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 fatpad/bone marrow, or organ biopsy, followed by typing of the light chain usingimmunohistochemistry or immunogold assay with confirmation by mass spectroscopy asneeded AND

• Proof of cardiac involvement by AL amyloidosis

• Abnormal cardiac biomarkers: abnormal high sensitivity TnT 5th generationlevels (> 15 ng/L) or abnormal age-appropriate NT-proBNP (abnormal values: < 50years: > 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 causesof increased LV wall thickness) OR

• Abnormal CMR (wall thickness > 12 mm, extracellular volume > 0.40 or typicalCMR appearance of cardiac amyloidosis with difficulty nulling images andnon-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 cardiacamyloidosis by standard criteria: Endomyocardial biopsy followed by typing of thetransthyretin amyloidosis using immunohistochemistry or immunogold assay withconfirmation 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 wallmotion abnormalities in a pattern consistent with an ischemic etiology in morethan one segment

Inclusion Criteria for hypertrophic cardiomyopathy subjects:

• Age > 18 years

• Willing and able to provide consent

• Hypertrophic cardiomyopathy: Diagnosis of hypertrophic cardiomyopathy by standardcriteria

• 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 (valvularheart disease or dilated cardiomyopathy)

• Body weight over limit for MRI table (>300 lbs)

• Contraindications for MRI (including non-compatible cardiac implantable electronicdevices, drug infusion pumps, and metallic or electric implants)

• Any other reason determined by the investigator to be unsuitable for the study