Authors

• Tomo Svaguša — Klinička bolnica Dubrava, Zagreb, Hrvatska — ORCID: 0000-0002-2036-1239
• Boris Starčević — Klinička bolnica Dubrava, Zagreb, Hrvatska — ORCID: 0000-0002-3090-2772
• Diana Rudan — Klinička bolnica Dubrava, Zagreb, Hrvatska — ORCID: 0000-0001-9473-2517

Abstract

**Background:** Amyloidosis is a heterogeneous group of diseases characterized by the accumulation of protein deposits. Some of them affect the heart, and the most common are the immunoglobulin light chains and transthyretin. (1-3) **Case report:** A patient at the age of 60 is admitted to emergency care due to chest pain. She has been taking medicines for dyslipidemia. Chest X ray in ER (emergency room) described a heart enlargement. As a result of the echocardiography, a preserved left ventricular fraction was observed and thickened wall of both ventricles. Coronarography excluded atherosclerotic changes in epicardial vessels. Further treatment of the patient verified an elevated NT-proBNP of 4055.4 pg/ml and a 24-hour proteinuria of 1.52 g. Electrophoresis of proteins and immunofixation indicates elevated lambda light chains. Bone marrow puncture is performed and multiplying plasma cells (15%) were observed. Due to kidney involvement, the kidney biopsy was performed with a positive pathohistological finding of amyloidosis with lambda light chains. The second patient at the age of 57 is admitted to emergency care due to signs of heart failure. He has a history of Hashimoto’s disease. Chest X ray in ER has described myopathic heart. 12-lead ECG is characterized by non-specific conduction disorders. As a result of the echocardiography, slightly lowered left ventricular fraction was observed and thickened walls of both ventricles. Further tests verified an increased NT-proBNP > 8000 pg/ml. Electrophoresis of proteins and immunofixation indicates elevated kappa light chains. Bone marrow puncture is performed and multiplied plasma cells (9%) were observed. Due to skin lesions of the face, the biopsy was performed with a positive pathohistological finding of amyloidosis with kappa light chains. **Conclusion:** The timely recognition of cardiac amyloidosis is of extreme importance for the treatment and prognosis of the disease. The timely detection of illness provides patients with adequate treatment and significant life extensions.

Keywords

amyloidosis, heart failure, echocardiography

DOI

Literature

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