Description
Inclusion Criteria:
- * Age \> 18 years for AL-CA, \> 65 years for ATTR-CA, \> 65 years for controls
- * Willing and able to provide consent
- * Diagnosis of systemic light chain amyloidosis by standard criteria (see below) and in hematological remission (normal serum free light chain levels)
- * (immunofixation of serum and urine, IgG 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 (\>9 ng/L: female, \>14 ng/L: male) or abnormal age appropriate N terminal pro-brain natriuretic peptide, 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) or
- * abnormal cardiac MRI (wall thickness \> 12 mm or extracellular volume \> 0.35) OR
- * Diagnosis of 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, or
- * grade 2 or grade 3 myocardial uptake of technetium-99m pyrophosphate (PYP) if AL amyloidosis is excluded
Exclusion Criteria:
- * Hemodynamic instability
- * Severe claustrophobia despite use of sedatives
- * Decompensated heart failure (unable to lie flat for 1 hour)
- * Concomitant non-ischemic non-amyloid heart disease (valvular heart disease or dilated cardiomyopathy)
- * Severe valve stenosis or regurgitation in the aortic, mitral or tricuspid valves, including prior valve replacement
- * Severe pulmonary artery hypertension
- * Severe lung disease
- * Known obstructive epicardial coronary artery disease with stenosis \> 50% in any single territory
- * Prior cardiac surgery
- * Regional wall motion abnormality on echocardiogram
- * Left ventricular ejection fraction \< 40%
- * Pregnant state
- * Documented allergy to N-13 ammonia or Definity
- * Contraindications to DEFINITY® (Perflutren Lipid Microsphere) Injectable Suspension:
- o Patients with known or suspected Right-to-left, bi-directional, or transient right-to-left cardiac shunts, Hypersensitivity to perflutren
- * Contraindications or challenges to sonotherapy
- * Severe electrolyte abnormalities
- * QTc prolongation (values are greater than 450 milliseconds in males and greater than 470 milliseconds in females)
- * BMI \> 35 kg/m2
- * Documented intracardiac thrombus
- * Atrial fibrillation not on anticoagulation
- * Prior history of stroke
- * Any other reason determined by the investigator that makes a subject a poor candidate for ultrasound therapy
Ages Eligible for Study:
18 Years to 90 Years (ADULT, OLDER_ADULT)
Sexes Eligible for Study: ALL
Accepts Healthy Volunteers:
Yes