Cardiac amyloidosis in the practice of a cardiologist and therapist. Case report

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Abstract

Amyloidosis is a heterogeneous group of diseases associated with abnormal protein aggregation and deposition in organs and tissues as insoluble fibrils. The heterogeneity of the clinical manifestations of these disorders is due to various precursor proteins and damage to various organs and systems. Diagnosis of the disease is complex and requires high clinical alertness from physicians. Unfortunately, in most cases, the patient's diagnostic path is unreasonably long, and the effectiveness of therapy is primarily determined by the timing of diagnosis. Often, the cardiologist is the first specialist to be approached by a patient with this severe disease. The article highlights approaches to early diagnosis of amyloidosis with heart involvement.

About the authors

T. V. Nikiforova

Sechenov First Moscow State Medical University (Sechenov University)

Author for correspondence.
Email: attrcmp@gmail.com
ORCID iD: 0000-0003-3072-8951

Graduate Student

Russian Federation, Moscow

Z. М. Magomedova

Sechenov First Moscow State Medical University (Sechenov University); Pirogov City Clinical Hospital

Email: attrcmp@gmail.com
ORCID iD: 0000-0001-6753-1525

Graduate Student

Russian Federation, Moscow; Moscow

M. F. Magaramova

Sechenov First Moscow State Medical University (Sechenov University)

Email: attrcmp@gmail.com
ORCID iD: 0009-0005-1889-5249

Graduate Student

Russian Federation, Moscow

E. S. Pershina

Sechenov First Moscow State Medical University (Sechenov University); Pirogov City Clinical Hospital

Email: attrcmp@gmail.com
ORCID iD: 0000-0002-3952-6865

Cand. Sci. (Med.), Assoc. Prof.

Russian Federation, Moscow; Moscow

D. Yu. Shchekochikhin

Sechenov First Moscow State Medical University (Sechenov University)

Email: attrcmp@gmail.com
ORCID iD: 0000-0002-8209-2791

Cand. Sci. (Med.)

Russian Federation, Moscow

D. A. Andreev

Sechenov First Moscow State Medical University (Sechenov University)

Email: attrcmp@gmail.com
ORCID iD: 0000-0002-0276-7374

D. Sci. (Med.)

Russian Federation, Moscow

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2. Fig. 1. Scintigraphic image 3 hours after administration of the radiopharmaceutical in a patient with sAL amyloidosis (GRADE 1): a – planar study – static scintigraphy in the anterior direct projection. Comparison of radiopharmaceutical accumulation in the heart area (2) with the contralateral rib area (1). When calculating the ratio – GRADE 1 – visible accumulation, but its intensity is lower than in bone structures; b – hybrid study – SPECT: the wall and cavity of the LV in the heart area (4) are not clearly differentiated, radiopharmaceutical accumulation is predominantly in the blood pool (shown in blue), does not exceed accumulation in the ribs. The spine is marked with number 3.

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3. Fig. 2. ECG of a patient with ATTR amyloidosis. In standard leads – decreased voltage of the QRS complex (amplitude of the complex is less than 5 mm).

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4. Fig. 3. Scintigraphic image in a patient with ATTRwt: a – planar study – static scintigraphy in the anterior direct projection. Comparison of radiopharmaceutical accumulation in the heart region (2) with the contralateral rib region (1). When calculating the ratio – Grade 3 – visible accumulation, but its intensity is higher than in bone structures; b – hybrid study – SPECT: the wall of the RV (3), LV (5) and interventricular septum (4) are clearly differentiated [shown in yellow], the accumulation of radiopharmaceuticals in which exceeds the accumulation in the ribs.

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5. Fig. 4. EchoCG image in a patient with ATTR amyloidosis: a – global longitudinal strain of the LV (“bull’s eye”, “icing on the cake”). Arrow “1” points to the apex of the LV, where the deformation parameters (in simple terms, contractility) are preserved (marked in red). Arrow “2” points to the basal and middle segments, where this parameter is reduced (apical sparing phenomenon); b – 4-chamber position in echoCG. Arrows “3” and “5” point to the walls of the RV and LV, respectively, “4” – the interventricular septum (note the presence of “granularity”).

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