Difficulties in the Diagnosis of Cardiac Amyloidosis and Treatment Options: Case Report

Česká verzia

Authors: Renáta Závodná ¹; Kamil Zeman ¹; Martin Pleva ²; Milan Kamínek ³
Authors place of work: Interní oddělení Nemocnice ve Frýdku-Místku ¹; Oddělení intervenční radiologie, Magnetická rezonanceKomplexního kardiovaskulárního centra, Nemocnice Podlesí, Třinec ²; Klinika nukleární medicíny LF UP a FN Olomouc ³
Published in the journal: Vnitř Lék 2020; 66(1): 71-77
Category: Case Report

Summary

Amyloidosis is a disease characterized by extracellular deposition of amyloid, an insoluble pathological protein. Clinical manifestation is based on the type of amyloid, and to define the specific type of amyloid is crucial to determining prognosis and optimal therapy. Echocardiography may be the first method to highlight the diagnosis of cardiac amyloidosis, but this is not a specific method. Typical echocardiographic findings in cardiac amyloidosis are myocardial enlargement, atrial dilatation, decreased longitudinal contraction with relatively long preserved systolic function of the left ventricle and left ventricular diastolic dysfunction with restrictive type of filling. Magnetic resonance gives a somewhat specific image of late gadolinium enhancement, but cannot distinguish individual types of amyloid. The biopsy can be falsely negative. In recent years, methods of nuclear medicine have become more important, especially in case of the transthyretin form of amyloidosis. Our case report shows a 72-year-old male, in whom 99mTc-DPD scintigraphy revealed the senile form of transthyretin amyloidosis. The therapeutic possibilities of transthyretin form of cardiac amyloidosis are currently being explored.

Keywords:

Biopsy – scintigraphy – magnetic resonance – echocardiography – Amyloidosis – doxycycline – tauroursodeoxycholic acid – 99mTc-Diphosphono-Propanodicarboxylic Acid

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