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1.
Transplantation ; 104(8): 1703-1711, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32732850

RESUMO

BACKGROUND: There are limited data on the outcome of transplant recipients with familial Mediterranean fever (FMF)-associated AA amyloidosis. The aim of the present study is to evaluate demographic, clinical, laboratory, and prognostic characteristics and outcome measures of these patients. METHODS: Eighty-one renal transplant recipients with FMF-associated AA amyloidosis (group 1) and propensity score-matched transplant recipients (group 2, n = 81) with nonamyloidosis etiologies were evaluated in this retrospective, multicenter study. Recurrence of AA amyloidosis was diagnosed in 21 patients (group 1a), and their features were compared with 21 propensity score-matched recipients with FMF amyloidosis with no laboratory signs of recurrence (group 1b). RESULTS: The risk of overall allograft loss was higher in group 1 compared with group 2 (25 [30.9%] versus 12 [14.8%]; P = 0.015 [hazard ratio, 2.083; 95% confidence interval, 1.126-3.856]). Patients in group 1 were characterized by an increased risk of mortality compared with group 2 (11 [13.6%] versus 0%; P = 0.001 [hazard ratio, 1.136; 95% confidence interval, 1.058-1.207]). Kaplan-Meier analysis revealed that 5- and 10-year patient survival rates in group 1 (92.5% and 70.4%) were significantly lower than in group 2 (100% and 100%; P = 0.026 and P = 0.023, respectively). Although not reaching significance, overall, 5- and 10-year graft survival rates (57.1%, 94.7%, and 53.8%, respectively) in group 1a were worse than in group 1b (76.2%, 95%, and 77.8%, respectively; P = 0.19, P = 0.95, and P = 0.27, respectively). CONCLUSIONS: AA amyloidosis is associated with higher risk of mortality after kidney transplantation. Inflammatory indicators should be monitored closely, and persistent high levels of acute-phase reactants should raise concerns about amyloid recurrence in allograft.


Assuntos
Amiloidose/cirurgia , Febre Familiar do Mediterrâneo/complicações , Rejeição de Enxerto/mortalidade , Falência Renal Crônica/cirurgia , Transplante de Rim/efeitos adversos , Adulto , Aloenxertos/imunologia , Aloenxertos/patologia , Amiloidose/imunologia , Amiloidose/mortalidade , Amiloidose/patologia , Biópsia , Febre Familiar do Mediterrâneo/imunologia , Febre Familiar do Mediterrâneo/mortalidade , Febre Familiar do Mediterrâneo/cirurgia , Feminino , Seguimentos , Rejeição de Enxerto/imunologia , Rejeição de Enxerto/patologia , Sobrevivência de Enxerto/imunologia , Humanos , Estimativa de Kaplan-Meier , Rim/imunologia , Rim/patologia , Falência Renal Crônica/imunologia , Falência Renal Crônica/mortalidade , Falência Renal Crônica/patologia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Proteína Amiloide A Sérica/imunologia , Proteína Amiloide A Sérica/metabolismo , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
2.
Acta Haematol ; 143(4): 352-364, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32535598

RESUMO

Amyloidosis comprises a diverse group of diseases characterized by misfolding of precursor proteins which eventually form amyloid aggregates and preceding intermediaries, which are deposited in target tissues causing progressive organ damage. In all forms of amyloidosis, vital organs may fail; depending on the specific amyloidosis type, this may occur rapidly or progress slowly. Beyond therapies to reduce the precursor protein (chemotherapy for light chain [AL] amyloidosis, anti-inflammatory therapy in serum A amyloid-osis [AA], and antisense RNA therapy in transthyretin amyloidosis [ATTR]), organ transplantation may also be a means to reduce amyloidogenic protein, e.g., in types of amyloid-osis in which the variant precursor is produced by the liver. Heart transplantation is a life-saving approach to the treatment of patients with advanced cardiac amyloidosis; however, amyloidosis may still be considered a contraindication to the procedure despite data supporting improved outcomes, similar to patients with other indications. Kidney transplantation is associated with particularly favorable outcomes in patients with amyloidosis, especially if the precursor protein has been eliminated. Overall, outcomes of solid organ transplantation are improving, but more data are needed to refine the selection criteria and the timing for organ transplantation, which should be performed in highly experienced centers involving multidisciplinary teams with close patient follow-up to detect amyloid recurrence.


