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1.
Can J Cardiol ; 36(3): 384-395, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32145866

RESUMO

Light-chain (AL) amyloidosis is a systemic syndrome characterized by progressive organ dysfunction leading to organ failure and death. The heart is the most commonly involved organ and the leading determinant of short- and long-term survival. Pathogenic free light chains, fragments of intact immunoglobulins, are the amyloidogenic proteins and are secreted by clonal bone marrow plasma cells. The goal of therapy is to cut off the supply of these light chains to allow organ recovery. Therapies for AL amyloidosis are based on therapies used to treat multiple myeloma, which is a more common plasma cell disorder. However, because patients with AL amyloidosis have organ dysfunction, including multiorgan involvement, they generally have poor treatment tolerance and increased treatment toxicity. Unfortunately, the consequences of toxicity and difficulty in tolerating treatment may result in a fatal outcome. Therefore, treatment should balance the goal of achieving a rapid and meaningful reduction in the circulating light chains while maximizing patient safety. This approach is best achieved by a multidisciplinary approach involving related medical disciplines. This review describes the challenges of managing patients with AL amyloidosis and provides a guide for physicians with a specific focus on cardiac management. We address the role of autologous stem cell transplantation vs standard-intensity therapies in a risk-adapted approach and discuss the management of commonly encountered toxicities. Guidance on response assessment, including organ response, is provided. With expansion in treatment options, we anticipate continuous improvement in outcome for this disease. Nonetheless, early diagnosis remains the best approach to improve disease burden and outcome.

2.
Artigo em Inglês | MEDLINE | ID: mdl-32062791

RESUMO

PURPOSE: The Phase 3 ENDEAVOUR study evaluated revusiran, an investigational RNA interference therapeutic targeting hepatic transthyretin (TTR) production, for treating cardiomyopathy caused by hereditary transthyretin-mediated (hATTR) amyloidosis. METHODS: Patients with hATTR amyloidosis with cardiomyopathy were randomized 2:1 to receive subcutaneous daily revusiran 500 mg (n = 140) or placebo (n = 66) for 5 days over a week followed by weekly doses. Co-primary endpoints were 6-min walk test distance and serum TTR reduction. RESULTS: Revusiran treatment was stopped after a median of 6.71 months; the study Sponsor prematurely discontinued dosing due to an observed mortality imbalance between treatment arms. Eighteen (12.9%) patients on revusiran and 2 (3.0%) on placebo died during the on-treatment period. Most deaths in both treatment arms were adjudicated as cardiovascular due to heart failure (HF), consistent with the natural history of the disease. A post hoc safety investigation of patients treated with revusiran found that, at baseline, a greater proportion of those who died were ≥ 75 years and showed clinical evidence of more advanced HF compared with those who were alive throughout treatment. Revusiran pharmacokinetic exposures and TTR lowering did not show meaningful differences between patients who died and who were alive. Revusiran did not deleteriously affect echocardiographic parameters, cardiac biomarkers, or frequency of cardiovascular and HF hospitalization events. CONCLUSIONS: Causes for the observed mortality imbalance associated with revusiran were thoroughly investigated and no clear causative mechanism could be identified. Although the results suggest similar progression of cardiac parameters in both treatment arms, a role for revusiran cannot be excluded. CLINICAL TRIAL REGISTRATION: NCT02319005.

3.
Muscle Nerve ; 61(1): 95-100, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31587306

RESUMO

INTRODUCTION: Although peripheral neuropathy and cardiomyopathy are well-recognized manifestations of transthyretin (ATTR) amyloidosis, myopathy has been rarely reported. METHODS: In this study we reviewed our muscle biopsy database (January 1998 to June 2018) to identify patients with ATTR amyloid myopathy confirmed by molecular or proteomic analysis. Clinical and laboratory findings were reviewed. RESULTS: We identified eight ATTR amyloid myopathy patients (5 hereditary ATTR [ATTRv] and 3 wild-type ATTR [ATTRwt]). Myopathy was the initial manifestation in all ATTRwt patients and followed peripheral neuropathy (4 patients) or cardiomyopathy (1 patient) in ATTRv patients. One ATTRv patient developed myopathy after liver transplant. Peripheral neuropathy and cardiac amyloidosis occurred in seven and six patients, respectively. Muscle biopsy showed interstitial amyloid deposition in all patients, rare necrotic/regenerating fibers in six, and vacuoles in four. DISCUSSION: Myopathy can be the initial manifestation of ATTRwt amyloidosis and can precede the peripheral neuropathy or occur after liver transplant in ATTRv amyloidosis.


