Dataset Information

Cardiac Amyloidosis: Multimodal Imaging of Disease Activity and Response to Treatment.

ABSTRACT: Cardiac amyloidosis (CA) is a disease characterized by the deposition of misfolded protein deposits in the myocardial interstitium. Although advanced CA confers significant morbidity and mortality, the magnitude of deposition and ensuing clinical manifestations vary greatly. Thus, an improved understanding of disease pathogenesis at both cellular and functional levels would afford critical insights that may improve outcomes. This review will summarize contemporary therapies for the 2 major types of CA, transthyretin and light chain amyloidosis, and outline the capacity of imaging modalities to both diagnose CA, inform prognosis, and follow response to available therapies. We explore the current landscape of echocardiography, cardiac magnetic resonance, and bone scintigraphy in the assessment of functional and cellular parameters of dysfunction in CA throughout disease pathogenesis. Finally, we examine the impact of concurrent advances in both therapeutics and imaging on future research questions that improve our understanding of underlying disease mechanisms. Multimodal imaging in CA affords an indispensable tool to offer individualized treatment plans and improve outcomes in patients with CA.

SUBMITTER: Patel RK

PROVIDER: S-EPMC8210461 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature

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Cardiac Amyloidosis: Multimodal Imaging of Disease Activity and Response to Treatment.

Patel Rishi K RK Fontana Marianna M Ruberg Frederick L FL

Circulation. Cardiovascular imaging 20210615 6

Cardiac amyloidosis (CA) is a disease characterized by the deposition of misfolded protein deposits in the myocardial interstitium. Although advanced CA confers significant morbidity and mortality, the magnitude of deposition and ensuing clinical manifestations vary greatly. Thus, an improved understanding of disease pathogenesis at both cellular and functional levels would afford critical insights that may improve outcomes. This review will summarize contemporary therapies for the 2 major types ...[more]

PMID: 34129344

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Project description:ImportanceCardiac magnetic resonance (CMR) imaging-derived extracellular volume (ECV) mapping, generated from precontrast and postcontrast T1, accurately determines treatment response in cardiac light-chain amyloidosis. Native T1 mapping, which can be derived without the need for contrast, has demonstrated accuracy in diagnosis and prognostication, but it is unclear whether serial native T1 measurements could also track the cardiac treatment response.ObjectiveTo assess whether native T1 mapping can measure the cardiac treatment response and the association between changes in native T1 and prognosis.Design, setting, and participantsThis single-center cohort study evaluated patients diagnosed with cardiac light-chain amyloidosis (January 2016 to December 2020) who underwent CMR scans at diagnosis and a repeat scan following chemotherapy. Analysis took place between January 2016 and October 2022.Main outcomes and measuresComparison of biomarkers and cardiac imaging parameters between patients with a reduced, stable, or increased native T1 and association between changes in native T1 and mortality.ResultsThe study comprised 221 patients (mean [SD] age, 64.7 [10.6] years; 130 male [59%]). At 6 months, 183 patients (mean [SD] age, 64.8 [10.5] years; 110 male [60%]) underwent repeat CMR imaging. Reduced native T1 of 50 milliseconds or more occurred in 8 patients (4%), all of whom had a good hematological response; by contrast, an increased native T1 of 50 milliseconds or more occurred in 42 patients (23%), most of whom had a poor hematological response (27 [68%]). At 12 months, 160 patients (mean [SD] age, 63.8 [11.1] years; 94 male [59%]) had a repeat CMR scan. A reduced native T1 occurred in 24 patients (15%), all of whom had a good hematological response, and was associated with a reduction in N-terminal pro-brain natriuretic peptide (median [IQR], 2638 [913-5767] vs 423 [128-1777] ng/L; P < .001), maximal wall thickness (mean [SD], 14.8 [3.6] vs 13.6 [3.9] mm; P = .009), and E/e' (mean [SD], 14.9 [6.8] vs 12.0 [4.0]; P = .007), improved longitudinal strain (mean [SD], -14.8% [4.0%] vs -16.7% [4.0%]; P = .004), and reduction in both myocardial T2 (mean [SD], 52.3 [2.9] vs 49.4 [2.0] milliseconds; P < .001) and ECV (mean [SD], 0.47 [0.07] vs 0.42 [0.08]; P < .001). At 12 months, an increased native T1 occurred in 24 patients (15%), most of whom had a poor hematological response (17 [71%]), and was associated with an increased N-terminal pro-brain natriuretic peptide (median [IQR], 1622 [554-5487] vs 3150 [1161-8745] ng/L; P = .007), reduced left ventricular ejection fraction (mean [SD], 65.8% [11.4%] vs 61.5% [12.4%]; P = .009), and an increase in both myocardial T2 (mean [SD], 52.5 [2.7] vs 55.3 [4.2] milliseconds; P < .001) and ECV (mean [SD], 0.48 [0.09] vs 0.56 [0.09]; P < .001). Change in myocardial native T1 at 6 months was independently associated with mortality (hazard ratio, 2.41 [95% CI, 1.36-4.27]; P = .003).Conclusions and relevanceChanges in native T1 in response to treatment, reflecting a composite of changes in T2 and ECV, are associated with in changes in traditional markers of cardiac response and associated with mortality. However, as a single-center study, these results require external validation in a larger cohort.