Assuntos
Amiloidose/terapia , Transplante de Órgãos , Amiloidose/diagnóstico , Amiloidose/etiologia , Amiloidose/mortalidade , Gerenciamento Clínico , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico , Amiloidose de Cadeia Leve de Imunoglobulina/etiologia , Amiloidose de Cadeia Leve de Imunoglobulina/terapia , Transplante de Órgãos/efeitos adversos , Transplante de Órgãos/métodos , Resultado do Tratamento
4.
Can J Cardiol ; 36(3): 432-440, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32145870

RESUMO

BACKGROUND: Right heart function is an important prognostic determinant in cardiac amyloidosis. In this study we characterized serial changes in right and left heart function and evaluated their prognostic significance. METHODS: Cardiac amyloidosis patients with baseline and follow-up echocardiograms were included. Right and left heart function measured at baseline, 1 year, and most recent follow-up were compared and correlated with all-cause mortality or cardiovascular hospitalization. RESULTS: Ninety-three patients were included; 36 (39%) with light chain amyloidosis and 57 (61%) with transthyretin amyloidosis. Among measures of right heart function for the study population and light chain and transthyretin amyloidosis subtypes, only absolute right ventricular (RV) free wall longitudinal strain (FWLS) changed significantly from baseline to 1 year and most recent follow-up echocardiogram. After a median of 26 months (range, 14-35 months), 21 (22%) patients died and 17 (18%) had a cardiovascular hospitalization. Baseline RV FWLS was significantly associated with the primary endpoint (hazard ratio, 1.2 per % change; 95% confidence interval, 0.8-2.6; P < 0.01), whereas change from baseline to 1 year was not for any measure of right heart function. Baseline left ventricular (LV) global longitudinal strain (GLS) and 1 year change were significantly associated with the primary end point. Change in RV FWLS at 1 year was significantly correlated with baseline LV GLS (r = 0.68; P = 0.01) and change at 1 year follow-up (r = 0.72; P < 0.01). CONCLUSIONS: In cardiac amyloidosis patients, baseline RV FWLS was associated with adverse outcomes whereas changes at follow-up was not. Change in RV FWLS was significantly correlated with baseline and follow-up change in LV GLS, possibly reflecting progressive biventricular amyloid deposition.


Assuntos
Amiloidose/mortalidade , Amiloidose/fisiopatologia , Cardiomiopatias/mortalidade , Cardiomiopatias/fisiopatologia , Idoso , Feminino , Testes de Função Cardíaca , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
5.
JACC Cardiovasc Imaging ; 13(1 Pt 1): 44-54, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31103587

RESUMO

OBJECTIVES: Because risk stratification data represents a key domain of biomarker validation, we compared associations between outcomes and various cardiovascular magnetic resonance (CMR) metrics quantifying myocardial fibrosis (MF) in noninfarcted myocardium: extracellular volume fraction (ECV), native T1, post-contrast T1, and partition coefficient. BACKGROUND: MF associates with vulnerability to adverse events (e.g., mortality and hospitalization for heart failure [HHF]), but investigators still debate its optimal measurement; most histological validation data show strongest ECV correlations with MF. METHODS: We enrolled 1,714 consecutive patients without amyloidosis or hypertrophic cardiomyopathy from a single CMR referral center serving an integrated healthcare network. We measured T1 (MOdified Look-Locker Inversion recovery [MOLLI]) in nonenhanced myocardium, averaged from 2 short-axis slices (basal and mid) before and 15 to 20 min after a gadolinium contrast bolus. We compared chi-square test values from CMR MF measures in univariable and multivariable Cox regression models. We assessed "dose-response" relationships in Kaplan-Meier curves using log-rank statistics for quartile strata. We also computed net reclassification improvement (NRI) and integrated discrimination improvement (IDI for Cox models with ECV vs. native T1). RESULTS: Over a median of 5.6 years, 374 events occurred after CMR (162 HHF events and 279 deaths, 67 with both). ECV yielded the best separation of Kaplan-Meier curves and the highest log-rank statistics. In univariable and multivariable models, ECV associated most strongly with outcomes, demonstrating the highest chi-square test values. Native T1 or post-contrast T1 did not associate with outcomes in the multivariable model. ECV provided added prognostic value to models with native T1, for example, in multivariable models IDI = 0.0037 (95% confidence interval [CI]: 0.0009 to 0.0071), p = 0.02; NRI = 0.151 (95% CI: 0.022 to 0.292), p = 0.04. CONCLUSIONS: Analogous to histological previously published validation data, ECV myocardial fibrosis measures exhibited more robust associations with outcomes than other surrogate CMR MF measures. Superior risk stratification by ECV supports claims that ECV optimally measures MF in noninfarcted myocardium.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatia Hipertrófica/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Miocárdio/patologia , Volume Sistólico , Função Ventricular Esquerda , Remodelação Ventricular , Idoso , Amiloidose/mortalidade , Amiloidose/patologia , Amiloidose/fisiopatologia , Cardiomiopatia Hipertrófica/mortalidade , Cardiomiopatia Hipertrófica/patologia , Cardiomiopatia Hipertrófica/fisiopatologia , Meios de Contraste/administração & dosagem , Progressão da Doença , Feminino , Fibrose , Gadolínio/administração & dosagem , Compostos Heterocíclicos/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Compostos Organometálicos/administração & dosagem , Valor Preditivo dos Testes , Prognóstico , Medição de Risco , Fatores de Risco
6.
Clin Res Cardiol ; 109(1): 78-88, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31134330

