Early identification of cardiac ATTR amyloidosis: a clinical case

Progress in instrumental diagnostics, as well as increased awareness among doctors about rare diseases, is steadily leading to an increased number of patients diagnosed with cardiac amyloidosis. Regardless of amyloid formation route, cardiac damage is the main cause of mortality in systemic amyloido...

Full description

Saved in:
Bibliographic Details
Main Authors: A. A. Vedernikov, E. M. Mezhonov, N. Е. Shirokov, V. A. Balina, O. M. Reitblat, D. V. Teffenberg, S. V. Shalaev
Format: Article
Language:English
Published: Столичная издательская компания 2024-07-01
Series:Рациональная фармакотерапия в кардиологии
Subjects:
Online Access:https://www.rpcardio.online/jour/article/view/3031
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1849227502219689984
author A. A. Vedernikov
E. M. Mezhonov
N. Е. Shirokov
V. A. Balina
O. M. Reitblat
D. V. Teffenberg
S. V. Shalaev
author_facet A. A. Vedernikov
E. M. Mezhonov
N. Е. Shirokov
V. A. Balina
O. M. Reitblat
D. V. Teffenberg
S. V. Shalaev
author_sort A. A. Vedernikov
collection DOAJ
description Progress in instrumental diagnostics, as well as increased awareness among doctors about rare diseases, is steadily leading to an increased number of patients diagnosed with cardiac amyloidosis. Regardless of amyloid formation route, cardiac damage is the main cause of mortality in systemic amyloidosis. The article presents a unique clinical observation of hereditary transthyretin (ATTR) amyloidosis mixed phenotype detection during skeletal scintigraphy. In the patient with severe shortness of breath, based on the results of radiological research, an oncological process in the lungs was suspected. The patient underwent skeletal scintigraphy to exclude metastatic lesions, during which a scintigraphy pattern characteristic of ATTR amyloidosis (Grade 3) was revealed. Subsequently, the oncological diagnosis was removed. An independent disease of hematopoietic system was excluded, and mutation in TTR gene was additionally confirmed. This clinical case illustrates the possibility of making a diagnosis of cardiac amyloidosis without resorting to myocardial biopsy, when the patient still has slight limitations in physical activity (NYHA class II) and there is no late gadolinium enhancement on magnetic resonance imaging.
format Article
id doaj-art-6bd34766cee441b8a962881a749c955a
institution Kabale University
issn 1819-6446
2225-3653
language English
publishDate 2024-07-01
publisher Столичная издательская компания
record_format Article
series Рациональная фармакотерапия в кардиологии
spelling doaj-art-6bd34766cee441b8a962881a749c955a2025-08-23T10:00:36ZengСтоличная издательская компанияРациональная фармакотерапия в кардиологии1819-64462225-36532024-07-0120335736610.20996/1819-6446-2024-30312216Early identification of cardiac ATTR amyloidosis: a clinical caseA. A. Vedernikov0E. M. Mezhonov1N. Е. Shirokov2V. A. Balina3O. M. Reitblat4D. V. Teffenberg5S. V. Shalaev6Regional Clinical Hospital No.1Regional Clinical Hospital No.1; Tyumen State Medical UniversityTomsk National Research Medical Center, Russian Academy of SciencesRegional Clinical Hospital No.1Regional Clinical Hospital No.1Tyumen Cardiology Research Center, Tomsk National Research Medical CenterRegional Clinical Hospital No.1; Tyumen State Medical UniversityProgress in instrumental diagnostics, as well as increased awareness among doctors about rare diseases, is steadily leading to an increased number of patients diagnosed with cardiac amyloidosis. Regardless of amyloid formation route, cardiac damage is the main cause of mortality in systemic amyloidosis. The article presents a unique clinical observation of hereditary transthyretin (ATTR) amyloidosis mixed phenotype detection during skeletal scintigraphy. In the patient with severe shortness of breath, based on the results of radiological research, an oncological process in the lungs was suspected. The patient underwent skeletal scintigraphy to exclude metastatic lesions, during which a scintigraphy pattern characteristic of ATTR amyloidosis (Grade 3) was revealed. Subsequently, the oncological diagnosis was removed. An independent disease of hematopoietic system was excluded, and mutation in TTR gene was additionally confirmed. This clinical case illustrates the possibility of making a diagnosis of cardiac amyloidosis without resorting to myocardial biopsy, when the patient still has slight limitations in physical activity (NYHA class II) and there is no late gadolinium enhancement on magnetic resonance imaging.https://www.rpcardio.online/jour/article/view/3031cardiac amyloidosistransthyretin amyloidosisscintigraphydiagnosisclinical case
spellingShingle A. A. Vedernikov
E. M. Mezhonov
N. Е. Shirokov
V. A. Balina
O. M. Reitblat
D. V. Teffenberg
S. V. Shalaev
Early identification of cardiac ATTR amyloidosis: a clinical case
Рациональная фармакотерапия в кардиологии
cardiac amyloidosis
transthyretin amyloidosis
scintigraphy
diagnosis
clinical case
title Early identification of cardiac ATTR amyloidosis: a clinical case
title_full Early identification of cardiac ATTR amyloidosis: a clinical case
title_fullStr Early identification of cardiac ATTR amyloidosis: a clinical case
title_full_unstemmed Early identification of cardiac ATTR amyloidosis: a clinical case
title_short Early identification of cardiac ATTR amyloidosis: a clinical case
title_sort early identification of cardiac attr amyloidosis a clinical case
topic cardiac amyloidosis
transthyretin amyloidosis
scintigraphy
diagnosis
clinical case
url https://www.rpcardio.online/jour/article/view/3031
work_keys_str_mv AT aavedernikov earlyidentificationofcardiacattramyloidosisaclinicalcase
AT emmezhonov earlyidentificationofcardiacattramyloidosisaclinicalcase
AT neshirokov earlyidentificationofcardiacattramyloidosisaclinicalcase
AT vabalina earlyidentificationofcardiacattramyloidosisaclinicalcase
AT omreitblat earlyidentificationofcardiacattramyloidosisaclinicalcase
AT dvteffenberg earlyidentificationofcardiacattramyloidosisaclinicalcase
AT svshalaev earlyidentificationofcardiacattramyloidosisaclinicalcase