Assuntos
Neuropatias Amiloides Familiares/patologia , Doenças Musculares/patologia , Pré-Albumina , Adulto , Idoso , Idoso de 80 Anos ou mais , Neuropatias Amiloides Familiares/complicações , Biópsia , Cardiomiopatias/patologia , Bases de Dados Factuais , Eletromiografia , Feminino , Humanos , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/patologia , Doenças Musculares/etiologia , Necrose , Condução Nervosa , Doenças do Sistema Nervoso Periférico/patologia , Proteômica
4.
Mod Pathol ; 2019 Nov 13.
Artigo em Inglês | MEDLINE | ID: mdl-31723241

RESUMO

Histomorphologic parameters of atrial appendages removed during the Cox-Maze procedure have been shown to correlate with recurrence of atrial fibrillation. While amyloid deposition has been noted within atrial appendages, the incidence and significance remains incompletely understood. More accurate amyloid typing methodologies and targeted pharmacotherapeutics have recently been developed, prompting pathologists to provide more detailed information about the type of amyloid identified in such samples. This study sought to fully characterize the morphologic characteristics of atrial amyloid as well as its incidence and clinical significance. Tissue archives were queried for atrial appendages removed during the cardiac surgeries (2010-2014). Patient demographics, imaging features, and salient clinical findings were recorded. Pattern and extent of amyloid deposition were recorded. Typing of the amyloid protein, when present, was performed on a subset of cases by laser capture microdissection with mass spectrometry-based proteomic analysis. A total of 383 atrial appendages from 345 consecutive patients were included in the study (mean age, 69 years; range, 26-92 years). Amyloid was present in 46% of patients. A linear relationship was observed between age and presence of atrial amyloidosis. Women were more likely to have atrial amyloidosis. Two distinct morphologies of amyloid were observed: filamentous and nonfilamentous, and correlated perfectly with amyloid type (filamentous = AANF-type amyloid; nonfilamentous = ATTR-type amyloid). Filamentous deposits were observed in 91% of those with amyloid. Amyloid was more likely to be found in the left atrial appendage than the right. Patients with atrial amyloid, irrespective of type, were more likely to have experienced stroke or TIA and more likely to have atrial arrhythmia preoperatively. Postoperatively, those with atrial amyloid are more likely to experience recurrence of arrhythmia than those who did not have atrial amyloid. Understanding the morphologic characteristics of AANF-type amyloid will allow for identification by the light microscopy and obviates the need for expensive ancillary typing techniques. The finding of nonfilamentous amyloid, should still prompt confirmation of amyloid type so that targeted therapy may be employed.

5.
Circ Heart Fail ; 12(9): e006075, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31480867

RESUMO

Cardiomyopathy is a manifestation of transthyretin amyloidosis (ATTR), which is an underrecognized systemic disease whereby the transthyretin protein misfolds to form fibrils that deposit in various tissues and organs. ATTR amyloidosis is debilitating and associated with poor life expectancy, especially in those with cardiac dysfunction, but a variety of treatment options have recently become available. Considered a rare disease, ATTR amyloidosis may be more prevalent than thought, particularly in older persons. Diagnosis is often delayed because of a lack of disease awareness and the heterogeneity of symptoms at presentation. Given the recent availability of effective treatments, early recognition and diagnosis are especially critical because treatment is likely more effective earlier in the disease course. The Amyloidosis Research Consortium recently convened a group of experts in ATTR amyloidosis who, through an iterative process, agreed on best practices for suspicion, diagnosis, and characterization of disease. This review describes these consensus recommendations for ATTR associated with cardiomyopathy as a resource to aid cardiologists and others in the recognition and diagnosis of ATTR associated with cardiomyopathy. Included in this review is an overview of red flag signs and symptoms and a recommended diagnostic approach, including testing for monoclonal protein, scintigraphy, or biopsy and, if ATTR associated with cardiomyopathy is identified, TTR genotyping.