RESUMO

BACKGROUND: Cardiac amyloidosis (CA) is an underappreciated cause of morbidity and mortality. Light-chain (AL) and transthyretin (ATTR) amyloidosis have different disease trajectories. No data are available on subtype-specific modes of death (MOD) in patients with CA. METHODS AND RESULTS: We retrospectively investigated 66 with AL and 48 with wild-type ATTR amyloidosis (ATTRwt) from 2000 to 2018. ATTRwt differed from AL by age (74.6 ± 5.4 years vs. 63 ± 10.8 years), posterior wall thickness (16.8 ± 3.3 mm vs. 14.3 ± 2.2 mm), left ventricular mass index (180.7 ± 63.2 g/m2 vs. 133.5 ± 42.2 g/m2), and the proportions of male gender (91.7% vs. 59.1%), atrial enlargement (92% vs. 68.2%) and atrial fibrillation (50% vs. 12.1%). In AL NYHA Functional Class and proteinuria (72.7% vs. 39.6%) were greater; mean arterial pressure (84.4 ± 13.5 mmHg vs. 90.0 ± 11.3 mmHg) was lower. Unadjusted 5-year mortality rate was 65% in AL-CA vs. 44% in the ATTRwt group. Individuals with AL-CA were 2.28 times ([95%CI 1.27-4.10]; p = 0.006) more likely to die than were individuals with ATTRwt-CA. Information on MOD was available in 56 (94.9%) of 59 deceased patients. MOD was cardiovascular in 40 (66.8%) and non-cardiovascular in 16 (27.1%) patients. Cardiovascular [28 (68.3%) vs. 13 (80%)] death events were distributed equally between AL and ATTRwt (p = 0.51). CONCLUSION: Our data indicate no differences in MOD between patients with AL and ATTRwt cardiac amyloidosis despite significant differences in clinical presentation and disease progression. Cardiovascular events account for more than two-thirds of fatal casualties in both groups.


Assuntos
Amiloidose/mortalidade , Cardiomiopatias/mortalidade , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Idoso , Idoso de 80 Anos ou mais , Amiloidose/fisiopatologia , Fibrilação Atrial/epidemiologia , Cardiomiopatias/fisiopatologia , Estudos de Coortes , Progressão da Doença , Feminino , Seguimentos , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pré-Albumina/metabolismo , Estudos Retrospectivos
10.
Am J Transplant ; 19(10): 2900-2909, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31152491

RESUMO

This study sought to retrospectively investigate the outcomes of patients with light-chain amyloidosis (AL) with advanced cardiac involvement who were treated with a strategy of heart transplantation (HT) followed by delayed autologous stem cell transplantation (ASCT) at 1-year posttransplant. Patients with AL amyloidosis with substantial cardiac involvement have traditionally had very poor survival (eg, several months). A few select centers have reported their outcomes for HT followed by a strategy of early ASCT (ie, 6 months) for CA. The outcomes of patients undergoing a delayed strategy have not been reported. All patients with AL amyloidosis at a single institution undergoing evaluation for HT from 2004-2018 were included. Retrospective analyses were performed. Sixteen patients underwent HT (including two combined heart-kidney transplant) for AL amyloidosis. ASCT was performed in a total of nine patients to date at a median 13.5 months (12.8-32.9 months) post-HT. Survival was 87.5% at 1 year and 76.6% at 5 years, comparable to institutional outcomes for nonamyloid HT recipients. In addition to these 16 patients, two patients underwent combined heart-lung transplantation. A strategy of delayed ASCT 1-year post-HT for patients with AL amyloidosis is feasible, safe, and associated with comparable outcomes to those undergoing an earlier ASCT strategy.


Assuntos
Amiloidose/mortalidade , Cardiomiopatias/mortalidade , Transplante de Coração/mortalidade , Transplante de Células-Tronco/mortalidade , Tempo para o Tratamento/estatística & dados numéricos , Adulto , Idoso , Amiloidose/complicações , Amiloidose/patologia , Amiloidose/terapia , Cardiomiopatias/complicações , Cardiomiopatias/patologia , Cardiomiopatias/terapia , Estudos de Casos e Controles , Terapia Combinada , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Transplante Autólogo , Resultado do Tratamento
11.
Avian Dis ; 63(3): 446-451, 2019 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-31967427

RESUMO

Twelve chukar partridges (Alectoris chukar) from a farm experiencing poor uniformity and increased mortality of up to 65% were submitted for diagnosis. Several birds had mild to moderate multifocal white foci or multifocal petechial hemorrhages throughout the liver. Livers and spleens of older birds were moderate to severely diffusely enlarged. In addition, some birds had caseous cores mixed with blood within the ceca as well as segmentally thickened cecal walls. Histopathology showed acute, multifocal, severe, often coalescing foci of necrosis with accumulation of fibrin and/or fibrinosuppurative inflammation in livers and spleens. Scattered within exudate were protozoa that were spherical or round and measured 12-20 µm in diameter. In the ceca, acute necrosis of the mucosa was observed, often with ulceration and fibrinosuppurative inflammation. Immunohistochemistry using an antiserum against Tritrichomonas foetus revealed round protozoa in ceca, small intestines, liver, spleen, and lung. Quantitative PCR to detect DNA of Histomonas meleagridis was negative. Non-species-specific PCRs amplifying the partial rDNA, the internal transcribed spacer (ITS) region, and the partial beta-tubulin gene yielded products of the expected size. Sequences of the PCR products had the highest homology to sequences of Tetratrichomonas gallinarum and less homology to sequences of H. meleagridis. In addition there was accumulation of amyloid in the space of Disse in the liver, splenic sinuses, and walls of the blood vessels. The typhlohepatitis and other inflammatory processes that were diagnosed might be the underlying cause of the amyloidosis. Other findings were clusters of Clostridium perfringens associated with the lesions in the ceca; multifocal granulomas in the lungs, occasionally associated with fungal hyphae; hyperkeratosis associated with bacteria and Candida sp. cells in the crop; mild infection of the bursal mucosa with Cryptosporidium.


Assuntos
Amiloidose/veterinária , Doenças das Aves/mortalidade , Galliformes , Hepatite Animal/mortalidade , Infecções Protozoárias em Animais/mortalidade , Trichomonadida/isolamento & purificação , Amiloidose/mortalidade , Amiloidose/parasitologia , Animais , Doenças das Aves/parasitologia , California/epidemiologia , Hepatite Animal/parasitologia , Infecções Protozoárias em Animais/parasitologia
12.
Cancer ; 125(2): 185-193, 2019 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-30480777

RESUMO

High-dose therapy (HDT) and autologous stem cell transplantation (ASCT) are established components in the treatment of multiple myeloma; however, undergoing transplantation usually requires hematopoietic support, which poses a challenge among patients who are unwilling to receive blood products. Most transplant centers decline HDT/ASCT to these patients because of safety concerns. Here, the authors' institutional data on safety, engraftment parameters, and survival outcomes after bloodless ASCT (BL-ASCT) are examined among patients with myeloma. This retrospective case-control study included patients who underwent BL-ASCT and Transfusion-supported ASCT (TS-ASCT) at Emory University Hospital between August 2006 and August 2016. In total, 24 patients who underwent BL-ASCT and 70 who underwent TS-ASCT were included. The median time for neutrophil engraftment, platelet engraftment and the median length of hospital stay all were equivalent for both groups. There were no transplant-related cardiovascular complications or mortality in either the BL-ASCT group or the TS-ASCT group. The median progression-free survival was 36 months and 44 months in the BL-ASCT and TS-ASCT groups, respectively (P = .277), and the median OS was not reached in either group at a median follow-up of 59 months after ASCT (P = .627). There was no transplant-related mortality at the 100-day or 1-year mark in either group. BL-ASCT is safe and feasible; transplant-related mortality, cardiovascular and hematologic complications are similar to those associated with TS-ASCT. Furthermore, BL-ASCT can yield similar engraftment and survival parameters comparable to those observed with TS-ASCT.


Assuntos
Mieloma Múltiplo/mortalidade , Mieloma Múltiplo/terapia , Transplante de Células-Tronco/métodos , Adulto , Idoso , Amiloidose/mortalidade , Amiloidose/terapia , Transfusão de Sangue , Doenças Cardiovasculares/etiologia , Estudos de Casos e Controles , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Transplante de Células-Tronco/efeitos adversos , Transplante de Células-Tronco/mortalidade , Análise de Sobrevida , Transplante Autólogo/efeitos adversos , Transplante Autólogo/métodos , Transplante Autólogo/mortalidade , Resultado do Tratamento
13.
Intern Med ; 58(4): 557-561, 2019 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-30333399

RESUMO

We herein report a case involving a 64-year-old Japanese woman with a pulmonary Mycobacterium abscessus infection complicated by reactive AA amyloidosis, which, to our knowledge, has not been reported to date. The patient underwent gastrointestinal endoscopy for diarrhea during the treatment of pulmonary M. abscessus infection and was diagnosed with AA amyloidosis according to the histopathological findings from the endoscopic specimen. She died four months later. The prognosis of AA amyloidosis associated with pulmonary M. abscessus infection may be very poor, and physicians should pay attention to this rare condition when difficult-to-treat diarrhea occurs in patients with pulmonary M. abscessus infection.


Assuntos
Amiloidose/etiologia , Amiloidose/mortalidade , Infecções por Mycobacterium não Tuberculosas/complicações , Infecções por Mycobacterium não Tuberculosas/mortalidade , Infecções Respiratórias/complicações , Infecções Respiratórias/mortalidade , Amiloidose/complicações , Amiloidose/terapia , Grupo com Ancestrais do Continente Asiático , Evolução Fatal , Feminino , Humanos , Pessoa de Meia-Idade , Infecções por Mycobacterium não Tuberculosas/terapia , Infecções Respiratórias/terapia
15.
Kidney Int ; 95(2): 405-411, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30580886

RESUMO

Therapies for AL amyloidosis have dramatically improved, leading to longer patient survival; however, more AL amyloidosis patients are reaching end-stage renal disease (ESRD). There are no clear guidelines regarding eligibility for kidney transplantation in patients with AL amyloidosis, and data on outcomes are limited. We evaluated the clinical and laboratory data of 49 patients who were followed in the Amyloidosis Center at Boston University and underwent kidney transplantation at a center in the United States between 1987-2017. During a median follow-up of 7.2 years (range 0-19), the median patient survival from diagnosis was 15.4 years, and from kidney transplantation was 10.5 years. One, three, and five-year graft survival were 94%, 89%, and 81%, respectively. Patients with hematologic complete response or very good partial response prior to kidney transplantation had significantly better patient survival than patients with partial response or no response, and the median time to graft loss was 10.4 years versus 5.5 years, respectively. This is the largest published series of kidney transplantation in patients with AL amyloidosis, suggesting that kidney transplantation can have a good outcome in carefully selected patients, particularly in those who have achieved a complete response or very good partial response at the time of kidney transplantation.


