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1.
Orv Hetil ; 161(5): 169-176, 2020 02.
Artigo em Húngaro | MEDLINE | ID: mdl-31984773

RESUMO

Introduction: Hypereosinophilic syndrome (HES) and immunoglobulin light-chain amyloidosis (ALA) are two, rare haematological disorders associated with cardiac alterations. Aim: The goal of the present study was a comparative assessment of left ventricular (LV) deformational parameters in HES and ALA patients using three-dimensional speckle-tracking echocardiography (3DSTE). Method: In the present study, results of 10 HES patients (mean age: 60.9 ± 14.7 years) and 19 ALA patients (mean age: 63.4 ± 7.8 years, 13 males) were analysed. The control group contained 13 age- and gender-matched healthy adults (mean age: 59.2 ± 4.3 years, 5 males). All patients underwent a complete two-dimensional Doppler echocardiography followed by 3DSTE. Results: All basal segmental LV strains were significantly reduced in ALA patients as compared to the control group. Global and mean segmental LV longitudinal strain (LS) values of ALA patients proved to be significantly decreased as compared to those of the healthy control group. During comparison of HES patients and healthy controls, significant difference could be detected in global LV-LS, while segmental basal LV-LS was also significantly reduced in HES patients. Basal LV radial and 3D strains showed significant differences when parameters of HES and ALA patient groups were compared. Conclusion: 3DSTE is a feasible tool for the detailed assessment of LV deformation in HES and ALA patients. Significant LV deformational abnormalities could be detected in both groups. In the case of ALA, these abnormalities are more prominent. Orv Hetil. 2020; 161(5): 169-176.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Ecocardiografia Tridimensional/métodos , Ventrículos do Coração/diagnóstico por imagem , Síndrome Hipereosinofílica , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Disfunção Ventricular Esquerda/diagnóstico por imagem , Adulto , Idoso , Cardiomiopatias/imunologia , Cardiomiopatias/fisiopatologia , Estudos de Casos e Controles , Ecocardiografia Doppler , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/imunologia , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Masculino , Pessoa de Meia-Idade , Disfunção Ventricular Esquerda/imunologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda
2.
Amyloid ; 26(3): 128-138, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31172799

RESUMO

Background: Atrial fibrillation (AF) commonly affects patients with cardiac amyloidosis (CA). Amyloid deposition within the left atrium may be responsible for the subtype of AF in either permanent or non-permanent form. The prognostic implications of AF and its clinical subtype according to the type of CA are still controversial in this population. This study sought to investigate the prevalence, incidence and prognostic implications of AF and the clinical subtype of AF (permanent or non-permanent) in patients with CA. Methods: Two hundred and thirty-eight patients with CA and full medical records were retrospectively enrolled in the study: About 115 (48%) with light chain (AL) amyloidosis and 123 (52%) with transthyretin amyloidosis (ATTR). Patient's medical records were reviewed to establish baseline prevalence, incidence and impact on all-cause and cardiovascular mortality during follow-up of AF. Results: One hundred and four (44%) patients had history of AF at the time of diagnosis: 62 (60%) permanent and 42 (40%) non-permanent. There were 30 (26%) and 74 (60%) patients with history of AF among patients with AL and ATTR (including 5 hereditary and 69 wild-type), respectively (p<.0001). During the follow-up, 48 new patients developed AF (29, 12 and 7 among patients with AL, wild-type ATTR and hereditary ATTR). After adjustment for age, survival was similar in patients with or without history of AF (HR 0.87 (95% CI, 0.60 to 1.27; p = .467). AF had no impact on cardiovascular mortality. Among the 152 patients with history of AF included in the whole study, there were 75 (49%) patients with permanent AF. After adjustment for age, survival was similar in patients with permanent and non-permanent AF: HR 1.29 (95% CI, 0.84 to 1.99; p = .251). The results were the same among patients with AL or wild-type amyloidosis. Subtype of AF had no impact on cardiovascular mortality. Conclusions: AF is common in patients with CA. However, AF and clinical subtype of AF have no impact on all-cause mortality, whatever the type of amyloidosis.


