Cardiac amyloidosis: non-invasive diagnosis

Cardiac amyloidosis is an infiltrative disease which requires a high degree of clinical suspicion for appropriate diagnosis. Early diagnosis and the definition of the type of amyloidosis play a key role in the early treatment and prognosis of this disease. In this context, the use of cardiac biomarkers such as troponins and NT-proBNT associated with analysis by multimodality imaging methods like echocardiographic techniques such as strain, nuclear medicine, and cardiovascular resonance imaging have an increasing role in patients with cardiac amyloidosis. This article details the role of non-invasive diagnostic methods in patients with cardiac amyloidosis.

Amyloidosis; Echocardiography; Magnetic resonance spectroscopy; Nuclear medicine; Biomarkers

Authorship

Viviane Tiemi Hotta

. Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.Universidade de São PauloBrasilSão Paulo, SP, Brasil. Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.

. Fleury Medicina e Saúde, São Paulo, SP, Brasil.Fleury Medicina e SaúdeBrasilSão Paulo, SP, Brasil. Fleury Medicina e Saúde, São Paulo, SP, Brasil.

http://orcid.org/0000-0002-9236-3815

Maria Clementina Pinto Giorgi

http://orcid.org/0000-0003-2265-3769

Fábio Fernandes

http://orcid.org/0000-0002-9323-8805

Maria Cristina Donadio Abduch

http://orcid.org/0000-0003-4679-9455

Andréa Maria Gomes Marinho Falcão

http://orcid.org/0000-0003-2333-2828

Charles Mady

http://orcid.org/0000-0002-8838-199X

CORRESPONDING AUTHOR: Viviane Tiemi Hotta. Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo Av. Dr. Enéas de Carvalho Aguiar, 44 Cerqueira César - ZIP Code: 05403-000 São Paulo, SP – Brasil Tel: 55 (11) 2661 5057 – Fax: 55 (11) 2661 5057 E-mail: viviane.hotta@gmail.com ; viviane.hotta@grupofleury.com.br

SCIMAGO INSTITUTIONS RANKINGS

. Fleury Medicina e Saúde, São Paulo, SP, Brasil.Fleury Medicina e SaúdeBrasilSão Paulo, SP, Brasil. Fleury Medicina e Saúde, São Paulo, SP, Brasil.

Figures

Figures (3)

Thumbnail

FIGURE 1
A. Electrocardiogram (ECG) of a patient with TTR amyloidosis showing low QRS complex voltage in the frontal plane, in addition to signs of an electrically inactive area in the anterior wall (“pseudo infarction”). B. Typical echocardiographic findings of a patient with AC: thickening of the left ventricular walls with a shiny and granular appearance in parasternal longitudinal view (i), Apical 4-chambers (ii) and Apical 2-Chambers (iii); global and segmental longitudinal myocardial deformation impairment (strain) (iv to vi). There is less involvement of the apical strain in relation to the basal and midsections (apical sparing pattern) (vi).

Thumbnail

FIGURE 2
A. 85-year-old patient with TTR amyloidosis. The images were acquired three hours after the administration of 20 mCi of pyrophosphate labelled with technetium 99m and show intense increased uptake in the projection of the entire cardiac area. B. Area 1: considers the average counts in the heart area (H = 66.1 counts per pixel). Area 2: considers the average counts in the contralateral thoracic area (CL = 40.5 counts per pixel). Heart/contralateral thoracic area ratio H/CL = 1.63.

Thumbnail

FIGURE 3
CMR images of four chambers (A) and transversal (B), demonstrating an increase in the thickness of the myocardial wall. In C and D, the CMR images show late gadolinium enhancement in four chambers. There is a diffuse increase of myocardial signal despite the use of multiple inversion times.

FIGURE 1 A. Electrocardiogram (ECG) of a patient with TTR amyloidosis showing low QRS complex voltage in the frontal plane, in addition to signs of an electrically inactive area in the anterior wall (“pseudo infarction”). B. Typical echocardiographic findings of a patient with AC: thickening of the left ventricular walls with a shiny and granular appearance in parasternal longitudinal view (i), Apical 4-chambers (ii) and Apical 2-Chambers (iii); global and segmental longitudinal myocardial deformation impairment (strain) (iv to vi). There is less involvement of the apical strain in relation to the basal and midsections (apical sparing pattern) (vi).

FIGURE 2 A. 85-year-old patient with TTR amyloidosis. The images were acquired three hours after the administration of 20 mCi of pyrophosphate labelled with technetium 99m and show intense increased uptake in the projection of the entire cardiac area. B. Area 1: considers the average counts in the heart area (H = 66.1 counts per pixel). Area 2: considers the average counts in the contralateral thoracic area (CL = 40.5 counts per pixel). Heart/contralateral thoracic area ratio H/CL = 1.63.

FIGURE 3 CMR images of four chambers (A) and transversal (B), demonstrating an increase in the thickness of the myocardial wall. In C and D, the CMR images show late gadolinium enhancement in four chambers. There is a diffuse increase of myocardial signal despite the use of multiple inversion times.

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Cardiac amyloidosis: non-invasive diagnosis

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[1] CORRESPONDING AUTHOR: Viviane Tiemi Hotta. Instituto do Coração (InCor), Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo Av. Dr. Enéas de Carvalho Aguiar, 44 Cerqueira César - ZIP Code: 05403-000 São Paulo, SP – Brasil Tel: 55 (11) 2661 5057 – Fax: 55 (11) 2661 5057 E-mail: viviane.hotta@gmail.com ; viviane.hotta@grupofleury.com.br