Assuntos
Amiloidose/complicações , Falência Renal Crônica/cirurgia , Transplante de Rim/estatística & dados numéricos , Seleção de Pacientes , Adulto , Idoso , Amiloidose/mortalidade , Amiloidose/cirurgia , Feminino , Seguimentos , Sobrevivência de Enxerto , Humanos , Estimativa de Kaplan-Meier , Falência Renal Crônica/etiologia , Falência Renal Crônica/mortalidade , Transplante de Rim/normas , Masculino , Pessoa de Meia-Idade , Guias de Prática Clínica como Assunto , Recidiva , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Estados Unidos/epidemiologia
16.
Amyloid ; 25(3): 167-173, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30193539

RESUMO

INTRODUCTION: Cardiopulmonary exercise testing (CPET) has repeatedly been reported to reliably predict adverse outcomes in different forms of heart failure. However, it has not been elucidated in detail in cardiac amyloidosis (CA). Therefore, we evaluated the predictive value of CPET parameters in patients with CA regarding disease severity and prediction of mortality. METHODS: Twenty-seven consecutive patients with CA were assessed noninvasively, including electrocardiography, echocardiography, CPET, and laboratory tests. Clinical data were correlated with CPET findings. Univariate and multivariate analyses were performed to evaluate predictors of mortality. RESULTS: Within median follow-up period of 38 (IQR 43) months 19 (70%) deaths occurred. Patient initially presented with signs and symptoms of congestive heart failure NYHA 3 (IQR 1), reduced exercise capacity (peak V'O2 15.2 mL/kg body weight) and inefficient ventilation in CPET (V'E/V'CO2 slope (30 (IQR 3)), markedly elevated cardiac biomarkers (NT-proBNP 1791 (IQR 3249) ng/mL) and echocardiographic signs of morphological (septum thickness 18 (IQR 6) mm) and functional cardiac involvement (TAPSE 19 (IQR 8) mm). Patients with peak V'O2 below median value presented with significantly longer QTc interval when compared to patients with peak V'O2 above the median. Further these patients tend to have more pronounced impairment of longitudinal function as indicated by lower MAPSE, TAPSE, and elevation of cardiac biomarkers. Multivariate analysis revealed peak V'O2 slope as the only independent predictor of survival. CONCLUSIONS: We identified reduced peak V'O2 as an independent predictor of mortality in patients with cardiac involvement in different forms of systemic amyloidosis.


Assuntos
Amiloidose/mortalidade , Amiloidose/fisiopatologia , Cardiomiopatias/mortalidade , Cardiomiopatias/fisiopatologia , Consumo de Oxigênio/fisiologia , Ecocardiografia , Humanos
17.
JAMA Cardiol ; 3(9): 865-870, 2018 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-30046835

RESUMO

Importance: Cardiac amyloidosis is an underdiagnosed disease and is highly fatal when untreated. Early diagnosis and treatment with the emerging novel therapies significantly improve survival. A comprehensive analysis of amyloidosis-related mortality is critical to appreciate the nature and distribution of underdiagnosis and improve disease detection. Objective: To evaluate the temporal and regional trends in age-adjusted amyloidosis-related mortality among men and women of various races/ethnicities in the United States. Design, Setting, and Participants: In this observational cohort study, death certificate information from the Centers for Disease Control and Prevention's Wide-ranging ONline Data for Epidemiologic Research database and the National Vital Statistics System from 1979 to 2015 was analyzed. A total of 30 764 individuals in the United States with amyloidosis listed as the underlying cause of death and 26 591 individuals with amyloidosis listed as a contributing cause of death were analyzed. Exposures: Region of residence. Main Outcomes and Measures: Age-adjusted mortality rate from amyloidosis per 1 000 000 population stratified by year, sex, race/ethnicity, and state and county of residence. Results: Of the 30 764 individuals with amyloidosis listed as the underlying cause of death, 17 421 (56.6%) were men and 27 312 (88.8%) were 55 years or older. From 1979 to 2015, the reported overall mean age-adjusted mortality rate from amyloidosis as the underlying cause of death doubled from 1.77 to 3.96 per 1 000 000 population (2.32 to 5.43 in men and 1.35 to 2.80 in women). Black men had the highest mortality rate (12.36 per 1 000 000), followed by black women (6.48 per 1 000 000). Amyloidosis contributed to age-adjusted mortality rates as high as 31.73 per 1 000 000 in certain counties. Most southern states reported the lowest US mortality rates despite having the highest proportions of black individuals. Conclusions and Relevance: The increased reported mortality over time and in proximity to amyloidosis centers more likely reflects an overall increase in disease diagnosis rather than increased lethality. The reported amyloidosis mortality is highly variable in different US regions. The lack of higher reported mortality rates in states with a greater proportion of black residents suggests underdiagnosis of amyloidosis, including cardiac forms of the disease, in many areas of the United States. Better understanding of the determinants of geographic and racial disparity in the reporting of amyloidosis deaths are warranted.


Assuntos
Amiloidose/etnologia , Amiloidose/mortalidade , Cardiopatias/mortalidade , Adulto , Afro-Americanos/estatística & dados numéricos , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Estudos de Coortes , Atestado de Óbito , Feminino , Disparidades nos Níveis de Saúde , Cardiopatias/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Estados Unidos , Adulto Jovem
18.
J Am Coll Cardiol ; 71(25): 2919-2931, 2018 06 26.
Artigo em Inglês | MEDLINE | ID: mdl-29929616

RESUMO

BACKGROUND: Prognosis in light-chain (AL) and transthyretin (ATTR) amyloidosis is influenced by cardiac involvement. ATTR amyloidosis has better prognosis than AL amyloidosis despite more amyloid infiltration, suggesting additional mechanisms of damage in AL amyloidosis. OBJECTIVES: The aim of the study was to assess the presence and prognostic significance of myocardial edema in patients with amyloidosis. METHODS: The study recruited 286 patients: 100 with systemic AL amyloidosis, 163 with cardiac ATTR amyloidosis, 12 with suspected cardiac ATTR amyloidosis (grade 1 on 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid), 11 asymptomatic individuals with amyloidogenic TTR gene mutations, and 30 healthy volunteers. All subjects underwent cardiovascular magnetic resonance with T1 and T2 mapping and 16 underwent endomyocardial biopsy. RESULTS: Myocardial T2 was increased in amyloidosis with the degree of elevation being highest in untreated AL patients (untreated AL amyloidosis 56.6 ± 5.1 ms; treated AL amyloidosis 53.6 ± 3.9 ms; ATTR amyloidosis 54.2 ± 4.1 ms; each p < 0.01 compared with control subjects: 48.9 ± 2.0 ms). Left ventricular (LV) mass and extracellular volume fraction were higher in ATTR amyloidosis compared with AL amyloidosis while LV ejection fraction was lower (p < 0.001). Histological evidence of edema was present in 87.5% of biopsy samples ranging from 5% to 40% myocardial involvement. Using Cox regression models, myocardial T2 predicted death in AL amyloidosis (hazard ratio: 1.48; 95% confidence interval: 1.20 to 1.82) and remained significant after adjusting for extracellular volume fraction and N-terminal pro-B-type natriuretic peptide (hazard ratio: 1.32; 95% confidence interval: 1.05 to 1.67). CONCLUSIONS: Myocardial edema is present in cardiac amyloidosis by histology and cardiovascular magnetic resonance T2 mapping. T2 is higher in untreated AL amyloidosis compared with treated AL and ATTR amyloidosis, and is a predictor of prognosis in AL amyloidosis. This suggests mechanisms additional to amyloid infiltration contributing to mortality in amyloidosis.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Edema/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Amiloidose/genética , Amiloidose/mortalidade , Amiloidose/patologia , Cardiomiopatias/genética , Cardiomiopatias/mortalidade , Cardiomiopatias/patologia , Edema/mortalidade , Edema/patologia , Feminino , Humanos , Londres/epidemiologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Miocárdio/patologia , Pré-Albumina/genética
19.
Arch Cardiovasc Dis ; 111(10): 582-590, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29709420

RESUMO

BACKGROUND: The outcome of cardiac amyloidosis (CA) has been reported mainly in stable populations; limited data are available in patients referred for acute heart failure (AHF) to an intensive cardiac care unit (ICCU). AIMS: To address the characteristics and outcomes of patients with confirmed CA admitted to an ICCU for AHF and then to identify the predictors of evolution to cardiogenic shock. METHODS: All patients with CA referred to an ICCU for AHF between 2009 and 2015 were included. The clinical endpoint was 3-month death. Data from the population with cardiogenic shock, obtained in a stable haemodynamic state, were matched with data from a control group to determine predictors of evolution to cardiogenic shock. RESULTS: Among the 421 patients followed for CA in our expert centre, 46 patients (mean age: 64±14 years; 65% light-chain [AL] CA) were referred to the ICCU for AHF (n=26 with cardiogenic shock). At 3 months, death occurred in 24 (52%) patients, mostly in the cardiogenic shock group (n=21/26, 81%). Most deaths occurred 5 days [interquartile range 3-9 days] after catecholamine infusion and 50% occurred in patients aged<65 years. The majority of deaths were reported in patients with AL CA (n=19/24, 79%). Independent variables associated with in-hospital mortality were cardiogenic shock and uraemia level. N-terminal prohormone of B-type natriuretic peptide (NT-proBNP) concentration obtained in a stable haemodynamic state was the only predictor of short-term evolution to cardiogenic shock (odds ratio: 8.7, 95% confidence interval: 2.2-34.6), with an optimal cut-off of 4040pg/mL (sensitivity=92%; specificity=81%). CONCLUSIONS: The study confirms the dramatic mortality associated with CA when presenting as cardiogenic shock and underlines the limited efficiency of conventional treatments. Given the rapid occurrence of death in a young population, an alternative strategy to dobutamine support should be investigated in patients with elevated NT-proBNP concentration.


