Differential Myocyte Responses in Patients with Cardiac Transthyretin Amyloidosis and Light-Chain Amyloidosis: A Cardiac MR Imaging Study

Autor: Janet A. Gilbertson, Marianna Fontana, Ashutosh D. Wechalekar, Julian D. Gillmore, Carol J. Whelan, Amna Abdel-Gadir, Sanjay M Banypersad, Thomas A. Treibel, David F. Hutt, Anna S Herrey, Viviana Maestrini, Helen J. Lachmann, Philip N. Hawkins, James C. Moon, Thirusha Lane
Rok vydání: 2015
Předmět:
Adult
Male
medicine.medical_specialty
Pathology
Cell
Contrast Media
Muscle hypertrophy
Meglumine
Fibrosis
Internal medicine
Organometallic Compounds
medicine
Extracellular
Humans
Myocyte
Radiology
Nuclear Medicine and imaging
Prospective Studies
Aged
Aged
80 and over
Amyloid Neuropathies
Familial
Muscle Cells
adult
aged
aged
80 and over
amyloid neuropathies familial
amyloidosis
biomarkers
cardiomyopathies
case-control studies
contrast media
echocardiography
female
humans
magnetic resonance imaging
male
meglumine
middle aged
muscle cells
organometalli compounds
prospective studies
radiology
nuclear medicine and imaging
biology
business.industry
Amyloidosis
nutritional and metabolic diseases
Middle Aged
medicine.disease
Magnetic Resonance Imaging
Transthyretin
Amyloid Neuropathy
medicine.anatomical_structure
Echocardiography
Case-Control Studies
biology.protein
Cardiology
Female
Cardiomyopathies
business
Biomarkers
Zdroj: Radiology. 277:388-397
ISSN: 1527-1315
0033-8419
Popis: To investigate cardiac magnetic resonance (MR) imaging measurements of extracellular volume (ECV) and total cell volume in immunoglobulin light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) in order to evaluate the amyloid and myocyte volumes.All ethics were approved, and participants provided written informed consent. Of the 257 subjects who were recruited, 92 had AL (mean age, 62 years ± 10), 44 had mutant ATTR (mean age, 68 years ± 10), and 66 had wild-type ATTR (mean age, 75 years ± 7). In addition, eight healthy subjects with ATTR mutations (mean age, 47 years ± 6) and 47 healthy volunteers (mean age, 45 years ± 15) participated. All participants underwent equilibrium contrast material-enhanced cardiac MR imaging. ECV and total cell volume were measured in the heart. T test, χ(2), and one-way analysis of variance with posthoc Bonferroni correction were used.Both the left ventricular indexed mass and ECV were elevated in patients with amyloidosis. For left ventricular indexed mass, mean AL was 107 g/m(2) ± 30; mean mutant ATTR was 137 g/m(2) ± 29; and mean wild-type ATTR was 133 g/m(2) ± 27 versus 65 g/m(2) ± 15 in healthy subjects (P.0001 for all measures). For ECV, mean AL was 0.54 ± 0.07, mean mutant ATTR was 0.60 ± 0.07, and mean wild-type ATTR was 0.57 ± 0.06 versus 0.27 ± 0.03 in healthy subjects (P.0001 for all measures). Patients with ATTR had a higher total cell volume than did healthy subjects (mean, 53 mL/m(2) ± 12 vs 45 mL/m(2) ± 11; P = .001), but in patients with AL, total cell volume was normal (mean, 47 mL/m(2) ± 17 vs 45 mL/m(2) ± 11; P.99). The result is that, in patients with AL, all of the increase in left ventricular indexed mass is extracellular volume, whereas in patients with ATTR, the increase is extracellular, with an additional 18% increase in the intracellular space.Quantification of ECV measures cardiac amyloid deposition in both types of amyloidosis and shows that amyloid deposition is more extensive in patients with ATTR than in those with AL; however, ATTR is associated with higher cell volume, which suggests concomitant cell hypertrophy.
Databáze: OpenAIRE
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