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3.
Artigo em Inglês | MEDLINE | ID: mdl-33093765

RESUMO

Background: Patients with cardiac sarcoidosis (CS) are at increased risk of atrioventricular blocks, ventricular arrhythmias, and sudden cardiac death. Objectives We aimed to investigate the characteristics associated with appropriate therapy in implantable cardiac defibrillator (ICD) -implanted CS patients. Methods: We performed a PubMed and Web of Science search for studies reporting patients with CS who underwent an ICD implantation. The primary criterion was an appropriate therapy. Results: We screened 705 studies, of which 5 were included in the final analysis. We conducted a meta-analysis including 464 patients (mean age 55 years, 282 males (60%)). The mean follow-up was 3.5 years. Among the 464 patients, 180 received an appropriate therapy (39%). Patients who received an appropriate therapy were younger (-3.33, 95% confidence interval (CI) -6.42 to -0.23, p=0.004), were more likely to be male (OR 2.06, 95% CI 1.37-3.09, p=0.0005), had a lower left ventricular ejection fraction (LVEF) (-10.5, 95% CI -18.23 to -2.78, p=0.008), had a higher rate of complete heart block (OR 2.19, 95% CI 1.20 to 3.99, p=0.01), and more frequently had ventricular pacing (OR 6.44 95% CI 2.57 to 16.16, p<0.0001). Conclusions: Appropriate ICD therapy during CS is associated with young age, male sex, low LVEF, history of complete heart block, and ventricular pacing. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1): 17-23).


Assuntos
Cardiomiopatias/terapia , Desfibriladores Implantáveis , Cardioversão Elétrica/instrumentação , Bloqueio Cardíaco/terapia , Sarcoidose/terapia , Adulto , Fatores Etários , Idoso , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/mortalidade , Cardiomiopatias/fisiopatologia , Morte Súbita Cardíaca/prevenção & controle , Cardioversão Elétrica/efeitos adversos , Cardioversão Elétrica/mortalidade , Feminino , Bloqueio Cardíaco/diagnóstico por imagem , Bloqueio Cardíaco/mortalidade , Bloqueio Cardíaco/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Medição de Risco , Fatores de Risco , Sarcoidose/diagnóstico por imagem , Sarcoidose/mortalidade , Sarcoidose/fisiopatologia , Fatores Sexuais , Volume Sistólico , Resultado do Tratamento , Função Ventricular Esquerda
4.
Radiol Med ; 125(11): 1087-1101, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32978708

RESUMO

Over the past few years, the approach to the 'arrhythmic patient' has profoundly changed. An early clinical presentation of arrhythmia is often accompanied by non-specific symptoms and followed by inconclusive electrocardiographic findings. In this scenario, cardiac magnetic resonance (CMR) has been established as a clinical tool of fundamental importance for a correct prognostic stratification of the arrhythmic patient. This technique provides a high-spatial-resolution tomographic evaluation of the heart, which allows studying accurately the ventricular volumes, identifying even segmental kinetic anomalies and properly detecting diffuse or focal tissue alterations through an excellent tissue characterization, while depicting different patterns of fibrosis distribution, myocardial edema or fatty substitution. Through these capabilities, CMR has a pivotal role for the adequate management of the arrhythmic patient, allowing the identification of those phenotypic manifestations characteristic of structural heart diseases. Therefore, CMR provides valuable information to reclassify the patient within the wide spectrum of potentially arrhythmogenic heart diseases, the definition of which remains the major determinants for both an adequate treatment and a poor prognosis. The purpose of this review study was to focus on the role of CMR in the evaluation of the main cardiac clinical entities associated with arrhythmogenic phenomena and to present a brief debate on the main pathophysiological mechanisms involved in the arrhythmogenesis process.