Assuntos
Amiloidose/complicações , Cardiomiopatias/complicações , Insuficiência Cardíaca/etiologia , Unidades de Terapia Intensiva , Admissão do Paciente , Choque Cardiogênico/etiologia , Doença Aguda , Idoso , Idoso de 80 Anos ou mais , Amiloidose/diagnóstico , Amiloidose/mortalidade , Amiloidose/terapia , Biomarcadores/sangue , Cardiomiopatias/diagnóstico , Cardiomiopatias/mortalidade , Cardiomiopatias/terapia , Distribuição de Qui-Quadrado , Progressão da Doença , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/terapia , Hemodinâmica , Mortalidade Hospitalar , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Peptídeo Natriurético Encefálico/sangue , Razão de Chances , Fragmentos de Peptídeos/sangue , Sistema de Registros , Fatores de Risco , Choque Cardiogênico/diagnóstico , Choque Cardiogênico/mortalidade , Choque Cardiogênico/terapia , Fatores de Tempo , Resultado do Tratamento
20.
J Thorac Cardiovasc Surg ; 156(1): 98-103, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29397971

RESUMO

BACKGROUND: Outcome data on aortic valve replacement in patients with amyloidosis are limited. To address this issue, we reviewed our experience of patients with amyloidosis who underwent aortic valve replacement. METHODS: We retrospectively reviewed the records of 16 patients with amyloidosis who underwent aortic valve replacement between May 2000 and February 2017. RESULTS: The cohort comprised 11 males (69%) and 5 females (31%). The median patient age was 76 years (interquartile range [IQR], 71-82 years), and Society of Thoracic Surgeons predicted rate of mortality was 5.0% (IQR, 2.4%-8.7%). Amyloidosis type was immunoglobulin light chain in 6 patients (38%), age-related in 6 (38%), and localized in 4 (25%). The operation was surgical aortic valve replacement in 11 patients (69%) and balloon-expandable transfemoral transcatheter aortic valve insertion in the other 5. There was no procedure-related stroke, need for new-onset dialysis or pacemaker, or death within 30 days of surgery. The median length of hospital stay was 1 day (IQR, 1-2 days) in the transcatheter valve insertion group and 6 days (IQR, 6-8 days) in the surgical group (P = .002). Follow-up was available for all patients at a median of 1.9 years (IQR, 1.2-4.8 years). During the follow-up period, there were 4 deaths, all occurring >1 year after surgery. CONCLUSIONS: Aortic valve replacement can be performed with low risk of operative morbidity and mortality in patients with amyloidosis. Transcatheter valve insertion has the advantage of reduced hospital length of stay. The 1-year survival is excellent.


Assuntos
Amiloidose/complicações , Valva Aórtica/cirurgia , Implante de Prótese Vascular , Cardiomiopatias/complicações , Doenças das Valvas Cardíacas/cirurgia , Substituição da Valva Aórtica Transcateter , Idoso , Idoso de 80 Anos ou mais , Amiloidose/mortalidade , Amiloidose/patologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/fisiopatologia , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/mortalidade , Cardiomiopatias/mortalidade , Cardiomiopatias/patologia , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/mortalidade , Hemodinâmica , Humanos , Tempo de Internação , Masculino , Miocárdio/patologia , Complicações Pós-Operatórias/mortalidade , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Substituição da Valva Aórtica Transcateter/efeitos adversos , Substituição da Valva Aórtica Transcateter/mortalidade , Resultado do Tratamento
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