Assuntos
Neuropatias Amiloides Familiares/diagnóstico por imagem , Fibrilação Atrial/diagnóstico por imagem , Átrios do Coração/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Neuropatias Amiloides Familiares/sangue , Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/mortalidade , Fibrilação Atrial/sangue , Fibrilação Atrial/complicações , Fibrilação Atrial/mortalidade , Ecocardiografia , Feminino , Átrios do Coração/fisiopatologia , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Pré-Albumina/metabolismo , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida
3.
Amyloid ; 26(1): 45-51, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30931628

RESUMO

BACKGROUND: T1 mapping allows quantitative assessment of "diffuse" deposition of amyloid protein in the myocardium. Early detection of cardiac involvement and potential prognostic improvement could benefit patients with AL amyloidosis. OBJECTIVES: This study aims to evaluate the regional variation of amyloid infiltration in the left ventricle and the prognostic value of T1 mapping in patients with AL amyloidosis. METHODS: We prospectively enrolled 77 patients with AL amyloidosis who underwent cardiac magnetic resonance on a 3.0-T scanner. Native T1 and extracellular volume (ECV) were quantitated on the basal, mid, and apical levels of the left ventricle. Late gadolinium enhancement (LGE) pattern (no or non-specific LGE, sub-endocardial LGE, and transmural LGE) was also assessed. Forty healthy subjects served as controls. The primary end point was all-cause mortality. RESULTS: Basal ECV (26.9 ± 2.8% versus 31.1 ± 4.9%, p < .001) were lower than apical ECV in the healthy controls; however, basal ECV (60.6 ± 11.5% versus 53.0 ± 9.6%, p = .003) were significantly higher than apical ECV in patients with transmural LGE. During the follow-up period (median duration, 28 months; 25th-75th percentile, 13.5-38.0 months), 46 patients died. Basal ECV has the largest area under the curve of 0.845 (95% CI, 0.747-0.917) to predict all-cause mortality. Multivariable Cox analysis indicated that basal ECV was an independent prognostic factor and showed incremental prognostic value beyond NYHA class, Mayo stage, and LGE pattern. CONCLUSION: We demonstrated that T1 mapping may have the potential to detect a characteristic amyloid deposition with a decreasing gradient from base to apex. Furthermore, myocardial ECV indicated that basal amyloid infiltration provided robust and incremental prognostic value in patients with AL amyloidosis.


Assuntos
Coração/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Imagem por Ressonância Magnética , Adulto , Idoso , Idoso de 80 Anos ou mais , Meios de Contraste , Feminino , Gadolínio , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Prognóstico , Estudos Prospectivos
4.
Int J Cardiovasc Imaging ; 35(5): 845-854, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30623354

