Heart transplantation for transthyretin amyloidosis. Clinical case

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Abstract

Amyloid cardiomyopathy is an infiltrative disease with extremely adverse prognosis. New treatments are now emerging, however they are not aimed at eliminating the existing deposits of amyloid, but at preventing new ones. Given the difficulties of diagnosing the disease at an early stage, patients often end up in the hospital with advanced heart failure. In such cases, the efficacy of a pathogenic therapy is extremely low, which makes the treatment of an advanced stage of the disease very important. The article discusses the possibility of heart transplantation for patients with amyloid cardiomyopathy. A clinical case of a patient with hereditary transthyretin amyloidosis who was performed biatric orthotopic heart transplantation is presented.

About the authors

Svetlana N. Nasonova

Chazov National Medical Research Center of Cardiology

Author for correspondence.
Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-0920-7417

канд. мед. наук, ст. науч. сотр. отд. заболеваний миокарда и сердечной недостаточности

Russian Federation, Moscow

Igor V. Zhirov

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-4066-2661

д-р мед. наук, вед. науч. сотр. отд. заболеваний миокарда и сердечной недостаточности

Russian Federation, Moscow

Anastasia A. Shoshina

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-9519-7373

аспирант отд. заболеваний миокарда и сердечной недостаточности

Russian Federation, Moscow

Yulia F. Osmolovskaya

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-7827-2618

канд. мед. наук, науч. сотр. отд. заболеваний миокарда и сердечной недостаточности

Russian Federation, Moscow

Olga Ya. Tchaikovskaya

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-2251-1675

врач функциональной диагностики отд-ния ультразвуковой диагностики

Russian Federation, Moscow

Svetlana V. Dobrovolskaya

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0003-0580-393X

канд. мед. наук, мл. науч. сотр. отд. ультразвуковых методов исследования

Russian Federation, Moscow

Marina A. Saidova

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-3233-1862

д-р мед. наук, проф., рук. отд. ультразвуковых методов исследования

Russian Federation, Moscow

Ekaterina A. Butorova

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0001-9706-7049

канд. мед. наук, врач-рентгенолог, зав. отд-нием лучевой диагностики

Russian Federation, Moscow

Alexey A. Ansheles

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-2675-3276

д-р мед. наук, вед. науч. сотр. отд. радионуклидной диагностики и позитронно-эмиссионной томографии

Russian Federation, Moscow

Maria S. Kazieva

Scientific Center of Neurology

Email: dr.nasonova@mail.ru
ORCID iD: 0009-0007-5683-0934

врач-невролог

Russian Federation, Moscow

Daria A. Grishina

Scientific Center of Neurology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-7924-3405

канд. мед. наук, рук. Центра заболеваний периферической нервной системы Института клинической и профилактической неврологии

Russian Federation, Moscow

Lev B. Krougly

Shumakov National Medical Research Center for Transplantology and Artificial Organs

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-1365-3970

канд. мед. наук, врач-кардиолог отд-ния кардиологии

Russian Federation, Moscow

Natalia P. Mozheiko

Shumakov National Medical Research Center for Transplantology and Artificial Organs

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-4841-7070

канд. мед. наук, зав. отд-нием патологической анатомии

Russian Federation, Moscow

Nadegda N. Koloskova

Shumakov National Medical Research Center for Transplantology and Artificial Organs

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0002-5819-9046

д-р мед. наук, зав. отд-нием кардиологии

Russian Federation, Moscow

Sergey N. Tereshchenko

Chazov National Medical Research Center of Cardiology

Email: dr.nasonova@mail.ru
ORCID iD: 0000-0001-9234-6129

д-р мед. наук, проф., рук. отд. заболеваний миокарда и сердечной недостаточности

Russian Federation, Moscow

References

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Supplementary files

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2. Fig. 1. Patient B.’s medical history.

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3. Fig. 2. Patient B.’s ECG.

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4. Fig. 3. Patient B.’s myocardial deformation parameters.

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5. Fig. 4. Cardiac MRI. Cine-MRI sequence: a – two-chamber long axis of the heart, end-diastolic phase; b – four-chamber axis of the heart, end-systolic phase. The arrows indicate a deformed LA with a thickened wall.

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6. Fig. 5. Contrast-enhanced cardiac MRI. Sequence with delayed contrast enhancement: a – long axis of the heart; b – four-chamber axis of the heart. The white arrows indicate the contrast agent uptake in the upper parts of the LA (in the residual part of the recipient’s atrium). The red arrows indicate the absence of uptake in the myocardium of the lower parts of the LA at the level of the atrioventricular ring (transplanted part).

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7. Fig. 6. Patient B.’s scintigram in planar imaging mode.

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8.  Fig. 7. Patient B.’s scintigram in SPECT mode.

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9. Fig. 8. Patient B.’s electroneuromyography data.

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