Heart involvement in AL-amyloidosis. Current state of the issue

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  • Authors: Trukhin I.V.1, Schekochikhin D.Y.1, Novikova N.A.1, Akselrod A.S.1, Syrkin A.L.1, Syrkina H.A.1, Grachev A.E.2, Gribanova H.O.2, Okhota V.K.2, Ryzhko V.V.2, Pershina E.S.3
  • Affiliations:
    1. I.M. Sechenov First Moscow State Medical University (Sechenov University)
    2. National Research Center for Hematology
    3. Pirogov First Moscow Clinical Hospital
  • Issue: Vol 74, No 5 (2019)
  • Pages: 307-316
  • Section: CARDIOLOGY AND CARDIOVASCULAR SURGERY: CURRENT ISSUES
  • URL: https://vestnikramn.spr-journal.ru/jour/article/view/1184
  • DOI: https://doi.org/10.15690/vramn1184
  • Cite item
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Abstract


AL cardiac amyloidosis is a relatively rare disorder that belongs to the group of infiltrative cardiomyopathies. Diagnosis of primary amyloidosis is challenging due to many unspecific symptoms and sings, which often leads to late diagnosis when treatment options are limited. Primary amyloidosis particularly needs to be excluded in patients with heart failure with preserved ejection fraction. Therapy in cardiac amyloidosis has to main vectors: 1) chemotherapy to eliminate amyloidogenic plasmatic cells 2) heart failure treatment. The main challenge for cardiologists is to support hemodynamics until response to chemotherapy occurs. In the article the issue of diagnostics, risk stratification and treatment of primary cardiac amyloidosis is addressed.


Ivan V. Trukhin

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: ivan.trukhin@gmail.com
ORCID iD: 0000-0003-1092-6563
SPIN-code: 1911-9569

Russian Federation, 8-2, Trubetskaya street, Moscow, 119992

Department of Preventive and Emergency Cardiology, resident physician

Dmitrij Yu. Schekochikhin

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: agishm@list.ru
ORCID iD: 0000-0002-8209-2791
SPIN-code: 3753-6915

Russian Federation, 8-2, Trubetskaya street, Moscow, 119992

MD, PhD

Nina A. Novikova

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: nanovikova@mail.ru
ORCID iD: 0000-0002-1645-781X
SPIN-code: 7876-8609

Russian Federation, 8-2, Trubetskaya street, Moscow, 119992

MD, PhD, Professor

Anna S. Akselrod

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: 7402898@mail.ru
ORCID iD: 0000-0003-3417-794X
SPIN-code: 4566-7759

Russian Federation, 8-2, Trubetskaya street, Moscow, 119992

д.м.н., профессор

Abram L. Syrkin

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: previntenscardiology@yandex.ru
ORCID iD: 0000-0002-9602-292X
SPIN-code: 8884-8014

Russian Federation, 8-2, Trubetskaya street, Moscow, 119992

MD, PhD, Professor

Helena A. Syrkina

I.M. Sechenov First Moscow State Medical University (Sechenov University)

Email: e_syrkina@mail.ru
ORCID iD: 0000-0002-8072-4513
SPIN-code: 7553-0371

Russian Federation, 8-2, Trubetskaya street, Moscow, 119992

MD, PhD

Alexander E. Grachev

National Research Center for Hematology

Email: Gra4al@yandex.ru
ORCID iD: 0000-0001-7221-9392
SPIN-code: 4281-3923

Russian Federation, 4, Novyi Zykovsky pr., Moscow, 125167

MD, PhD

Helena O. Gribanova

National Research Center for Hematology

Email: el.gribanova@mail.ru
ORCID iD: 0000-0002-4155-7820
SPIN-code: 3772-7386

Russian Federation, 4, Novyi Zykovsky pr., Moscow, 125167

MD, PhD

Valeria K. Okhota

National Research Center for Hematology

Email: Sheveleva10@gmail.com
ORCID iD: 0000-0002-2457-1428
SPIN-code: 2821-3160

Russian Federation, 4, Novyi Zykovsky pr., Moscow, 125167

Vyacheslav V. Ryzhko

National Research Center for Hematology

Email: Gra4al@yandex.ru
ORCID iD: 0000-0003-1221-3495
SPIN-code: 1289-1850

Russian Federation, 4, Novyi Zykovsky pr., Moscow, 125167

MD, PhD

Ekaterina S. Pershina

Pirogov First Moscow Clinical Hospital

Email: pershina86@mail.ru
ORCID iD: 0000-0002-3952-6865
SPIN-code: 7311-9276

Russian Federation, 8, Leninskiy avenue, Moscow, 119049

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Supplementary files

Supplementary Files Action
1. Fig. Algorithm for the diagnosis and typing of cardiac amyloidosis in patients with heart failure of unknown etiology (adapted from [9]) View (500KB) Indexing metadata

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