RESUMO

Cardiac involvement in systemic light chain (AL) amyloidosis carries a poor prognosis mainly through involvement of the left ventricular (LV) myocardium. Despite its limitations, two-dimensional transthoracic echocardiography (2D-TTE) remains the main tool used for the assessment of LV systolic function in AL patients. We hypothesize that 3D-TTE coupled with speckle tracking imaging allows earlier detection of LV systolic dysfunction than 2D-TTE in AL amyloidosis. We prospectively studied 71 subjects including 58 patients with confirmed AL amyloidosis (mean age 66 ± 10 years, 60% male) and 21 healthy control (mean age 64 ± 7 years, 48% male) from 2011 to 2014 at the University Hospital of Limoges. The AL patients were divided into three groups according to Mayo Clinic (MC) staging and all subjects underwent 2D-TTE and 3D-TTE at the same setting. Using 2D-TTE, there was no significant difference in LV ejection fraction (EF) between the groups [LVEF = 63 ± 7% (control), 59 ± 6% (MC stage I), 60 ± 8% (MC stage II) and 57 ± 14% (MC stage III) (p = 0.24)]. In contrast, 3D-TTE demonstrated significantly worse LV systolic function in stage II and III patients using 3D-LVEF [MC II and III 45 ± 8% and 39 ± 12% vs. control 53 ± 8% (p < 0.0001)], global longitudinal strain (GLS) [MC II and III - 11 ± 4% and - 8 ± 3% vs. control - 15 ± 3% (p < 0.0001)] and global radial strain (GRS) [MC II and III 14 ± 9% and 10 ± 8% vs. control 25 ± 10% (p < 0.0001)]. Furthermore, MC III patients had significantly worse global circumferential strain and area tracking [- 17 ± 6% and - 25 ± 8% vs. - 24 ± 7% and - 36 ± 7% for control (p < 0.0001)]. Additionally, MC I had significantly better 3D GLS, GRS and global strain (- 15 ± 3%, 25 ± 10% and 28 ± 12%) than MC II (- 11 ± 4%, 14 ± 9% and 16 ± 10%) and MC III patients (- 8 ± 3%, 10 ± 8% and 12 ± 8%), respectively. Despite an apparently preserved LVEF by 2D-TTE, AL patients in MC stage II and III demonstrate evidence of LV systolic dysfunction by 3D imaging using LVEF and strain analysis. Worse LV involvement by AL amyloidosis was associated with more impaired 3D-TTE LV systolic parameters.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Ecocardiografia Tridimensional , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Função Ventricular Esquerda , Idoso , Cardiomiopatias/imunologia , Cardiomiopatias/fisiopatologia , Estudos de Casos e Controles , Bases de Dados Factuais , Diagnóstico Precoce , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/imunologia , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Sístole , Disfunção Ventricular Esquerda/imunologia , Disfunção Ventricular Esquerda/fisiopatologia
6.
BMC Cardiovasc Disord ; 18(1): 221, 2018 12 04.
Artigo em Inglês | MEDLINE | ID: mdl-30509186

RESUMO

BACKGROUND: Cardiac Amyloidosis (CA) pertains to the cardiac involvement of a group of diseases, in which misfolded proteins deposit in tissues and cause progressive organ damage. The vast majority of CA cases are caused by light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). The increased awareness of these diseases has led to an increment of newly diagnosed cases each year. METHODS: We performed multiple searches on MEDLINE, EMBASE and the Cochrane Database of Systematic Reviews. Several search terms were used, such as "cardiac amyloidosis", "diagnostic modalities cardiac amyloidosis" and "staging cardiac amyloidosis". Emphasis was given on original articles describing novel diagnostic and staging approaches to the disease. RESULTS: Imaging techniques are indispensable to diagnosing CA. Novel ultrasonographic techniques boast high sensitivity and specificity for the disease. Nuclear imaging has repeatedly proved its worth in the diagnostic procedure, with efforts now focusing on standardization and quantification of amyloid load. Because the latter would be invaluable for any staging system, those spearheading research in magnetic resonance imaging of the disease are also trying to come up with accurate tools to quantify amyloid burden. Staging tools are currently being developed and validated for ATTR CA, in the spirit of the acclaimed Mayo Staging System for AL. CONCLUSION: Cardiac involvement confers significant morbidity and mortality in all types of amyloidosis. Great effort is made to reduce the time to diagnosis, as treatment in the initial stages of the disease is tied to better prognosis. The results of these efforts are highly sensitive and specific diagnostic modalities that are also reasonably cost effective.


Assuntos
Neuropatias Amiloides Familiares/sangue , Neuropatias Amiloides Familiares/diagnóstico por imagem , Cardiomiopatias/sangue , Cardiomiopatias/diagnóstico por imagem , Ecocardiografia , Amiloidose de Cadeia Leve de Imunoglobulina/sangue , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Imagem por Ressonância Magnética , Tomografia Computadorizada de Emissão , Biomarcadores/sangue , Humanos , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Índice de Gravidade de Doença
8.
J Cardiovasc Magn Reson ; 20(1): 58, 2018 08 16.
Artigo em Inglês | MEDLINE | ID: mdl-30115079

