Cardiac Amyloidosis echo case by dr Chatziathanasiou-diagnosis, ECG, echo and treatment

1 Views

07/08/23

The ECG and the echocardiogram of a 65 year old man with manifestations of heart failure (paroxysmal nocturnal dyspnea, dyspnea during light activity and ankle edema) are shown. ECG shows low voltage QRS and poor progression of the R waves in the precordial leads. Echo shows hypertrophic left ventricular walls with mildly granular-sparkling appearrance, nearly normal left ventricular systolic function, dilated left atrium, mildly thickened interatrial septum and mitral valve and doppler evidence of severe diastolic dysfunction. The diastolic dysfunction results in the restrictive pattern of mitral inflow Doppler velocities, which was noted in this case and in reduced early diastolic mitral annular velocity (recorded by pulse wave tissue Doppler). Biopsy of rectal mucosa, abdominal subcutaneous fat and endomyocardial biopsy showed tissue infiltration by AL (primary) amyloid. In primary amyloidosis the abnormal protein, which is deposited in various organs causing organ dysfunction, is composed by kappa and lamda immunoglobulin light chains. These antibody-fragments are produced by a proliferating clone of plasma cells. The prognosis in cardiac AL amyloidosis is usually severe.