florbetapir-f-18 and Immunoglobulin-Light-chain-Amyloidosis

Cardiac amyloidosis is an important clinical entity associated with significant morbidity and mortality. Although the signs and symptoms can be apparent early in the disease course, diagnoses are often made late because of inadequate recognition. A diagnosis of cardiac amyloidosis requires careful scrutiny of a patient's symptoms, an electrocardiogram, and imaging studies, including echocardiography and magnetic resonance imaging. Further evaluation is required through the measurement of serum and urine light chains and the use of bone scintigraphy imaging to differentiate transthyretin amyloidosis from light-chain cardiac amyloidosis. The available treatments have expanded tremendously in recent years and have improved outcomes in the population with this disorder. Thus, it has become increasingly important to diagnose cardiac amyloidosis and provide timely therapies. This article will clarify the various misconceptions about cardiac amyloidosis and provide a framework for primary care providers to better identify this disease in their practice.

Topics: Amyloid Neuropathies, Familial; Amyloidosis; Aniline Compounds; Assisted Circulation; Atrial Fibrillation; Cardiac Imaging Techniques; Cardiomyopathies; Diagnosis, Differential; Echocardiography; Electrocardiography; Ethylene Glycols; Humans; Immunoglobulin Light-chain Amyloidosis; Magnetic Resonance Imaging; Positron-Emission Tomography; Radiopharmaceuticals; Stilbenes

The clinical diagnosis of pulmonary involvement in individuals with systemic AL amyloidosis remains challenging. [. We prospectively enrolled 58 subjects with biopsy-proven AL amyloidosis and 9 control subjects (5 without amyloidosis and 4 with ATTR cardiac amyloidosis). Pulmonary [. On visual analysis, pulmonary tracer uptake was absent in most AL subjects (40/58, 69%); 12% (7/58) of AL subjects demonstrated intense bilateral homogeneous tracer uptake. In this group, compared to the control group, Vs (median Vs 30-fold higher, 9.79 vs. 0.26, p < 0.001), TBR (median TBR 12.0 vs. 1.71, p < 0.001), and RI (median RI 0.310 vs. 0.033, p < 0.001) were substantially higher. Notably, the AL group without visually apparent pulmonary [

Topics: Aniline Compounds; Ethylene Glycols; Humans; Immunoglobulin Light-chain Amyloidosis; Lung; Positron Emission Tomography Computed Tomography

Cardiac involvement in systemic light chain amyloidosis (AL) is the main determinant of prognosis and, therefore, guides management. The hypothesis of this study was that myocardial AL deposits and expansion of extracellular volume (ECV) could be identified before increases in N-terminal pro-B-type natriuretic peptide or wall thickness.. The active-CA group demonstrated the largest myocardial AL amyloid burden, quantified by [. Evidence of cardiac amyloid infiltration was found based on direct and indirect imaging biomarkers in subjects without CA by conventional criteria. The findings from [

Topics: Aged; Aniline Compounds; Cardiomyopathies; Early Diagnosis; Echocardiography, Doppler; Ethylene Glycols; Female; Fluorine Radioisotopes; Humans; Immunoglobulin Light-chain Amyloidosis; Magnetic Resonance Imaging, Cine; Male; Middle Aged; Multimodal Imaging; Myocardium; Positron Emission Tomography Computed Tomography; Predictive Value of Tests; Prospective Studies; Radiopharmaceuticals; Severity of Illness Index; United States

Topics: Aged; Aniline Compounds; Ethylene Glycols; Female; Follow-Up Studies; Heart Diseases; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Pilot Projects; Positron-Emission Tomography; Prognosis; Prospective Studies

We present a case of both F-FDG and F-florbetapir uptake in a biopsy-confirmed immunoglobulin light chain (AL) amyloidosis involving the peripheral nerves. AL amyloidosis is the most common cause of acquired amyloid polyneuropathy, manifesting with both sensorimotor and autonomic neuronal dysfunction. Given the overlapping MRI and FDG PET/CT appearances of several different causes of peripheral neuropathy, F-florbetapir PET/CT provides another potential tool in the imaging algorithm of these patients and may guide targeted fascicular biopsy for pathologic confirmation.

Topics: Amyloid Neuropathies; Amyloidosis; Aniline Compounds; Ethylene Glycols; Fluorodeoxyglucose F18; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Multimodal Imaging; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed