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2.
Europace ; 23(7): 1072-1083, 2021 07 18.
Artigo em Inglês | MEDLINE | ID: mdl-33792661

RESUMO

AIMS: The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients. METHODS AND RESULTS: In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117-1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211-3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231-3.690, P = 0.007; HR: 3.161, 95% CI: 1.750-5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084-2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort. CONCLUSION: In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation.


Assuntos
Cardiomiopatia Dilatada , Desfibriladores Implantáveis , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/terapia , Meios de Contraste , Feminino , Gadolínio , Humanos , Imagem Cinética por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Masculino , Valor Preditivo dos Testes , Prognóstico , Sistema de Registros , Volume Sistólico , Função Ventricular Esquerda
5.
Trends Cardiovasc Med ; 31(6): 370-379, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-32497572

RESUMO

Acute myocarditis (AM), a recent-onset inflammation of the heart, has heterogeneous clinical presentations, varying from minor symptoms to high-risk cardiac conditions with severe heart failure, refractory arrhythmias, and cardiogenic shock. AM is moving from being a definitive diagnosis based on histological evidence of inflammatory infiltrates on cardiac tissue to a working diagnosis supported by high sensitivity troponin increase in association with specific cardiac magnetic resonance imaging (CMRI) findings. Though experts still diverge between those advocating for histological definition versus those supporting a mainly clinical definition of myocarditis, in the real-world practice the diagnosis of AM has undoubtedly shifted from being mainly biopsy-based to solely CMRI-based in most of clinical scenarios. It is thus important to clearly define selected settings where EMB is a must, as information derived from histology is essential for an optimal management. As in other medical conditions, a risk-based approach should be promoted in order to identify the most severe AM cases requiring appropriate bundles of care, including early recognition, transfer to tertiary centers, aggressive circulatory supports with inotropes and mechanical devices, histologic confirmation and eventual immunosuppressive therapy. Despite improvements in recognition and treatment of AM, including a broader use of promising mechanical circulatory supports, severe forms of AM are still burdened by dismal outcomes. This review is focused on recent clinical studies and registries that shed new insights on AM. Attention will be paid to contemporary outcomes and predictors of prognosis, the emerging entity of immune checkpoint inhibitors-associated myocarditis, updated CMRI diagnostic criteria, new data on the use of temporary mechanical circulatory supports in fulminant myocarditis. The role of viruses as etiologic agents will be reviewed and a brief update on pediatric AM is also provided. Finally, we summarize a risk-based approach to AM, based on available evidence and clinical experience.


Assuntos
Cardiotônicos/uso terapêutico , Coração Auxiliar , Miocardite , Implantação de Prótese/instrumentação , Doença Aguda , Cardiotônicos/efeitos adversos , Técnicas de Apoio para a Decisão , Humanos , Inibidores de Checkpoint Imunológico/efeitos adversos , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Miocardite/diagnóstico por imagem , Miocardite/etiologia , Miocardite/imunologia , Miocardite/terapia , Implantação de Prótese/efeitos adversos , Medição de Risco , Fatores de Risco , Resultado do Tratamento , Viroses/virologia
7.
Circ Heart Fail ; 13(11): e007405, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33176455

RESUMO

Myocarditis is an inflammatory disease of the heart that may occur because of infections, immune system activation, or exposure to drugs. The diagnosis of myocarditis has changed due to the introduction of cardiac magnetic resonance imaging. We present an expert consensus document aimed to summarize the common terminology related to myocarditis meanwhile highlighting some areas of controversies and uncertainties and the unmet clinical needs. In fact, controversies persist regarding mechanisms that determine the transition from the initial trigger to myocardial inflammation and from acute myocardial damage to chronic ventricular dysfunction. It is still uncertain which viruses (besides enteroviruses) cause direct tissue damage, act as triggers for immune-mediated damage, or both. Regarding terminology, myocarditis can be characterized according to etiology, phase, and severity of the disease, predominant symptoms, and pathological findings. Clinically, acute myocarditis (AM) implies a short time elapsed from the onset of symptoms and diagnosis (generally <1 month). In contrast, chronic inflammatory cardiomyopathy indicates myocardial inflammation with established dilated cardiomyopathy or hypokinetic nondilated phenotype, which in the advanced stages evolves into fibrosis without detectable inflammation. Suggested diagnostic and treatment recommendations for AM and chronic inflammatory cardiomyopathy are mainly based on expert opinion given the lack of well-designed contemporary clinical studies in the field. We will provide a shared and practical approach to patient diagnosis and management, underlying differences between the European and US scientific statements on this topic. We explain the role of histology that defines subtypes of myocarditis and its prognostic and therapeutic implications.