Assuntos
Arritmias Cardíacas/diagnóstico por imagem , Técnicas de Imagem Cardíaca/métodos , Cardiomiopatias/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/fisiopatologia , Cardiomegalia Induzida por Exercícios , Cardiomiopatias/complicações , Cardiomiopatias/fisiopatologia , Diagnóstico Diferencial , Ecocardiografia/métodos , Eletrocardiografia , Fibrose Endomiocárdica/complicações , Fibrose Endomiocárdica/diagnóstico por imagem , Humanos , Prolapso da Valva Mitral/complicações , Prolapso da Valva Mitral/diagnóstico por imagem
5.
Radiol Med ; 125(11): 1072-1086, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32970272

RESUMO

The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. In this context, cardiac magnetic resonance (CMR) may play a crucial role in defining the diagnosis and guiding treatments, by offering a robust myocardial characterization based on the inherent magnetic properties of abnormal tissues, thus limiting the use of endomyocardial biopsy. In this review article, we explore the role of CMR in the assessment of a wide range of myocardial diseases causing restrictive patterns, from iron overload to cardiac amyloidosis, endomyocardial fibrosis or radiation-induced heart disease. Here, we emphasize the incremental value of novel relaxometric techniques such as T1 and T2 mapping, which may recognize different storage diseases based on the intrinsic magnetic properties of the accumulating metabolites, with or without the use of gadolinium-based contrast agents. We illustrate the importance of these CMR techniques and their great support when contrast media administration is contraindicated. Finally, we describe the useful role of cardiac computed tomography for diagnosis and management of restrictive cardiomyopathies when CMR is contraindicated.


Assuntos
Técnicas de Imagem Cardíaca/métodos , Cardiomiopatia Restritiva/diagnóstico por imagem , Imagem por Ressonância Magnética/métodos , Adulto , Idoso de 80 Anos ou mais , Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatia Restritiva/classificação , Fibrose Endomiocárdica/diagnóstico por imagem , Feminino , Humanos , Sobrecarga de Ferro/diagnóstico por imagem , Doenças por Armazenamento dos Lisossomos/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Lesões por Radiação/complicações , Lesões por Radiação/diagnóstico por imagem , Sarcoidose/diagnóstico por imagem
6.
PLoS One ; 15(9): e0238391, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32946452

RESUMO

BACKGROUND: Prognostic factors are lacking in cardiac sarcoidosis (CS), and the effects of immunosuppressive treatments are unclear. OBJECTIVES: To identify prognostic factors and to assess the effects of immunosuppressive drugs on relapse risk in patients presenting with CS. METHODS: From a cohort of 157 patients with CS with a median follow-up of 7 years, we analysed all cardiac and extra-cardiac data and treatments, and assessed relapse-free and overall survival. RESULTS: The 10-year survival rate was 90% (95% CI, 84-96). Baseline factors associated with mortality were the presence of high degree atrioventricular block (HR, 5.56, 95% CI 1.7-18.2, p = 0.005), left ventricular ejection fraction below 40% (HR, 4.88, 95% CI 1.26-18.9, p = 0.022), hypertension (HR, 4.79, 95% CI 1.06-21.7, p = 0.042), abnormal pulmonary function test (HR, 3.27, 95% CI 1.07-10.0, p = 0.038), areas of late gadolinium enhancement on cardiac magnetic resonance (HR, 2.26, 95% CI 0.25-20.4, p = 0.003), and older age (HR per 10 years 1.69, 95% CI 1.13-2.52, p = 0.01). The 10-year relapse-free survival rate for cardiac relapses was 53% (95% CI, 44-63). Baseline factors that were independently associated with cardiac relapse were kidney involvement (HR, 3.35, 95% CI 1.39-8.07, p = 0.007), wall motion abnormalities (HR, 2.30, 95% CI 1.22-4.32, p = 0.010), and left heart failure (HR 2.23, 95% CI 1.12-4.45, p = 0.023). After adjustment for cardiac involvement severity, treatment with intravenous cyclophosphamide was associated with a lower risk of cardiac relapse (HR 0.16, 95% CI 0.033-0.78, p = 0.024). CONCLUSIONS: Our study identifies putative factors affecting morbidity and mortality in cardiac sarcoidosis patients. Intravenous cyclophosphamide is associated with lower relapse rates.