6.
Brain Behav ; 9(9): e01371, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31368669

RESUMO

INTRODUCTION: Amyloid transthyretin amyloidosis (ATTR) is a progressive and often fatal disease caused by the buildup of mutated (hereditary ATTR [hATTR]; also known as ATTR variant [ATTRv]) or normal transthyretin (wild-type ATTR) throughout the body. Two new therapies-inotersen, an antisense oligonucleotide therapy, and patisiran, an RNA interference therapy-received marketing authorization and represent a significant advance in the treatment of amyloidosis. Herein, we describe the clinical presentation of ATTR, commonly used procedures in its diagnosis, and current treatment landscape for ATTR, with a focus on hATTR. METHODS: A PubMed search from 2008 to September 2018 was conducted to review the literature on ATTR. RESULTS: Until recently, there have been few treatment options for polyneuropathy of hATTR. Inotersen and patisiran substantially reduce the amyloidogenic precursor protein transthyretin and have demonstrated efficacy in patients with early- and late-stage disease and in slowing or improving neuropathy progression. In contrast, established therapies, such as liver transplantation, typically reserved for patients with early-stage disease, and tafamidis, indicated for the treatment of early-stage disease in Europe, or diflunisal, a nonsteroidal anti-inflammatory drug that is used off-label, are associated with side effects and/or unclear efficacy in certain patient populations. Thus, inotersen and patisiran are positioned to be the preferred therapeutic modalities. CONCLUSIONS: Important differences between inotersen and patisiran, including formulation, dosing, requirements for premedications, and safety monitoring, require an understanding and knowledge of each treatment for informed decision making.

7.
Br J Haematol ; 187(5): 588-594, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31298751

RESUMO

Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long-term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis (n = 186) were the subject of this study. Ten-year survivors represented 22% of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10-year survivors compared to those who did not survive to 10 years. All-time best haematological response was complete response in 67%, very good partial response in 30%, partial response in 2% and no response in 1%, with 11% having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first-line therapy was 76%, which increased to 86% when considering subsequent line(s) of therapy. Forty-seven percent of the 10-year survivors did not require a second-line therapy. The median treatment-free survival (TFS) among the 10-year survivors was 10·5 years (interquartile range 7·4-12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long-term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features.

10.
J Am Soc Echocardiogr ; 32(8): 958-968.e4, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31230779

RESUMO

BACKGROUND: The aim of this study was to test the hypothesis that intrinsic cardiac elastography can detect diastolic tissue abnormalities produced by cardiac amyloid infiltration and that measurements may have incremental value beyond traditional echocardiographic measures. The specific aims were (1) to evaluate the relationship between left ventricular myocardial stiffness (by elastography) and measures of diastolic chamber stiffness and systolic strain in patients with amyloidosis and (2) to compare their prognostic potential. METHODS: We prospectively studied 67 patients with amyloidosis (cardiac amyloidosis, n = 48; noncardiac amyloidosis, n = 19) and 40 normal subjects. Patients underwent comprehensive echocardiography including measurement of left ventricular global longitudinal strain (GLS) by speckle-tracking. Intrinsic velocity propagation of myocardial stretch (iVP), a direct measure of myocardial elasticity, was quantified using intrinsic cardiac elastography. Chamber stiffness was evaluated from the end-diastolic pressure-volume relationships (P = αVß). The major end point at follow-up was the composite of death, cardiac hospitalization, worsening heart failure, and stroke. RESULTS: The iVP of myocardial stretch was highest in patients with cardiac amyloidosis compared with those with noncardiac amyloidosis and normal subjects (3.2 ± 1.0, 1.8 ± 0.4, and 1.6 ± 0.2 m/sec, respectively; P < .0001) and correlated with chamber stiffness, function, and structure (ß coefficient, operating chamber stiffness, GLS, wall thickness; P ≤ .001 for all). At follow-up (median, 2.6 years), measures of left ventricular and myocardial stiffness, GLS, diastolic dysfunction grade, and N-terminal pro-brain natriuretic peptide were associated with excess events. At multivariate analysis, iVP of myocardial stretch remained an independent predictor of adverse events, incremental to GLS and N-terminal pro-brain natriuretic peptide. CONCLUSIONS: Measurements by cardiac elastography correlate with functional and structural derangements produced by cardiac amyloid infiltration but provide unique information that is incremental to conventional echocardiography.

11.
J Am Coll Cardiol ; 73(22): 2872-2891, 2019 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-31171094

RESUMO

Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) in older adults, resulting from myocardial deposition of misfolded transthyretin (TTR) or pre-albumin. Characteristic patterns of echocardiography and cardiac magnetic resonance can strongly suggest the disease but are not diagnostic. The diagnosis can be made with noninvasive nuclear imaging when there is no evidence of a monoclonal protein. Amyloid fibril formation results from a destabilizing mutation in hereditary ATTR amyloidosis (hATTR) or from an aging-linked process in wild-type ATTR amyloidosis (wtATTR). Recent studies have suggested that up to 10% to 15% of older adults with HF may have unrecognized wtATTR. Associated features, including carpal tunnel syndrome and lumbar spinal stenosis, raise suspicion and may afford a means for early diagnosis. Previously treatable only by organ transplantation, pharmaceutical therapy that slows or halts ATTR-CM progression and favorably affects clinical outcomes is now available. Early recognition remains essential to afford the best treatment efficacy.

13.
Blood Cancer J ; 9(3): 30, 2019 03 05.
Artigo em Inglês | MEDLINE | ID: mdl-30837451

RESUMO

The amyloidoses are a group of disorders with overlapping clinical presentations, characterized by aggregation and tissue deposition of misfolded proteins. The nature and source of the amyloidogenic protein determines therapy, therefore correct subtyping is critical to patient management. We report the clinicopathologic features of nine patients diagnosed with two amyloid types confirmed by liquid chromatography-coupled tandem mass spectrometry. The most common types were transthyrethin (n = 9) and immunoglobulin-derived (n = 7). Two patients did not have immunoglobulin-derived amyloidosis despite the presence of a monoclonal gammopathy. Eight patients were diagnosed with two types concurrently, and one patient had an 11-year interval between diagnoses. Histopathological distribution of amyloid was variable with vascular, interstitial, and periosteal deposits seen. Identification of a second type was incidental in seven patients, but led to genetic counselling in one patient and therapy directed at both amyloid subtypes in another. With longer survival of myeloma and AL amyloidosis patients and increasing prevalence of patients with wild-type transthyretin amyloidosis due to an aging population, the phenomenon of two amyloid types in a single patient will be encountered more frequently. In light of revolutionary new therapies for transthyretin amyloidosis (patisiran, tafamidis, and inotersen), recognition of dual amyloid types is highly clinically relevant.


Assuntos
Amiloidose/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Biomarcadores , Cromatografia Líquida , Diagnóstico Diferencial , Feminino , Humanos , Microdissecção e Captura a Laser , Masculino , Espectrometria de Massas , Pessoa de Meia-Idade , Especificidade de Órgãos , Avaliação de Sintomas
14.
J Am Coll Cardiol ; 74(3): 285-295, 2019 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-30885685

RESUMO

BACKGROUND: Transthyretin (TTR) amyloidosis is an underdiagnosed disease caused by destabilization of TTR due to pathogenic mutations or aging. Both pathogenic and protective mutations illuminate mechanisms of disease and potential interventions. AG10 is a selective, oral TTR stabilizer under development for transthyretin amyloidosis cardiomyopathy (ATTR-CM) that mimics a protective TTR mutation. OBJECTIVES: This randomized, double-blind, placebo-controlled study evaluated safety, tolerability, pharmacokinetics, and pharmacodynamics of AG10 in ATTR-CM patients with symptomatic, chronic heart failure. METHODS: ATTR-CM, New York Heart Association functional class II to III subjects (n = 49, mutant or wild-type) were randomized 1:1:1 to AG10 400 mg, AG10 800 mg, or placebo twice daily for 28 days. Safety and tolerability were assessed by clinical and laboratory criteria. AG10 plasma levels were measured. TTR stability was assessed by changes in serum TTR, and 2 established ex vivo assays (fluorescent probe exclusion and Western blot). RESULTS: AG10 treatment was well-tolerated, achieved target plasma concentrations and demonstrated near-complete stabilization of TTR. TTR stabilization was more complete and less variable at the higher dose with stabilization by fluorescent probe exclusion of 92 ± 10% (mean ± SD) at trough and 96 ± 9% at peak (both p < 10-12 vs. placebo). Average serum TTR increased by 36 ± 21% and 51 ± 38% at 400 and 800 mg, respectively (both p < 0.0001 vs. placebo). Baseline serum TTR in treated subjects was below normal in 80% of mutant and 33% of wild-type subjects. AG10 treatment restored serum TTR to the normal range in all subjects. CONCLUSIONS: AG10 has the potential to be a safe and effective treatment for patients with ATTR-CM. A phase 3 trial is ongoing. (Study of AG10 in Amyloid Cardiomyopathy; NCT03458130).

16.
Mayo Clin Proc ; 94(3): 447-454, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30718069

RESUMO

OBJECTIVE: To determine the utility of the serum free light chain (FLC) assay for routine screening of light chain amyloidosis (AL) in patients with heart failure. PATIENTS AND METHODS: We studied consecutive new patients referred to the Heart Failure Clinic who had the FLC assay performed for routine screening at Mayo Clinic's campus in Rochester, Minnesota, from January 1, 2011, through December 31, 2015. An FLC ratio between 0.26 and 1.65 was considered normal. RESULTS: Of the 1173 patients in the study (mean age, 64±15 years), 207 had an abnormal FLC ratio. Light chain amyloidosis was diagnosed in 0.5% of all patients (6 of 1173) and in 2.9% of those with an abnormal FLC ratio (6 of 207). To increase the pretest probability of an abnormal FLC ratio in predicting AL, we considered patients with an N-terminal pro B-type natriuretic peptide level of 5000 pg/mL or greater (to convert to pmol/L, multiply by 0.1182) and a left ventricular posterior wall thickness of 13 mm or greater; this increased the diagnostic yield to 66.7% (6 of 9). CONCLUSION: The prevalence of AL in patients with heart failure could be 0.5% or higher. Compared with the use of the serum FLC assay for routine screening, a targeted approach of the serum FLC assay in those with higher N-terminal pro B-type natriuretic peptide level (≥5000 pg/mL) and increased posterior wall thickness (≥13 mm) has a markedly higher yield for the diagnosis of AL.


Assuntos
Amiloidose/sangue , Insuficiência Cardíaca/sangue , Cadeias Leves de Imunoglobulina/sangue , Cadeias kappa de Imunoglobulina/sangue , Cadeias lambda de Imunoglobulina/sangue , Adulto , Idoso , Amiloidose/diagnóstico , Biomarcadores/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miocárdio/metabolismo
17.
Mayo Clin Proc ; 94(3): 455-464, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30718070

RESUMO

OBJECTIVE: To evaluate the prognostic impact of Holter findings in patients with light chain amyloidosis. PATIENTS AND METHODS: We evaluated 239 patients in whom light chain amyloidosis was diagnosed from January 1, 2010, through December 31, 2015, who underwent 24-hour Holter monitoring. RESULTS: Holter testing was done before stem cell transplant evaluation in 183 of the 239 patients (76.6%) and at diagnosis in 50 (20.9%). Holter findings were nonsustained ventricular tachycardia (NSVT) in 60 patients (25.1%), ventricular couplets in 103 (43.1)%, accelerated idioventricular rhythm in 32 (13.4%), and atrial fibrillation (AF) in 18 (7.5%). Overall survival (OS) at 3 and 6 months after Holter monitoring in patients with AF vs without AF was 78% (95% CI, 54%-91%) vs 96% (95% CI, 92%-98%) (P=.002) and 61% (95% CI, 38%-80%) vs 92% (95% CI, 87%-95%), (P<.001), respectively. In patients with and without NSVT, 3- and 6-month OS after Holter testing was 90% (95% CI, 80%-94%) vs 96% (95% CI, 91%-98%) (P=.12) and 77% (95% CI, 64%-85%) vs 94% (95% CI, 89%-97%) (P<.001), respectively. For patients with and without ventricular couplets, 3- and 6-month OS was 94% (95% CI, 88%-97%) vs 94% (95% CI, 89%-97%) (P=.98) and 84% (95% CI, 75%-89%) vs 94% (95% CI, 89%-97%) (P=.01), respectively. Atrial fibrillation (hazard ratio, 2.5; 95% CI, 1.2-5.0; P=.02) and NSVT (hazard ratio, 2.0; 95% CI, 1.1-3.5; P=.02) were independent predictors for OS after accounting for age and Mayo stage. For patients undergoing routine testing before stem cell transplant, AF (P=.002) and NSVT (P=.02) were associated with inferior OS at 6 months but did not retain statistical significance after adjusting for Mayo stage (P=.10 and P=.54, respectively). CONCLUSION: Atrial fibrillation and NSVT on 24-hour Holter monitoring are associated with inferior short-term OS outcomes but do not impact peritransplant mortality.


