Diagnostic and therapeutic challenges in rapidly progressing cardiac amyloidosis: a literature review based on case report

Figures

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  Fig. 1. The electrocardiogram obtained at the patient’s presentation Shows low-voltage cardiac waves, which were evident in the limb leads

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  Fig. 2. The patient’s chest X-ray showed nodular densities, suggesting amyloid deposition (red arrows)

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  Fig. 3. The echocardiogram findings were suggestive of restrictive cardiomyopathy

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  Fig. 4. Cardiac MRI showed evidence of restricted cardiomyopathy suggestive of cardiac amyloidosis. The left and right atrium were high-normal in size (LA area: 19cm2, LA area index: 11 cm2/m2, RA area: 22cm2, RA area index: 12 cm2/m2), and the LAS was hypertrophied. LV hypertrophy (maximal septal thickness 14 mm in the inferoseptal area) and a mild reduced LV systolic function (LV ejection fraction: 46%, and LV end-diastolic volume:100 ml) could be seen. Mild RVH and mild reduced RV systolic dysfunction could also be detected (RV ejection fraction: 43% and RV end-diastolic volume: 100 ml). Moreover, mild mitral regurgitation, mild tricuspid regurgitation, and mild mitral thickness were demonstrated in the valvular evaluation. The main pulmonary artery was mildly dilated (13 mm). Moderate left-sided pleural effusion was obvious, with evidence of mild pericardial effusion (anterior LV side: 6 mm and inferior LV side: 9 mm) without any compressive effect on the LV or RV. In the gadolinium phase, diffuse subendocardial to transmural enhancement is achieved throughout LV and RV and bilateral walls and enhancement of inter-atrial septum and atrioventricular valves

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  Fig. 5. The bone marrow biopsy of the patient showed amyloid deposition, confirming the provisional diagnosis of systemic and cardiac amyloidosis