Assuntos
Cardiomiopatias/tratamento farmacológico , Imunossupressores/uso terapêutico , Sarcoidose/tratamento farmacológico , Adulto , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/mortalidade , Estudos de Coortes , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Paris/epidemiologia , Prognóstico , Intervalo Livre de Progressão , Recidiva , Estudos Retrospectivos , Sarcoidose/diagnóstico por imagem , Sarcoidose/mortalidade
7.
J Cardiovasc Magn Reson ; 22(1): 66, 2020 09 14.
Artigo em Inglês | MEDLINE | ID: mdl-32921308

RESUMO

BACKGROUND: Cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) is a valuable technique for detecting myocardial disorders and fibrosis. However, we sometimes observe a linear, mid-wall high intensity signal in the basal septum in the short axis view, which often presents diagnostic difficulties in the clinical setting. The purpose of this study was to compare the linear, mid-wall high intensity in the basal septum identified by LGE with the anterior septal perforator arteries identified by coronary computed tomography angiography (CorCTA). METHODS: We retrospectively selected 148 patients who underwent both CorCTA and CMR LGE within 1 year. In the interpretation of LGE, we defined a positive linear high intensity (LHI+) as follows: ① LHI in the basal septum and ② observable for 1.5 cm or more. All other patients were defined as a negative LHI (LHI-). In LHI+ patients, we assessed the correlation between the LHI length and the septal perforator artery length on CorCTA. We also compared the length of the septal perforator artery on CorCTA between LHI+ patients and LHI- patients. RESULTS: A population of 111 patients were used for further analysis. Among these , there were 55 LHI+ patients and 56 LHI- patients. In LHI+ patients, linear regression analysis revealed that there was a good agreement between LGE LHI and septal perforator arteries by CorCTA in terms of length measurements. The measured length of the anterior septal perforator arteries was significantly shorter in LHI- patients than in LHI+ patients (10 ± 8 mm vs. 21 ± 8 mm; P < 0.05). CONCLUSIONS: The LHI observed in the basal septum on short axis LGE may reflect contrast enhancement of the anterior septal perforator arteries. It is important to interpret this septal LHI against knowledge of anatomic structure, to avoid misinterpretations of LGE and prevent misdiagnosis.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Angiografia por Tomografia Computadorizada , Meios de Contraste/administração & dosagem , Angiografia Coronária , Vasos Coronários/diagnóstico por imagem , Gadolínio DTPA/administração & dosagem , Septos Cardíacos/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Isquemia Miocárdica/diagnóstico por imagem , Idoso , Artefatos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos
8.
Clin Imaging ; 67: 246-249, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32871429

RESUMO

PURPOSE: The aims of our study were to compare cardiac magnetic resonance (CMR) scan time (time elapsed between first and last acquired sequence) and room time (time elapsed between patients' entrance and exit of the MRI room) between sedated and non-sedated children and to assess the contributing factors. METHODS: It is a cohort study performed at a CMR facility in a tertiary-care center. We compared scan time and room time between sedated and non-sedated children and assessed the influence of gender, age, weight, height, body surface area, number of sequences, and patients' diagnosis/previous procedures. RESULTS: We included 140 children (74 sedated and 66 non-sedated), age 7.6 years (3-12) and body surface area 0.83 m2 (0.57-1.25). The most common diagnosis/previous procedures were repaired tetralogy of Fallot, cardiomyopathies and single ventricle. Number of sequences: 18 (16-22). Scan time: 42 min (35-54.2); room time: 53 min (46-63). There was difference in room time between sedated and non-sedated children (57.6 ± 13.6 vs 52.2 ± 15 min, p < 0.05), but not in scan time (47.2 ± 14.1 vs 43.9 ± 16 min, p 0.2). The only factor associated with scan time was the number of sequences (r 0.75, p < 0.001). The only factor associated with room time was single ventricle as indication (r 0.26, p 0.007). CONCLUSIONS: Sedated children spent 5 more minutes in the CMR room than non-sedated children. Single ventricle was associated with increased room time. Number of sequences correlated with scan time. Sedation did not influence these times.


Assuntos
Anestesia , Cardiomiopatias/diagnóstico por imagem , Espectroscopia de Ressonância Magnética , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Coração , Humanos , Imagem por Ressonância Magnética , Masculino
9.
Life Sci ; 259: 118199, 2020 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-32781064

RESUMO

Cellular senescence, a process whereby cells enter a state of permanent growth arrest, appears to regulate cardiac pathological remodeling and dysfunction in response to various stresses including myocardial infarction (MI). However, the precise role as well as the underlying regulatory mechanism of cardiac cellular senescence in the ischemic heart disease remain to be further determined. Herein we report an inhibitory role of Nrf2, a key transcription factor of cellular defense, in regulating cardiac senescence in infarcted hearts as well as a therapeutic potential of targeting Nrf2-mediated suppression of cardiac senescence in the treatment of MI-induced cardiac dysfunction. MI was induced by left coronary artery ligation for 28 days in mice. Heart tissues from the infarct border zone were used for the analyses. The MI-induced cardiac dysfunction was associated with increased myocardial cell senescence, oxidative stress and apoptosis in adult wild type (WT) mice. In addition, a downregulated Nrf2 activity was associated with upregulated Keap1 levels and increased phosphorylation of JAK and FYN in the infarcted border zone heart tissues. Nrf2 Knockout (Nrf2-/-) enhanced the MI-induced myocardial, cardiac dysfunction and senescence. Qiliqiangxin (QLQX), a herbal medicine which could reverse the MI-induced suppression of Nrf2 activity, significantly inhibited the MI-induced cardiac senescence, apoptosis, and cardiac dysfunction in WT mice but not in Nrf2-/- mice. These results indicate that MI downregulates Nrf2 activity thus promoting oxidative stress to accelerate cellular senescence in the infarcted heart towards cardiac dysfunction and Nrf2 may be a drug target for suppressing the cellular senescence-associated pathologies in infarcted hearts.


Assuntos
Cardiomiopatias/genética , Cardiomiopatias/patologia , Senescência Celular/genética , Infarto do Miocárdio/genética , Infarto do Miocárdio/patologia , Miocárdio/patologia , Fator 2 Relacionado a NF-E2/genética , Animais , Cardiomiopatias/diagnóstico por imagem , Ecocardiografia , Inativação Gênica , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Infarto do Miocárdio/diagnóstico por imagem , Miócitos Cardíacos/metabolismo , RNA Interferente Pequeno/farmacologia , Remodelação Ventricular/fisiologia
10.
J Cardiovasc Magn Reson ; 22(1): 60, 2020 08 20.
Artigo em Inglês | MEDLINE | ID: mdl-32814579

RESUMO

BACKGROUND: Tissue characterisation with cardiovascular magnetic resonance (CMR) parametric mapping has the potential to detect and quantify both focal and diffuse alterations in myocardial structure not assessable by late gadolinium enhancement. Native T1 mapping in particular has shown promise as a useful biomarker to support diagnostic, therapeutic and prognostic decision-making in ischaemic and non-ischaemic cardiomyopathies. METHODS: Convolutional neural networks (CNNs) with Bayesian inference are a category of artificial neural networks which model the uncertainty of the network output. This study presents an automated framework for tissue characterisation from native shortened modified Look-Locker inversion recovery ShMOLLI T1 mapping at 1.5 T using a Probabilistic Hierarchical Segmentation (PHiSeg) network (PHCUMIS 119-127, 2019). In addition, we use the uncertainty information provided by the PHiSeg network in a novel automated quality control (QC) step to identify uncertain T1 values. The PHiSeg network and QC were validated against manual analysis on a cohort of the UK Biobank containing healthy subjects and chronic cardiomyopathy patients (N=100 for the PHiSeg network and N=700 for the QC). We used the proposed method to obtain reference T1 ranges for the left ventricular (LV) myocardium in healthy subjects as well as common clinical cardiac conditions. RESULTS: T1 values computed from automatic and manual segmentations were highly correlated (r=0.97). Bland-Altman analysis showed good agreement between the automated and manual measurements. The average Dice metric was 0.84 for the LV myocardium. The sensitivity of detection of erroneous outputs was 91%. Finally, T1 values were automatically derived from 11,882 CMR exams from the UK Biobank. For the healthy cohort, the mean (SD) corrected T1 values were 926.61 (45.26), 934.39 (43.25) and 927.56 (50.36) for global, interventricular septum and free-wall respectively. CONCLUSIONS: The proposed pipeline allows for automatic analysis of myocardial native T1 mapping and includes a QC process to detect potentially erroneous results. T1 reference values were presented for healthy subjects and common clinical cardiac conditions from the largest cohort to date using T1-mapping images.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Interpretação de Imagem Assistida por Computador , Imagem por Ressonância Magnética , Miocárdio/patologia , Redes Neurais de Computação , Automação , Teorema de Bayes , Cardiomiopatias/patologia , Cardiomiopatias/fisiopatologia , Estudos de Casos e Controles , Humanos , Valor Preditivo dos Testes , Controle de Qualidade , Reprodutibilidade dos Testes , Volume Sistólico , Incerteza , Função Ventricular Esquerda
11.
Am J Cardiol ; 133: 126-133, 2020 10 15.
Artigo em Inglês | MEDLINE | ID: mdl-32811652

RESUMO

Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of advanced cardiomyopathy. We aimed to describe early echocardiographic features in patients subsequently diagnosed with CA. Preamyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic subtype (immunoglobulin light-chain [AL] or amyloid transthyretin [ATTR]). Abnormalities were defined based on published guidelines. Our cohort included 75 CA patients of whom 42 (56%) were diagnosed with AL and 33 (44%) with ATTR. Forty-two patients had an earlier echocardiography exam available for review. Patients presented with increased wall thickness (1.3 [interquartile range {IQR} 1.0, 1.5] cm) ≥3 years before the diagnosis of CA and relative wall thickness was increased (0.47 [IQR 0.41, 0.50]) ≥7 years prediagnosis. One to 3 years before CA diagnosis restrictive left ventricular (LV) filling pattern was present in 19% of patients and LV ejection fraction ≤50% was present in 21% of patients. Right ventricular dysfunction was detected concomitantly with disease diagnosis. The echocardiographic phenotype of ATTR versus AL-CA showed increased relative wall thickness (0.74 [IQR 0.62, 0.92] versus 0.62 [IQR 0.54, 0.76], p = 0.004) and LV mass index (144 [IQR 129, 191] versus 115 [IQR 105, 146] g/m2, p = 0.020) and reduced LV ejection fraction (50 [IQR 44, 58] versus (60 [IQR 53, 60]%, p = 0.009) throughout the time course of CA progression, albeit survival time was similar. In conclusion, increased wall thickness and diastolic dysfunction in CA develop over a time course of several years and can be diagnosed in their earlier stages by standard echocardiography.


Assuntos
Neuropatias Amiloides Familiares/complicações , Neuropatias Amiloides Familiares/diagnóstico por imagem , Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico por imagem , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Neuropatias Amiloides Familiares/mortalidade , Cardiomiopatias/mortalidade , Progressão da Doença , Ecocardiografia , Feminino , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo
12.
Int J Cardiovasc Imaging ; 36(11): 2199-2207, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32613384