RESUMO

BACKGROUND: To assess the diagnostic and prognosis value of myocardial native T2 measurement in the distinction between Light-chain (AL) and Transthyretin (ATTR) cardiac amyloidosis (CA). METHODS: Forty-four patients with CA (24 AL; 20 ATTR) and 40 healthy subjects underwent 1.5 T cardiovascular magnetic resonance (CMR). They all underwent T1 and T2 mapping (modified Look-Locker inversion recovery), cine and late gadolinium enhancement (LGE) imaging. The Query Amyloid Late Enhancement (QALE) score, myocardial native T2, T1 and extra cellular volume fraction (ECV) were calculated for all patients. RESULTS: Of the 44 patients, 36 (82%) exhibited enhancement on LGE images. Mean QALE score of AL (7.9 ± 6) and ATTR (10.5 ± 5) patients were similar (p = 0.6). Myocardial native T2 was significantly (p < 0.0001) higher in AL (63.2 ± 4.7 ms) than in ATTR (56.2 ± 3.1 ms) patients, and both higher (p < 0.001) than healthy subjects (51.1 ± 3.1 ms). Myocardial native T2 was highly correlated with myocardial native T1 (Spearman's rho = 0.79; p < 0.001) and exhibited higher diagnostic performance than T1 to separate AL and ATTR patients: the area under curve (AUC) of T2 was 0.94 (95% CI: 0.86-1, p < 0.001) and the AUC of T1 was 0.77 (95% CI: 0.62-0.91, p = 0.03). Myocardial native T2 did not impact overall survival in patients (HR 1.03 (0.94-1.12); p = 0.53) in contrast to ECV that was the best predictor of outcome (HR 1.66 per 0.1 increase in ECV (1.24-2.22); p = 0.0006). CONCLUSIONS: Myocardial native T2 significantly is increased in CA, especially in AL patients in comparison to ATTR patients. Myocardial native T2 does not impact survival in CA patients in contrast to ECV that was the best predictor of outcome. TRIAL REGISTRATION: Trial Registration and unique number: CNIL cardio 1778041. Date of registration: 20 December 2012.


Assuntos
Neuropatias Amiloides Familiares/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Miocárdio/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Neuropatias Amiloides Familiares/patologia , Cardiomiopatias/patologia , Meios de Contraste/administração & dosagem , Diagnóstico Diferencial , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Reprodutibilidade dos Testes , Estudos Retrospectivos
10.
Curr Hematol Malig Rep ; 13(3): 212-219, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29951831

RESUMO

PURPOSE OF REVIEW: Light-chain-associated (AL) amyloidosis is a rare disease with a poor prognosis. However, we have made recent strides in more accurate diagnosis and effective treatment. Here, we discuss the most recent updates and advancements during the past year in the diagnosis, prognostication, and management of AL amyloidosis both in the upfront and relapsed setting. RECENT FINDINGS: New imaging modalities, such as cardiac magnetic resonance (CMR) and use of fluorine-labeled radiotracers, are emerging as an important diagnostic tool in conjunction with biomarkers in the diagnosis, prognosis, and monitoring of the effects of therapy. In addition, ongoing evaluation of plasma cell-directed therapeutics, including daratumumab, pomalidomide, and ixazomib, as well as promising targeted novel therapies, such as the monoclonal antibody NEOD001, are in development. In conclusion, incorporating the use of plasma cell-directed therapy and novel agents targeting the amyloid deposits itself hold enormous potential in achieving improved outcomes in AL amyloidosis.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Compostos de Boro/uso terapêutico , Glicina/análogos & derivados , Amiloidose de Cadeia Leve de Imunoglobulina , Imagem por Ressonância Magnética , Talidomida/análogos & derivados , Glicina/uso terapêutico , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/tratamento farmacológico , Prognóstico , Talidomida/uso terapêutico
11.
Amyloid ; 25(2): 109-114, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29706127

RESUMO

Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using 18F-florbetapir. We hypothesize that combining whole body PET/CT with 18F-Florbetapir can be useful to characterize the burden of the disease. We included 25 patients, 13 of them with different types of amyloidosis, and 12 with Alzheimer's disease as controls. Target-to-background ratio was computed for multiple organ using maximum standardized uptake values. Organ involvement was described (standardized techniques versus PET) according to different kinds of amyloidosis showing promising results in AA and AL types. Heart involvement showed poorer results when compared to tongue, lung or thyroid gland. Multiple organ involvement in patients previously labelled as having negative organ affectation could be identified. This is the first study to evaluate the utility of 18F-florbetapir in the assessment of the global extension of disease. Our results show that this technique is useful for its diagnosis.


