Long-Term Effect of Tafamidis on Clinical Parameters and Prognostic Predictors in Patients With Transthyretin Amyloid Cardiomyopathy.

Background: Accurate prediction of prognosis in transthyretin amyloid cardiomyopathy (ATTR-CM) is crucial for optimal treatment selection, including tafamidis, the only approved therapy for ATTR-CM. Although tafamidis has been proven to improve prognosis, the long-term serial changes in comprehensive parameters related to ATTR-CM, including cardiac biomarkers and imaging parameters, under tafamidis remain unknown.

Methods And Results: In this study, we used Cox regression analysis on data from 258 consecutive patients diagnosed with ATTR-CM at Kumamoto University to determine prognostic factors.

CT-derived Extracellular Volume Fraction in Aortic Stenosis, Cardiac Amyloidosis and Dual Pathology.

Aims: To investigate CT-derived extracellular volume fraction (CT-ECV) in patients with lone aortic stenosis (AS), dual pathology of AS and transthyretin cardiac amyloidosis (AS-ATTR), and lone ATTR, and to examine the diagnostic performance and optimal cutoff values of CT-ECV for differentiating between patients with lone AS and AS-ATTR, and between patients with lone AS and lone ATTR.

Methods And Results: This retrospective study included consecutive patients with severe AS (including lone AS and AS-ATTR) and lone ATTR who underwent CT-ECV analysis and technetium 99m pyrophosphate (99mTc-PYP) scintigraphy. The diagnostic performance of CT-ECV for detecting cardiac amyloidosis was evaluated using the area under the receiver operating characteristic curve (AUC).

Early experience with daratumumab-containing regimens in patients with light-chain cardiac amyloidosis.

Background: Immunoglobulin light-chain (AL) amyloidosis is a lethal condition resulting from misfolded immunoglobulin ALs produced by clonal CD38-positive plasma cells. Treatment with daratumumab, an anti-human CD38 monoclonal antibody, led to higher frequencies of complete hematologic response and better clinical outcomes compared with conventional treatment. This study sought to evaluate the survival benefit of daratumumab-containing regimens in patients with AL cardiac amyloidosis.

Cardiac Biomarker Change at 1 Year After Tafamidis Treatment and Clinical Outcomes in Patients With Transthyretin Amyloid Cardiomyopathy.

Background: Although tafamidis treatment improves prognosis in patients with wild-type transthyretin amyloid cardiomyopathy, an optimal surrogate marker monitoring its therapeutic effect remains unclear. This study investigated the association between changes in cardiac biomarkers, high-sensitivity cardiac troponin T (hs-cTnT) and B-type natriuretic peptide (BNP) during the first year after tafamidis treatment and clinical outcomes.

Methods And Results: In 101 patients with wild-type transthyretin amyloid cardiomyopathy receiving tafamidis at our institution, change in cardiac biomarkers from baseline to 1 year after tafamidis administration and its association with composite outcomes (composite of all-cause death and hospitalization attributable to heart failure) was assessed.

Clinical characteristics of patients with high extracellular volume fraction evaluated by cardiac computed tomography for coronary artery evaluation.

Aims: This study aims to evaluate the distribution of extracellular volume fraction detected via computed tomography, clinical characteristics of high extracellular volume fraction detected via computed tomography, and the rate of incidental detection of cardiac amyloidosis in patients undergoing cardiac computed tomography for coronary artery evaluation.

Methods And Results: This study included 874 consecutive patients (mean age, 74.4 ± 7.