Project description:ObjectivesTo assess whether single-photon emission computed tomography (SPECT/CT) quantification of bone scintigraphy would improve diagnostic accuracy and offer a means of quantifying amyloid burden.BackgroundTransthyretin-related cardiac amyloidosis is common and can be diagnosed noninvasively using bone scintigraphy; interpretation, however, relies on planar images. SPECT/CT imaging offers 3-dimensional visualization.MethodsThis was a single-center, retrospective analysis of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scans reported using the Perugini grading system (0 = negative; 1 to 3 = increasingly positive). Conventional planar quantification techniques (heart/contralateral lung, and heart/whole-body retention ratios) were performed. Heart, adjacent vertebra, paraspinal muscle and liver peak standardized uptake values (SUVpeak) were recorded from SPECT/CT acquisitions. An SUV retention index was also calculated: (cardiac SUVpeak/vertebral SUVpeak) × paraspinal muscle SUVpeak. In a subgroup of patients, SPECT/CT quantification was compared with myocardial extracellular volume quantification by CT imaging (ECVCT).ResultsA total of 100 DPD scans were analyzed (patient age 84 ± 9 years; 52% male): 40 were Perugini grade 0, 12 were grade 1, 41 were grade 2, and 7 were grade 3. Cardiac SUVpeak increased from grade 0 to grade 2; however, it plateaued between grades 2 and 3 (p < 0.001). Paraspinal muscle SUVpeak increased with grade (p < 0.001), whereas vertebral SUVpeak decreased (p < 0.001). The composite parameter of SUV retention index overcame the plateauing of the cardiac SUVpeak and increased across all grades (p < 0.001). Cardiac SUVpeak correlated well (r2 = 0.73; p < 0.001) with ECVCT. Both the cardiac SUVpeak and SUV retention index had excellent diagnostic accuracy (area under the curve [AUC]: 0.999). The heart to contralateral lung ratio performed the best of the planar quantification techniques (AUC: 0.987).ConclusionsSPECT/CT quantification in DPD scintigraphy is possible and outperforms planar quantification techniques. Differentiation of Perugini grade 2 or 3 is confounded by soft tissue uptake, which can be overcome by a composite SUV retention index. This index can help in the diagnosis of cardiac amyloidosis and may offer a means of monitoring response to therapy.

Dataset Information

Cardiac Amyloidosis: Multimodal Imaging of Disease Activity and Response to Treatment.

Publications

Cardiac Amyloidosis: Multimodal Imaging of Disease Activity and Response to Treatment.

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