Assuntos
Amiloidose/diagnóstico por imagem , Compostos de Anilina/análise , Etilenoglicóis/análise , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Idoso , Amiloidose Familiar/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
12.
J Cardiovasc Magn Reson ; 20(1): 2, 2018 01 03.
Artigo em Inglês | MEDLINE | ID: mdl-29298704

RESUMO

BACKGROUND: Cardiac impairment is associated with high morbidity and mortality in immunoglobulin light chain (AL) type amyloidosis, for which early identification and risk stratification is vital. For myocardial tissue characterization, late gadolinium enhancement (LGE) is a classic and most commonly performed cardiovascular magnetic resonance (CMR) parameter. T1 mapping with native T1 and extracellular volume (ECV) are recently developed quantitative parameters. We aimed to investigate the prognostic value of native T1, ECV and LGE in patients with AL amyloidosis. METHODS: Eighty-two patients (55.5 ± 8.5 years; 52 M) and 20 healthy subjects (53.2 ± 11.7 years; 10 M) were prospectively recruited. All subjects underwent CMR with LGE imaging and T1 mapping using a Modified Look-Locker Inversion-recovery (MOLLI) sequence on a 3 T scanner. Native T1 and ECV were measured semi-automatically using a dedicated CMR software. The left ventricular (LV) LGE pattern was classified as none, patchy, and global groups. Global LGE was considered when there was diffuse, transmural LGE in more than half of the short axis images. Follow-up was performed for all-cause mortality using Cox proportional hazards regression analysis and Kaplan-Meier survival curves. RESULTS: The patients demonstrated an increase in native T1 (1438 ± 120 ms vs. 1283 ± 46 ms, P = 0.001) and ECV (43.9 ± 10.9% vs. 27.0 ± 1.7%, P = 0.001) compared to healthy controls. Native T1, ECV and LGE showed significant correlation with Mayo Stage, and ECV and LGE showed significant correlation with echocardiographic E/E' and LV ejection fraction. During the follow-up for a median time of 8 months, 21 deaths occurred. ECV ≥ 44.0% (hazard ratio [HR] 7.249, 95% confidence interval (CI) 1.751-13.179, P = 0.002) and global LGE (HR 4.804, 95% CI 1.971-12.926, P = 0.001) were independently prognostic for mortality over other clinical and imaging parameters. In subgroups with the same LGE pattern, ECV ≥ 44.0% remained prognostic (log rank P = 0.029). Median native T1 (1456 ms) was not prognostic for mortality (Tarone-Ware, P = 0.069). CONCLUSIONS: During a short-term follow-up, both ECV and LGE are independently prognostic for mortality in AL amyloidosis. In patients with a similar LGE pattern, ECV remained prognostic. Native T1 was not found to be a prognostic factor.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Meios de Contraste/administração & dosagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Volume Sistólico , Função Ventricular Esquerda , Adulto , Cardiomiopatias/mortalidade , Cardiomiopatias/fisiopatologia , Estudos de Casos e Controles , Causas de Morte , China , Feminino , Humanos , Interpretação de Imagem Assistida por Computador , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Prospectivos , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Fatores de Tempo
13.
Amyloid ; 25(4): 247-252, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30661419

