiac magnetic resonance (CMR) imaging. A total of 413 patients with ATTRwt-CM, matched for independent predictors of prognosis (age, NAC Stage, decade of first presentation), were used as a comparator group. At time of diagnosis, patients with ATTRv-CM had significant functional impairment by New York Heart Association classification (NHYA class ≥ III; 38%) and 6-min walk test distance (median 276 m). Median 5-year survival in ATTRv-CM patients was 31 versus 59 months in matched patients with ATTRwt-CM (p < 0.001). Patients with ATTRv-CM had significant impairment of functional parameters by echocardiography including biventricular impairment, high burden of regurgitant valvular disease and low cardiac output. Multivariable analysis revealed the prognostic importance of right ventricular dysfunction. CMR and histological analysis revealed myocyte atrophy and widespread myocardial infiltration in ATTRv-CM., Conclusion: p.(V142I)-ATTRv-CM has an aggressive phenotype characterized by myocyte loss and widespread myocardial infiltration which may account for frequent biventricular failure and poor prognosis in this ATTR-CM genotypic subgroup., (© 2023 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Purpose: To develop a real-time phase contrast MR sequence with high enough temporal resolution to assess cardiac time intervals., Methods: The sequence utilized spiral trajectories with an acquisition strategy that allowed a combination of temporal encoding (Unaliasing by fourier-encoding the overlaps using the temporal dimension; UNFOLD) and parallel imaging (Sensitivity encoding; SENSE) to be used (UNFOLDed-SENSE). An in silico experiment was performed to determine the optimum UNFOLD filter. In vitro experiments were carried out to validate the accuracy of time intervals calculation and peak mean velocity quantification. In addition, 15 healthy volunteers were imaged with the new sequence, and cardiac time intervals were compared to reference standard Doppler echocardiography measures. For comparison, in silico, in vitro, and in vivo experiments were also carried out using sliding window reconstructions., Results: The in vitro experiments demonstrated good agreement between real-time spiral UNFOLDed-SENSE phase contrast MR and the reference standard measurements of velocity and time intervals. The protocol was successfully performed in all volunteers. Subsequent measurement of time intervals produced values in keeping with literature values and good agreement with the gold standard echocardiography. Importantly, the proposed UNFOLDed-SENSE sequence outperformed the sliding window reconstructions., Conclusion: Cardiac time intervals can be successfully assessed with UNFOLDed-SENSE real-time spiral phase contrast. Real-time MR assessment of cardiac time intervals may be beneficial in assessment of patients with cardiac conditions such as diastolic dysfunction., (© 2014 Wiley Periodicals, Inc.)

Purpose: To create a high temporal- and spatial-resolution retrospectively cardiac-gated, tissue phase mapping (TPM) sequence, using an image-based respiratory navigator calculated from the data itself., Methods: The sequence was based on a golden-angle spiral acquisition. Reconstruction of real-time images allowed creation of an image-based navigator. The expiratory spiral interleaves were then retrospectively cardiac-gated using data binning. TPM data were acquired in 20 healthy volunteers and 10 patients with pulmonary hypertension. Longitudinal and radial myocardial velocities were calculated in the left ventricle and right ventricle., Results: The image-based navigator was shown to correlate well with simultaneously acquired airflow data in 10 volunteers(r=0.93±0.04). The TPM navigated images had a significantly higher subjective image quality and edge sharpness (P<0.0001) than averaged spiral TPM. No significant differences in myocardial velocities were seen between conventional Cartesian TPM with navigator respiratory-gating and the proposed self-navigated TPM technique, in 10 volunteers. Significant differences in the velocities were seen between the volunteers and patients in the left ventricle at systole and end diastole and in the right ventricle at end diastole., Conclusion: The feasibility of measuring myocardial motion using a golden-angle spiral TPM sequence was demonstrated, with an image-based respiratory navigator calculated from the TPM data itself., (Copyright © 2013 Wiley Periodicals, Inc.)