Ultrasound Therapy In Cardiac Amyloidosis
This is a prospective pilot clinical study of subjects with cardiac amyloidosis and control subjects without amyloidosis where we plan to evaluate changes in myocardial blood flow, systolic and diastolic function before and after sonotherapy.
• 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