RESUMO

18F-florbetapir is a promising tracer in amyloidosis. This study evaluates its use in patients with systemic AL amyloidosis (AL) before and after treatment as well as its serial utility in monitoring. Fifteen AL patients with cardiac involvement underwent 18F-florbetapir PET imaging and three patients underwent repeat imaging after chemotherapy. All patients had demonstrable cardiac uptake with 18F-florbetapir. Cardiac uptake appeared greater in chemotherapy-naïve vs. chemotherapy-established AL patients median (left ventricular retention index 0.21 vs. 0.14 min-1, respectively) and greater in patients that had not achieved at least a partial haematological response (left ventricular retention index 0.2 vs. 0.14 min-1, respectively). There was no interval difference in cardiac uptake and no correlation in cardiac uptake with cardiac biomarkers or serum free light chains. This is the largest study of 18F-florbetapir in patients with AL amyloidosis. It is the first study to include patients prior to starting chemotherapy and uniquely includes patients who underwent repeat imaging after chemotherapy. All patients had cardiac uptake with 18F-florbetapir, regardless of haematological or NT-proBNP response to chemotherapy. There was a suggestion that treatment-naïve patients may have higher cardiac uptake. Larger studies are required to establish the role of this tracer in screening patients with amyloidosis for cardiac involvement, discriminating between ATTR and AL amyloidosis, and in disease monitoring.


Assuntos
Compostos de Anilina/metabolismo , Etilenoglicóis/metabolismo , Cardiopatias/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Idoso , Feminino , Seguimentos , Cardiopatias/metabolismo , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/metabolismo , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Prognóstico , Estudos Prospectivos
14.
Amyloid ; 25(1): 18-25, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29260587

RESUMO

OBJECTIVES: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. METHODS: We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN. The prognostic value of these parameters was analysed in the AL amyloidosis group. RESULTS: Free-wall right ventricular longitudinal strain (FWRVLS) worse than -21.2% discriminates AL amyloidosis [area under the curve (AUC) = 0.744)] from patients with IWT due to other aetiologies. In AL amyloidosis, FWRVLS is the strongest echocardiographic prognostic marker with AUC =0.722 and -16.5% as the optimal cut-off value, beyond which overall mortality increases significantly. It is also the only independent echocardiographic predictor of overall mortality (HR =1.113; 95%CI 1.029-1.204; p = .007), even when adjusted to the Mayo stage and global left ventricular longitudinal strain. CONCLUSIONS: FWRVLS should be considered in the diagnostic and prognostic workup in light-chain amyloidosis.


Assuntos
Cardiomiopatias , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Hipertensão , Amiloidose de Cadeia Leve de Imunoglobulina , Idoso , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/etiologia , Cardiomiopatias/mortalidade , Intervalo Livre de Doença , Feminino , Humanos , Hipertensão/diagnóstico por imagem , Hipertensão/etiologia , Hipertensão/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
15.
Circ J ; 82(3): 739-746, 2018 02 23.
Artigo em Inglês | MEDLINE | ID: mdl-29093431

RESUMO

BACKGROUND: Late gadolinium enhancement (LGE) pattern is a powerful imaging biomarker for prognosis of cardiac amyloidosis. It is unknown if the query amyloid late enhancement (QALE) score in light-chain (AL) amyloidosis could provide increased prognostic value compared with LGE pattern.Methods and Results:Seventy-eight consecutive patients with AL amyloidosis underwent contrast-enhanced cardiovascular magnetic resonance imaging. Patients with cardiac involvement were grouped by LGE pattern and analyzed using QALE score. Receiver operating characteristic curve was used to identify the optimal cut-off for QALE score in predicting all-cause mortality. Survival of these patients was analyzed with the Kaplan-Meier method and multivariate Cox regression. During a median follow-up of 34 months, 53 of 78 patients died. The optimal cut-off for QALE score to predict mortality at 12-month follow-up was 9.0. On multivariate Cox analysis, QALE score ≥9 (HR, 5.997; 95% CI: 2.665-13.497; P<0.001) and log N-terminal pro-brain natriuretic peptide (HR, 1.525; 95% CI: 1.112-2.092; P=0.009) were the only 2 independent predictors of all-cause mortality. On Kaplan-Meier analysis, patients with subendocardial LGE can be further risk stratified using QALE score ≥9. CONCLUSIONS: The QALE scoring system provides powerful independent prognostic value in AL cardiac amyloidosis. QALE score ≥9 has added value to differentiate prognosis in AL amyloidosis patients with a subendocardial LGE pattern.