RESUMO

Cardiac sarcoidosis (CS) is an increasingly recognized condition, but cardiac magnetic resonance (CMR) image interpretation in these patients may be challenging as findings are often non-specific. The main objective of this study was to investigate the inter-reader agreement for the overall interpretation of CMR for the diagnosis of CS in an experienced reference center and investigate factors that may lead to discrepancies between readers. Consecutive patients undergoing CMR imaging to investigate for CS were included. CMR images were independently reviewed by two readers, blinded to all clinical, imaging and demographic information. The readers classified each scan as "consistent with cardiac sarcoidosis", "not consistent with cardiac sarcoidosis" or "indeterminate". Inter-reader agreement was assessed using κ-statistics. When there was disagreement on the overall interpretation, a third reader reviewed the images. Also, two readers independently commented on the presence of edema, presence of LGE (both ventricles) and quantified the extent of left ventricular LGE. 87 patients (43 women, mean age 54.3 ± 12.2 years) were included in the study. There was agreement regarding the overall interpretation in 72 of 87 (83%) CMR scans. The κ value was 0.64, indicating moderate agreement. There was similar moderate agreement in the interpretation of LGE parameters. In an experienced referral center, we found moderate agreement between readers in the interpretation of CMR in patients with suspected CS. Physicians should be aware of this inter-observer variability in interpretation of CMR studies in patients with suspected CS.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Sarcoidose/diagnóstico por imagem , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Valor Preditivo dos Testes , Estudos Prospectivos , Encaminhamento e Consulta , Reprodutibilidade dos Testes
13.
Clin Nucl Med ; 45(9): e416-e418, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32604122

RESUMO

Cardiac sarcoidosis (CS) has a poor prognosis related to life-threating arrhythmias and heart failure. Treatment includes anti-inflammatory therapies and implantable pacemaker and/or cardioverter defibrillator. The presence of cardiac devices and physiologic myocardial glucose uptake are major limitations of both cardiac magnetic resonance and F-FDG PET/CT, reducing their diagnostic value. Somatostatin-based PET/CT has been proposed to detect active CS. Contrarily to F-FDG uptake, which reflects nonspecific leukocyte infiltration, Ga-DOTATOC may identify active granulomatosis. Herein, we underline the specificity of Ga-DOTATOC PET in challeging clinical situations including refractory CS, and chronic CS in patients with cardiac device, or false-positive F-FDG PET/CT results.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/terapia , Octreotida/análogos & derivados , Compostos Organometálicos , Tomografia por Emissão de Pósitrons , Sarcoidose/diagnóstico por imagem , Sarcoidose/terapia , Adulto , Idoso , Reações Falso-Positivas , Feminino , Fluordesoxiglucose F18 , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Sensibilidade e Especificidade , Resultado do Tratamento
14.
Rev Cardiovasc Med ; 21(2): 181-190, 2020 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-32706207

RESUMO

Heart failure with preserved ejection fraction is a very common clinical problem. Its prevalence is increasing with aging of the population. A diverse group of risk factors and etiologies comprise the HFpEF syndrome. No specific therapies have been shown to improve survival for the vast majority of HFpEF cases. Restrictive cardiomyopathies account for a significant portion of HFpEF patients and are characterized by diastolic dysfunction due to infiltration of the myocardium or ventricular hypertrophy. Many of these restrictive diseases occur in the context of myocardial infiltration by other substances such as amyloid, iron or glycogen or endomyocardial fibrosis. These infiltrative diseases usually have important clues in the clinical picture and on cardiac imaging that may allow differentiation from the usual HFpEF phenotype (that is commonly seen in the older, hypertensive patient). Noninvasive diagnosis has replaced endomyocardial biopsy for most instances in the workup of these conditions. Early recognition is important to institute specific therapies and to improve prognosis. In this review, we describe 4 major infiltrative cardiomyopathies (Cardiac Amyloidosis, Sarcoidosis, Hemochromatosis and Fabry disease), and their key imaging features.


