Ultrasound Therapy In Cardiac Amyloidosis

Eligibility 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
• * 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:
• * 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