Características clínicas, eletrocardiográficas e ecocardiográficas na amiloidose cardíaca significativa detectada apenas à necrópsia: comparação com casos diagnosticados em vida

Gutierrez, Paulo Sampaio; Fernandes, Fábio; Mady, Charles; Higuchi, Maria de Lourdes

doi:10.1590/s0066-782x2008000300009

Cited by 13 publications

(8 citation statements)

References 14 publications

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“… 1 The real incidence of cardiac amyloidosis (CA) is not known precisely and is often diagnosed only during autopsy. 8 Heart failure usually occurs in CA due to the combination of decreased myocardial compliance and compressed myocardial cells. These changes develop due to the infiltration by amyloid deposits and could lead to restrictive cardiomyopathy.…”

Section: Introductionmentioning

confidence: 99%

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Nemes¹,

Földeák²,

Domsik³

et al. 2018

Arquivos Brasileiros De Cardiologia

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BackgroundLight-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis.ObjectivesTo characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE).MethodsA total of 16 patients (mean age: 64.5 ± 10.1 years, 11 males) with AL-CA were examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant.ResultsSignificant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients.ConclusionSignificantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA.

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Section: Introductionmentioning

confidence: 99%

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Nemes¹,

Földeák²,

Domsik³

et al. 2018

Arquivos Brasileiros De Cardiologia

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“…5 Patients in G1 had increased PR interval (mean of 0.230s ± 0.060), which was significantly different from G2 (p=0.015), G3 (p=0.044) and G4 (p=0.005). Sayed et al 22 also showed an association between disturbances in electrical conduction and decreased longitudinal strain, and a negative prognostic value for this association, although the study was conducted with cardiac AL amyloidosis.…”

Section: Discussionmentioning

confidence: 81%

“…The echocardiogram may show symmetrical thickening of left ventricular (LV) wall, hypokinesia, right ventricular free wall thickening, atrial septal thickening, valve thickening or valve failure, atrial dilatation and pericardial effusion 5 (Figure 1). Two dimensional (2D) speckle tracking echocardiography (STE) consists in the capture and tracking of speckles along the cardiac cycle, generating motion vectors and deformation curves.…”

Section: Introductionmentioning

confidence: 99%

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy

Rocha¹,

Ferreira²,

Nacif³

et al. 2016

Arquivos Brasileiros De Cardiologia

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BackgroundAmyloidosis is a disease caused by deposits of insoluble fibrils in extracellular spaces. The most common type of familial amyloidosis is mediated by mutation of transthyretin, especially Val30Met. Symptoms and ejection fraction decrease may occur in cardiac amyloidosis only in case of poor prognosis. Myocardial strain detected by two-dimensional speckle tracking echocardiography can indicate changes in myocardial function at early stages of the disease.ObjectiveTo determine the accuracy of left ventricular longitudinal strain by two-dimensional speckle tracking echocardiography in patients with familial amyloidosis caused by Val30Met transthyretin mutation.MethodsEighteen consecutive patients, carriers of transthyretin mutation, were evaluated by two-dimensional speckle tracking echocardiography, by which myocardial strain curves were obtained, following the American Society of Echocardiography recommendations.ResultsPatients were divided into three groups: 1- Val30Met with cardiac amyloidosis; 2-Val30Met with extracardiac amyloidosis; 3 - Val30Met without evidence of disease. As the three groups were compared by the Mann-Whitney test, we found a statistically significant difference between groups 1 and 2 in the mean longitudinal tension (p=0.01), mean basal longitudinal strain (p=0.014); in mean longitudinal tension and mean longitudinal strain between groups 1 and 3 (p=0.005); and in the ratio of longitudinal strain of apical septum segment to longitudinal strain of basal septum (p=0.041) between groups 2 and 3.ConclusionLeft ventricular longitudinal strain detected by two-dimensional speckle tracking echocardiography is able to diagnose left ventricular dysfunction in early stages of familial amyloidosis caused by transthyretin Val30Met mutation.

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“…O eletrocardiograma é anormal em 90% dos casos com envolvimento cardíaco, 2 sendo o achado mais característico a presença de complexos QRS de baixa voltagem. 5 Arritmias, particularmente a fibrilação atrial, são comuns e podem estar relacionadas com a infiltração por amilóide dos átrios, assim também como a infiltração do sistema de condução pode levar a bloqueios. 2 No ecocardiograma, os principais achados incluem aumento da espessura das paredes ventriculares (espessura ≥ 15 mm é um achado independente para eventos adversos), 1 dilatação dos átri-os, cavidades ventriculares normais, espessamento do septo interatrial e pequeno derrame pericár-dico.…”

Section: Comentáriosunclassified

Cardiac cmyloidosis: case report

Carvalho

Rodrigues

Vitório

2017

Medicina (Ribeirão Preto)

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RESUMOA amiloidose é resultante do depósito extracelular de proteínas séricas (amiloide) e pode acometer quase todos os órgãos. A amiloidose cardíaca é uma cardiomiopatia geralmente progressiva que apresenta prognóstico reservado. Neste relato apresentamos um paciente com sinais e sintomas de insuficiência cardíaca e hipertrofia ventricular esquerda, que devido ao diagnóstico tardio de amiloidose cardía-ca, acabou evoluindo a óbito. Assim, esta doença deve ser sempre considerada na ausência de outra causa que justifique tais achados. Palavras-chave:Amiloide. Insuficiência Cardíaca. Sinais e Sintomas. ABSTRACTAmyloidosis is a result of the extracellular deposit of seric proteins (amyloid) and almost all organs can be compromised. Cardiac amyloidosis is usually a progressive cardiomyopathy that has a bad prognosis. We present here a case report about a patient that had signs and symptoms of heart failure and left ventricle hypertrophy. Because it was not possible to make the diagnosis of cardiac amyloidosis in time, he died. This disease must be considered when no other cause for those findings exists.

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Características clínicas, eletrocardiográficas e ecocardiográficas na amiloidose cardíaca significativa detectada apenas à necrópsia: comparação com casos diagnosticados em vida

Cited by 13 publications

References 14 publications

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Speckle Tracking and Transthyretin Amyloid Cardiomyopathy

Cardiac cmyloidosis: case report

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