Case of lifetime diagnosis of isolated primary amyloidosis of the heart
https://doi.org/10.18087/cardio.2022.1.n1049
Abstract
The article presents a case of intravital diagnosis of a rare heart disease, isolated primary amyloidosis. The clinical onset of the disease was heart failure (HF) that was resistant to treatment; chemotherapy was ineffective and was poorly tolerated by the patient. The diagnostics was based on a combination of refractory HF and changes in echocardiography data (atrial dilatation, small size of the left ventricular cavity, interventricular septal hypertrophy with the presence of hyperechoic inclusions of the “granular fluorescence” type), and changes detected by contrast-enhanced cardiac magnetic resonance imaging. The diagnosis of amyloidosis was confirmed by results of pathohistological examination of the material obtained during autopsy.
About the Authors
I. V. DemkoRussian Federation
MD, PhD, professor, Head, Department of internal diseases and immunology with a course of postgraduate education
L. I. Pelinovskaya
Russian Federation
PhD, assistent, Department of internal diseases and immunology with a course of postgraduate education
A. Yu. Kraposhina
Russian Federation
PhD, associate professor, Department of internal diseases and immunology with a course of postgraduate education
V. E. Selin
Russian Federation
Assistant, Department of pathological anatomy named after prof. P.G. Podzolkova
A. E. Ryazanov
Russian Federation
Head, Cardiology department No. 1
Ya. I. Verigo
Russian Federation
PhD, doctor cardiologist, Cardiology department № 1
V. A. Mosina
Russian Federation
PhD, associate professor, Department of internal diseases and immunology with a course of postgraduate education
References
1. Ukholkina G.B., Kuchin G.A., Bychkova O.P., Chikhirev O.A. Cardiac amyloidosis in clinical practice. Russian Heart Failure Journal. 2016;17(1):57– 68. DOI: 10.18087/rhfj.2016.1.2201
2. Gillmore JD, Wechalekar A, Bird J, Cavenagh J, Hawkins S, Kazmi M et al. Guidelines on the diagnosis and investigation of AL amyloidosis. British Journal of Haematology. 2015;168(2):207–18. DOI: 10.1111/bjh.13156
3. Grogan M, Scott CG, Kyle RA, Zeldenrust SR, Gertz MA, Lin G et al. Natural History of Wild-Type Transthyretin Cardiac Amyloidosis and Risk Stratification Using a Novel Staging System. Journal of the American College of Cardiology. 2016;68(10):1014–20. DOI: 10.1016/j.jacc.2016.06.033
4. Zhdanova E.A., Gudkova K.V., Rameev V.V., Safarova A.F., Moiseev S.V. Modern Conceptions of Cardiac Amyloidosis. Kardiologiia. 2013;53(12):70–8.
5. Demko I.V., Pelinovskaya L.I., Mosina V.A., Kraposhina A.Yu., Gordeeva N.V., Solovyova I.A. et al. Case of primary amyloidosis with involvement of the heart and pericardium in cardiology practice. Russian Journal of Cardiology. 2017;22(2):103–5. DOI: 10.15829/1560-4071-2017-2-103-105
6. Mershina E.A. Cardiac magnetic resonance imaging in differential diagnosis of cardiomyopathies. Therapy. 2019;5(4(30)):70–8.
7. Zhirov I.V., Safiullina A.A., Nevolina A.V., Sergienko V.B., Tereshenko S.N. Isolated atrial amyloidosis - current state. Clinical pharmacology and therapy. 2018;27(3):48–52.
8. Galeeva Z.M., Galyavich A.S., Baleeva L.V., Galimzyanova L.A., Rafikov A.Yu., Gizatullina N.F. et al. The Case of Amyloidosis in Cardiological Practice. Kardiologiia. 2019;59(1):93–6. DOI: 10.18087/cardio.2019.1.10221
9. Tereshchenko Yu.A., Panchenko T.L., Matveeva I.V. Primary amyloidosis: difficulty in diagnostics. Siberian Medical Review. 2010;5(65):73–6.
10.
Review
For citations:
Demko I.V., Pelinovskaya L.I., Kraposhina A.Yu., Selin V.E., Ryazanov A.E., Verigo Ya.I., Mosina V.A. Case of lifetime diagnosis of isolated primary amyloidosis of the heart. Kardiologiia. 2022;62(1):106-108. (In Russ.) https://doi.org/10.18087/cardio.2022.1.n1049