Assuntos
Amiloidose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Doença de Fabry/diagnóstico por imagem , Insuficiência Cardíaca/etiologia , Hemocromatose/diagnóstico por imagem , Volume Sistólico , Função Ventricular Esquerda , Amiloidose/complicações , Amiloidose/fisiopatologia , Amiloidose/terapia , Cardiomiopatias/complicações , Cardiomiopatias/fisiopatologia , Cardiomiopatias/terapia , Diagnóstico Diferencial , Diagnóstico Precoce , Doença de Fabry/complicações , Doença de Fabry/fisiopatologia , Doença de Fabry/terapia , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Hemocromatose/complicações , Hemocromatose/fisiopatologia , Hemocromatose/terapia , Humanos , Valor Preditivo dos Testes , Prognóstico
15.
Ann Hematol ; 99(9): 1967-1977, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32621178

RESUMO

Thalassemia is characterized by a defect in the synthesis of one or more of the globin subunits of hemoglobin. This defect results in imbalance in the α/ß-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and iron overload. With advances in diagnosis, treatment, and transfusion support, the prognosis of patients with thalassemia has improved over the past few decades. An increasing number of patients with thalassemia is living with long-term complications, including cardiomyopathy, chronic liver disease, endocrinopathy, and infections. In this paper, we review common complications that bring the patient with thalassemia to urgent or emergent medical attention. We also discuss the aspects of emergency care that are most relevant while caring for the patient with thalassemia in the emergency department.


Assuntos
Serviços Médicos de Emergência/tendências , Serviço Hospitalar de Emergência/tendências , Doenças Raras/diagnóstico por imagem , Doenças Raras/terapia , Talassemia/diagnóstico por imagem , Talassemia/terapia , Betacoronavirus , Transfusão de Sangue/métodos , Transfusão de Sangue/tendências , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/epidemiologia , Cardiomiopatias/terapia , Infecções por Coronavirus/diagnóstico por imagem , Infecções por Coronavirus/epidemiologia , Infecções por Coronavirus/terapia , Diagnóstico Diferencial , Serviços Médicos de Emergência/métodos , Humanos , Hepatopatias/diagnóstico por imagem , Hepatopatias/epidemiologia , Hepatopatias/terapia , Pandemias , Pneumonia Viral/diagnóstico por imagem , Pneumonia Viral/epidemiologia , Pneumonia Viral/terapia , Doenças Raras/epidemiologia , Talassemia/epidemiologia
16.
Clin Imaging ; 67: 136-142, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32622334

RESUMO

We report a marked abnormality in myocardial attenuation on non-gated contrast-enhanced CT in a patient with multiorgan sarcoidosis and correlate our findings with CMR, PET and SPECT. The noteworthy observation of myocardial hypoattenuation, in correspondence with the multimodality cardiovascular imaging findings, suggests that standard contrast-enhanced CT may provide information regarding tissue characterization. This report also demonstrates the independent clinical utility of CMR and PET in the evaluation and management of cardiac sarcoidosis.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Sarcoidose/diagnóstico por imagem , Feminino , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Miocárdio , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada de Emissão de Fóton Único/métodos , Tomografia Computadorizada por Raios X/métodos
18.
Am J Cardiol ; 128: 174-180, 2020 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-32650916

RESUMO

Patients with preserved left ventricular (LV) ejection function (EF) and atrioventricular block (AVB) who are anticipated for high-burden of right ventricular (RV) pacing possess a risk to develop pacing-induced cardiomyopathy (PIC) and adverse clinical outcomes. Hence, the aim of the study is to evaluate the incidence, predictors, and clinical outcomes of RV PIC in patients with preserved LVEF, AVB, and high-burden of RV pacing. One thousand and thirteen patients with second or third-degree AVB underwent first time pacemaker implantation between January 2002 and August 2016. A total of 203 patients with a newly implanted pacemaker, normal baseline LVEF, and high burden of RV pacing were included in the present study. Follow-up echocardiography was examined for a new decrease in LVEF of 10% or higher. Alternative causes for cardiomyopathy were ruled out. Patient characteristics, echocardiographic measurements, device clinic data, mortality, and hospitalizations for heart failure were collected and compared between the PIC and the non-PIC groups. Fifty-one patients (25%) developed LV dysfunction with 22 patients (11%), showing LVEF < 40%. During a mean follow-up of 49.2 months, the risk of heart failure hospitalization or all-cause mortality was significantly higher in the PIC group versus non-PIC group (35.3% vs 19.1%, p = 0.009). In conclusion the incidence of PIC in patients with normal LVEF and AVB, who are anticipated for high-burden of RV pacing is high. PIC in patients with a previously normal LV function is associated with unfavorable long-term clinical outcomes, including higher rates of heart failure hospitalizations and all-cause mortality.


