Molecular Imaging of Myocardial Fibrosis in Cardiac Amyloidosis

Inclusion Criteria for AL-amyloid subjects: Age > 18 years Willing and able to provide consent AL-CA: Diagnosis of systemic light chain amyloidosis by standard criteria: Immunofixation of serum, serum free light chain (FLC) assay, a biopsy of fat pad/bone marrow, or organ biopsy, followed by typing of the light chain using immunohistochemistry or immunogold assay with confirmation by mass spectroscopy as needed AND Proof of cardiac involvement by AL amyloidosis Abnormal cardiac biomarkers: abnormal high sensitivity TnT 5th generation levels (> 15 ng/L) or abnormal age-appropriate NT-proBNP (abnormal values: < 50 years: > 450 pg/ml; 50-75 years: > 900 pg/ml; > 75 years: > 1800 pg/ml) OR Abnormal echocardiogram (wall thickness > 12 mm in the absence of other causes of increased LV wall thickness) OR Abnormal CMR (wall thickness > 12 mm, extracellular volume > 0.40 or typical CMR appearance of cardiac amyloidosis with difficulty nulling images and non-coronary distribution late gadolinium enhancement) OR Positive endomyocardial biopsy Inclusion Criteria for ATTR-amyloid subjects: Age > 18 years Willing and able to provide consent ATTR-CA: Diagnosis of either wildtype or hereditary transthyretin cardiac amyloidosis by standard criteria: Endomyocardial biopsy followed by typing of the transthyretin amyloidosis using immunohistochemistry or immunogold assay with confirmation by mass spectroscopy as needed Extracardiac biopsy with typical cardiac imaging findings Hereditary ATTR amyloidosis by genetic testing OR Grade 2 or grade 3 myocardial uptake of 99mTc-PYP if AL amyloidosis is excluded Inclusion Criteria for recent myocardial infarction subjects: Age > 18 years Willing and able to provide consent Recent MI: Diagnosis of recent type 1 myocardial infarction by standard criteria More than 6 weeks from diagnosis of MI but within 6 months Imaging evidence of loss of viable myocardium or persistent regional wall motion abnormalities in a pattern consistent with an ischemic etiology in more than one segment Inclusion Criteria for hypertrophic cardiomyopathy subjects: Age > 18 years Willing and able to provide consent Hypertrophic cardiomyopathy: Diagnosis of hypertrophic cardiomyopathy by standard criteria MRI evidence of late gadolinium enhancement Inclusion Criteria for recent healthy control subjects: Age > 18 years Willing and able to provide consent No known cardiac amyloidosis or recent myocardial infarction Exclusion Criteria: Dialysis NYHA (New York Heart Association) Class IV Acute myocardial infarction within 6 weeks Pregnancy or nursing History of adverse events from or allergy to gadolinium contrast media Hemodynamic instability Severe claustrophobia despite use of sedatives Decompensated heart failure (unable to lie flat for 1 hour) Concomitant clinically significant non-ischemic non-amyloid heart disease (valvular heart disease or dilated cardiomyopathy) Body weight over limit for MRI table (>300 lbs) Contraindications for MRI (including non-compatible cardiac implantable electronic devices, drug infusion pumps, and metallic or electric implants) Any other reason determined by the investigator to be unsuitable for the study