Excerpt

In this issue of the Journal of Nuclear Cardiology, Persia Paulino et al. present a case of a 40 year old man with a positiveTechnetium-99m-3,3-diphosphono-1,2-2 propanodicarboxylic acid (DPD) scan who was found to have AL cardiac amyloidosis by cardiac biopsy.1 The patient had no prior medical history and was not taking any medications when he presented to the emergency room with symptomatic atrial flutter. Laboratory tests revealed elevated serum troponin, NT-pro B-type brain natriuretic peptide, and creatinine. Echocardiography showed mild global left ventricular hypokinesis with longitudinal strain pattern demonstrating preserved apical function with progressive dysfunction towards the base and Doppler imaging consistent with restrictive physiology. Cardiac magnetic resonance imaging showed late gadolinium enhancement with diffuse subendocardial pattern and difficulty in complete suppression. Since these findings were highly suggestive of cardiac amyloidosis, a DPD scan was done which demonstrated significant cardiac uptake (Perugini visual score Grade 3, Heart-to-Contralateral ratio 1.67). Serum analysis demonstrated a monoclonal gammopathy with a high Lambda light chain. Endomyocardial biopsy was performed with histology showing amyloid deposition in the heart with positive Kappa and Lambda light chains on immunohistochemistry. …