JACC. Cardiovascular imaging
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JACC Cardiovasc Imaging · Nov 2019
ReviewMyocardial Amyloidosis: The Exemplar Interstitial Disease.
Cardiac involvement drives prognosis and treatment choices in cardiac amyloidosis. Echocardiography is the first-line examination for patients presenting with heart failure, and it is the imaging modality that most often raises the suspicion of cardiac amyloidosis. Echocardiography can provide an assessment of the likelihood of cardiac amyloid infiltration versus other hypertrophic phenocopies and can assess the severity of cardiac involvement. ⋯ CMR structural and functional assessment combined with tissue characterization can redefine cardiac involvement by tracking different disease processes, ranging from amyloid infiltration, to the myocardial response associated with amyloid deposition, through the visualization and quantification of myocardial edema and myocyte response. Bone scintigraphy (paired with exclusion of serum free light chains) is emerging as the technique of choice for distinguishing ATTR from light chain cardiac amyloidosis and other cardiomyopathies; it has transformed the diagnostic pathway for ATTR, allowing noninvasive diagnosis of ATTR without the need for a tissue biopsy in the majority of patients. CMR with tissue characterization and bone scintigraphy are rewriting disease understanding, classification, and definition, and leading to a change in patient care.
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JACC Cardiovasc Imaging · Nov 2019
Multicenter StudyRelative Importance of Baseline and Longitudinal Evaluation in the Follow-Up of Vasodilator Therapy in Pulmonary Arterial Hypertension.
The aim of this study was to evaluate the relative value of baseline and follow-up echocardiographic assessment of pulmonary artery systolic pressure (PASP) and right ventricular (RV) function in assessing response to vasodilator therapy in pulmonary arterial hypertension (PAH). ⋯ In PAH, baseline RV function (RVFWS) is a strong predictor of outcome, independent of PASP. Changes throughout therapy appear minimal, and only changes in RVFWS, inferior vena cava, size, and right atrial area were associated with outcome.