Case Session 05: Systemic and Inflammatory Disease (2018)-Cardiac Amyloidosis with Normal Transthoracic Echo

Cardiac Amyloidosis with Normal Transthoracic Echo

Video Transcription

Video Summary

A 58-year-old woman with dyspnea, orthopnea, edema, and severe hypoalbuminemia had a normal ECG and largely normal echocardiogram, including normal systolic and diastolic function and normal strain. Because of concern for systemic disease and nephrotic syndrome, cardiac MRI was performed and showed elevated native T1, abnormal nulling, diffuse subendocardial and atrial late gadolinium enhancement, and small pleural/pericardial effusions—findings suggestive of cardiac amyloidosis. SPEP/UPEP revealed IgA lambda monoclonal protein. Renal biopsy confirmed AL amyloid, and bone marrow biopsy showed 66% lambda-restricted plasma cells. She began chemotherapy and diuretic therapy.

Keywords

cardiac amyloidosis

AL amyloid

IgA lambda monoclonal protein

nephrotic syndrome

cardiac MRI