IJCS | Volume 31, Nº4, July / August 2018

458 Jorge et al Cardiac amyloidosis Int J Cardiovasc Sci. 2018;31(4)457-460 Case Report Figure 1 - (A) Apical four-chamber view showing biatrial enlargement, thickened interatrial septum, left ventricular wall hypertrophy, with hyperechoic texture of the myocardium. (B) Parasternal short axis view. Concentric left ventricular hypertrophy. (C) Bullseyes. Global longitudinal systolic strain (mean value of (-)12%), with prominent basal and medium impairment, and preserved apical mechanical function. (D) Apical four-chamber strain showing that the ratio between the apical inferior septal strain and the lower basal septal strain is greater than 3. (E) Cine-mode of the stationary state free precession showing diffuse left ventricular myocardial thickening. (F) Long axis post-gadolinium image showing diffuse late improvements (arrows) in the left atrium, as well as subendocardial enlargement of the left ventricle, more prominent in the anterior wall. (G) Short axis post- gadolinium image showing diffuse subendocardial enhancement (arrows) in the left ventricle. (H) Four-chamber post-gadolinium image showing late enhancement (arrows) not only in the left chambers, but also of the right atrium. The diagnosis was systemic amyloidosis andmultiple myeloma associated with cardiac involvement. Pulse therapy with prednisone was initiated. The HF became refractory to treatment, and the patient died 3 months after the disease onset. Discussion Amyloidosis is a rare and multisystemic disease. Patients with amyloidosis usually have few specific symptoms, which makes the diagnosis difficult in the initial phase, as the case presented herein. Cardiac impairment due to amyloidosis can lead to HF, as well as conduction system involvement, with low voltage at the ECG, which increases clinical suspicion. 4 In addition to myocardial infiltration, amyloid infiltrates can be found in the conduction system, valve tissues, coronary arteries, large vessels and autonomic or peripheral nerves, leading to many clinical manifestations. 2 More than 25 proteins have been described as possible amyloid-forming agents; however, two of them predominate in cardiovascular impairment: transthyretin (TTR) and immunoglobulin light chains – amyloid light chains orAL. 2 The TTRprotein is synthesized and secreted by the liver and choroid plexus, and functions as a carrier of thyroxine and retinol binding protein. This protein is typically found in soluble tetramers in their native form. TTR has become the most prevalent form of CA found in clinical practice, with greater identification being made by noninvasive imaging tools. 5 Cardiac involvement by TTR occurs most commonly in the sixth and seventh decades of life as HFpEF, 6 with the wild-type or senile