Assuntos
Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Imagem por Ressonância Magnética/métodos , Idoso , Feminino , Gadolínio , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Peptídeo Natriurético Encefálico/análise , Fragmentos de Peptídeos/análise , Valor Preditivo dos Testes , Prognóstico , Curva ROC , Medição de Risco , Análise de Sobrevida
19.
J Cardiol ; 71(4): 419-427, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29153741

RESUMO

BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography. METHODS: A total of 77 patients (67±10 years, 60% men) with confirmed AL and 39 healthy controls were included. All standard 2D echocardiographic and 3D-LA parameters were obtained. RESULTS: Fourteen patients (18%) were in Mayo Clinic (MC) stage I, 30 (39%) in stage II, and 33 (43%) in stage III at initial evaluation. There was no significant difference among the MC stages groups in terms of age, gender, or cardiovascular risk factors. As compared to patients in MC II and MC I, those in MC III had significantly larger indexed 3D-LA volumes (MCIII: 46±15mL/m2, MC II: 38±12mL/m2, and MC I: 23±9mL/m2, p<0.0001), lower 3D-LA total emptying fraction (3D-tLAEF) (21±13% vs. 31±15% vs. 43±7%, respectively, p<0.0001), and worse 3D peak atrial longitudinal strain (3D-PALS) (11±9% vs. 18±13% vs. 20±7%, respectively, p=0.007). Two-year survival was significantly lower in patients with 3D-tLAEF <+34% (p=0.003) and in those with 3D-PALS <+14% (p=0.034). Both parameters provided incremental prognostic value over maximal LA volume in multivariate analysis. CONCLUSION: Functional LA parameters are progressively altered in AL patients according to the MC stage. A decrease in 3D-PALS is associated with worse outcome, independently of LA volume.


Assuntos
Ecocardiografia Tridimensional/métodos , Ecocardiografia/métodos , Átrios do Coração/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Idoso , Função do Átrio Esquerdo/fisiologia , Feminino , Átrios do Coração/fisiopatologia , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico
20.
J Nucl Cardiol ; 25(5): 1559-1567, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-28176254

RESUMO

BACKGROUND: Non-invasive imaging to diagnose and quantify amyloid load, progression, and response to treatment are central for the care of patients with cardiac amyloidosis. 18Fluorine-labeled sodium fluoride (18F-NaF) is a widely available radioisotope and PET imaging allows for absolute quantification of tracer uptake. METHODS: Patients with biopsy-proven transthyretin (ATTR-CA) and light-chain cardiac amyloidosis (AL) (3 ATTRwt, 2 ATTRV122I, 2 AL) and controls (n = 5), underwent 18F-NaF PET imaging. Scans were assessed visually for radiotracer uptake and analyzed using standard uptake values in the entire myocardium (SUVm) and in a 17-segment cardiac model. Wilcoxon rank-sum tests were used for statistical analyses. RESULTS: Qualitative 18F-NaF uptake was absent in controls and AL patients. There was qualitative 18F-NaF uptake in ATTR-CA patients, with slightly increased uptake in wild-type patients. SUVm for controls and AL patients overlapped at 0.8(0.4-0.9) and 0.95(0.9-1.0), respectively (P = 0.434). At 1.5(1.4-1.7), SUVm for ATTR-CA patients was ≈1.5*SUVm of controls (P = 0.012) and AL patients (P = 0.078). While there was diffuse 18F-NaF myocardial in ATTR-CA patients, the degree of uptake varied according to cardiac segment. CONCLUSION: 18F-NaF PET effectively imaged and differentiated ATTR-CA patients. Semi-automatic software enabled quantification of radiotracer uptake and regional distribution. 18F-NaF PET may be useful for disease monitoring and localizing amyloid deposition in ATTR-CA patients.


Assuntos
Neuropatias Amiloides Familiares/diagnóstico por imagem , Tomografia por Emissão de Pósitrons/métodos , Idoso , Idoso de 80 Anos ou mais , Biomarcadores , Cardiomiopatias/diagnóstico por imagem , Feminino , Radioisótopos de Flúor , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Estudos Prospectivos , Fluoreto de Sódio
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