Assuntos
Fibrilação Atrial/fisiopatologia , Eletrocardiografia Ambulatorial/métodos , Amiloidose de Cadeia Leve de Imunoglobulina/fisiopatologia , Amiloidose de Cadeia Leve de Imunoglobulina/terapia , Idoso , Fibrilação Atrial/mortalidade , Eletrocardiografia , Feminino , Transplante de Células-Tronco Hematopoéticas , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico
18.
J Am Coll Cardiol ; 73(5): 589-597, 2019 02 12.
Artigo em Inglês | MEDLINE | ID: mdl-30732713

RESUMO

BACKGROUND: Arrhythmias, conduction abnormalities, and intracardiac thrombus are common in patients with cardiac amyloidosis (CA). Outcomes of direct-current cardioversion (DCCV) for atrial arrhythmias in patients with CA are unknown. OBJECTIVES: This study sought to examine DCCV procedural outcomes in patients with CA. METHODS: Patients with CA scheduled for DCCV for atrial arrhythmias from January 2000 through December 2012 were identified and matched 2:1 with control patients by age, sex, type of atrial arrhythmia, and date of DCCV. RESULTS: CA patients (n = 58, mean age 69 ± 9 years, 81% male) were included. CA patients had a significantly higher cardioversion cancellation rate (28% vs. 7%; p < 0.001) compared with control patients, mainly due to intracardiac thrombus identified on transesophageal echocardiogram (13 of 16 [81%] vs. 2 of 8 [25%]; p = 0.02); 4 of 13 of the CA patients (31%) with intracardiac thrombus on transesophageal echocardiogram received adequate anticoagulation ≥3 weeks and another 2 of 13 (15%) had arrhythmia duration <48 h. DCCV success rate (90% vs. 94%; p = 0.4) was not different. Procedural complications were more frequent in CA versus control patients (6 of 42 [14%] vs. 2 of 106 [2%]; p = 0.007); complications in CA included ventricular arrhythmias in 2 and severe bradyarrhythmias requiring pacemaker implantation in 2. The only complication in the control group was self-limited bradyarrhythmias. CONCLUSIONS: Patients with CA undergoing DCCV had a significantly high cancellation rate mainly due to a high incidence of intracardiac thrombus even among patients who received adequate anticoagulation. Although the success rate of restoring sinus rhythm was high, tachyarrhythmias and bradyarrhythmias complicating DCCV were significantly more frequent in CA patients compared with control patients.


Assuntos
Amiloidose , Fibrilação Atrial , Cardioversão Elétrica , Cardiopatias , Trombose , Idoso , Amiloidose/complicações , Amiloidose/diagnóstico , Fibrilação Atrial/etiologia , Fibrilação Atrial/terapia , Contraindicações de Procedimentos , Ecocardiografia Transesofagiana/métodos , Cardioversão Elétrica/efeitos adversos , Cardioversão Elétrica/métodos , Feminino , Cardiopatias/complicações , Cardiopatias/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Minnesota/epidemiologia , Estudos Retrospectivos , Risco Ajustado , Trombose/diagnóstico por imagem , Trombose/etiologia
20.
J Cardiovasc Imaging ; 27(1): 1-10, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30701710

RESUMO

Cardiac amyloidosis is a rare disease that frequently presents as ventricular hypertrophy. However, diagnosis is not always easy or straightforward as there are several myocardial disorders that phenocopy cardiac amyloidosis. Here, we present a narrative review of the current modalities that are actively used or being developed for diagnosis and follow-up of cardiac amyloidosis. Although not all of the findings may be present in those with cardiac amyloidosis, there are some clues in each diagnostic step that help lead to confirmatory diagnosis of cardiac amyloidosis; we believe that cardiologists should be familiar with these clues.

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