Assuntos
Bloqueio Atrioventricular/terapia , Estimulação Cardíaca Artificial/efeitos adversos , Cardiomiopatias/epidemiologia , Disfunção Ventricular Esquerda/epidemiologia , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/etiologia , Cardiomiopatias/fisiopatologia , Ecocardiografia , Feminino , Insuficiência Cardíaca/epidemiologia , Hospitalização/estatística & dados numéricos , Humanos , Incidência , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Mortalidade , Análise Multivariada , Marca-Passo Artificial , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda
19.
Am J Cardiol ; 128: 60-66, 2020 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-32650925

RESUMO

Left atrial calcium (LAC) is often observed in patients who have undergone mitral valve (MV) surgery, but little is known about its characteristics and clinical implications. Therefore, we sought to investigate the structural and hemodynamic significance of LAC and its association with clinical outcomes. We investigated 327 patients with repaired or prosthetic MV who underwent cardiac CT from 2010 to 2017. The degree of LAC was analyzed and classified into three groups: group 1 (no LAC), group 2 (mild-to-moderate LAC), and group 3 (severe LAC). Clinical and echocardiographic characteristics and clinical outcomes were compared in three groups. LAC was seen in 79 (24.2%) patients. Groups 2 and 3 showed more prevalent atrial fibrillation, a rheumatic etiology, a higher number of previous surgeries, a larger LA volume index, and higher pulmonary artery systolic pressure than group 1. Paravalvular leakage of the MV increased progressively according to severity of LAC (15.4% in group 1, 39.3% in group 2, and 66.7% in group 3, p <0.001). Event-free survival rate for major adverse cardiovascular adverse events (log rank p = 0.033) and all-cause mortality (log rank p <0.001) were significantly different according to LAC group. In Cox regression analyses, presence of severe LAC was an independent predictor of all-cause mortality (hazard ratio: 4.44, 95% confidence interval: 1.71 to 11.58, p = 0.002). LAC on cardiac CT is not uncommon and reflects more advanced LA remodeling and a stiff LA. The presence and severity of LAC are associated with a worse clinical outcome after MV surgery.


Assuntos
Calcinose/epidemiologia , Cardiomiopatias/epidemiologia , Átrios do Coração/diagnóstico por imagem , Implante de Prótese de Valva Cardíaca , Anuloplastia da Valva Mitral , Insuficiência da Valva Mitral/cirurgia , Estenose da Valva Mitral/cirurgia , Complicações Pós-Operatórias/epidemiologia , Idoso , Fibrilação Atrial/epidemiologia , Calcinose/diagnóstico por imagem , Cardiomiopatias/diagnóstico por imagem , Causas de Morte , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mortalidade , Complicações Pós-Operatórias/diagnóstico por imagem , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Cardiopatia Reumática/cirurgia , Tomografia Computadorizada por Raios X
20.
Methodist Debakey Cardiovasc J ; 16(2): 97-105, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32670469

RESUMO

Cardiovascular magnetic resonance (CMR) has emerged as a key modality to assess nonischemic cardiomyopathies. Its ability to detect cardiac morphology and function with fast cine imaging, myocardial edema with T2-based techniques, and fibrosis with late gadolinium enhancement techniques has enabled noninvasive characterization of cardiac tissue, thus helping clinicians assess cardiovascular risk and determine the most effective management strategy. Active investigations into parametric imaging techniques will further expand the potential clinical applications of CMR for cardiac tissue characterization. This review discusses the use of CMR techniques in characterizing the major morphofunctional phenotypes of nonischemic cardiomyopathies.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Cardiomiopatias/etiologia , Humanos , Fenótipo , Valor Preditivo dos Testes
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