Assuntos
Cardiologia/normas , Miocardite/terapia , Doença Aguda , Doença Crônica , Consenso , Humanos , Miocardite/diagnóstico , Miocardite/epidemiologia , Miocardite/imunologia , Valor Preditivo dos Testes , Fatores de Risco , Terminologia como Assunto , Resultado do Tratamento
8.
G Ital Cardiol (Rome) ; 21(12): 969-989, 2020 Dec.
Artigo em Italiano | MEDLINE | ID: mdl-33231216

RESUMO

Myocarditis is an inflammatory heart disease that can occur acutely, as in acute myocarditis, or persistently, as in chronic myocarditis or chronic inflammatory cardiomyopathy. Different agents can induce myocarditis, with viruses being the most common triggers. Generally, acute myocarditis affects relatively young people and men more than women. Myocarditis has a broad spectrum of clinical presentations and evolution trajectories, although most cases resolve spontaneously. Patients with reduced left ventricular ejection fraction, heart failure symptoms, advanced atrioventricular block, sustained ventricular arrhythmias or cardiogenic shock (the latter known as fulminant myocarditis) are at increased risk for death and heart transplantation. The presentation of chronic inflammatory cardiomyopathy may be more subtle, with progressive symptoms of heart failure or appearance of rhythm disturbance, not rarely preceded by an infective episode. Autoimmune disorder or systemic inflammatory conditions can be another significant predisposing substrate of myocarditis, especially in women. Emerging causes of myocarditis are drug-related like the new anticancer therapies, the immune checkpoint inhibitors. In this Italian Association of Hospital Cardiologists (ANMCO) and Italian Society of Cardiology (SIC) expert consensus document on myocarditis, we propose diagnostic strategies for identifying possible causes of the disease and factors associated with increased risk. Finally, we propose potential treatments and when referring patients to tertiary centers, especially for high-risk patients. Even if endomyocardial biopsy is the invasive diagnostic tool for making definitive diagnosis and differentiation of histological subtypes (i.e., lymphocytic vs eosinophilic vs giant cell myocarditis), it is not always readily available in all centers. Thus, we propose when this exam is mandatory or when it can be postponed or substituted by cardiac magnetic resonance imaging. This document reflects the Italian perspective on managing patients with myocarditis and their follow-up, considering also current US and European scientific position statements.


Assuntos
Cardiologia , Miocardite , Adolescente , Biópsia , Consenso , Feminino , Humanos , Masculino , Miocardite/diagnóstico , Miocardite/etiologia , Miocardite/terapia , Volume Sistólico , Função Ventricular Esquerda
10.
Ann Hematol ; 99(9): 2065-2072, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32572524

RESUMO

Sickle hepatopathy is a severe and not rare complication of sickle cell disease (SCD), showing mainly a cholestatic pattern. So far, no effective approaches to prevent or treat this condition have been recognized. We conducted a single-center observational study in 68 adult sickle cell patients, encompassing 17 with sickle cell anemia (SCA), 38 with sickle cell thalassemia (HbS/ß-Thal), and 13 with HbSC disease. The aim of our study was to assess liver damage in the three main forms of SCD, through the evaluation of clinical, laboratory, and imaging findings. In our population, the role of hepatotropic viruses, high BMI, and alcohol consumption in liver damage was ruled out. SCA and HbS/ß-Thal patients with lower Hb (p < 0.001), higher HbS (p < 0.001), and frequent vaso-occlusive crises showed functional (GGT values: SCA and HbS/ß-Thal vs HbSC p = 0.047 and p = 0.009, respectively) and structural liver abnormalities, defined by abdominal ultrasound and vibration-controlled transient elastography (liver stiffness values: SCA and HbS/ß-Thal vs HbSC p 0.022 and p 0.19, respectively), more severe than HbSC patients. Through univariate and multivariate analyses, male sex, SCA genotype, lower HbF, frequent transfusions, increased GGT values, and abnormal liver ultrasound and stiffness were identified as potentially early markers of sickle hepatopathy.


Assuntos
Anemia Falciforme/sangue , Anemia Falciforme/diagnóstico por imagem , Genótipo , Hepatopatias/sangue , Hepatopatias/diagnóstico por imagem , Adulto , Anemia Falciforme/genética , Feminino , Humanos , Hepatopatias/genética , Masculino , Pessoa de Meia-Idade , Fatores de Risco
11.
Eur Heart J Acute Cardiovasc Care ; 9(7): NP3-NP7, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27221956

RESUMO

We report the case of a 23-year-old man who developed an acute ST-elevation myocardial infarction secondary to acute thrombotic occlusion of the proximal left anterior descending coronary artery five years after undergoing chemotherapy, radiotherapy, haematopoietic stem cell transplantation for acute lymphoblastic leukaemia and bulky mediastinal mass involving the pleura and pericardium. His medical history also included Graft versus Host Disease developed 13 months after transplantation and acute myocarditis three months before the actual hospital admission. To the best of our knowledge, coronary artery disease as a complication of haematopoietic stem cell transplantation and low-dose mediastinal radiation therapy in young patients has been rarely reported in the medical literature. Clinicians should have a high degree of suspicion of coronary artery disease in patients treated with allogeneic haematopoietic stem cell transplantation, especially in patients previously treated with target mediastinal radiotherapy, as a group at risk of premature and significantly accelerated atherosclerosis, in order to make a timely and correct diagnosis.


Assuntos
Aterosclerose/complicações , Transplante de Células-Tronco de Sangue Periférico/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgia , Infarto do Miocárdio com Supradesnível do Segmento ST/etiologia , Aterosclerose/diagnóstico , Angiografia Coronária , Eletrocardiografia , Humanos , Masculino , Fatores de Risco , Infarto do Miocárdio com Supradesnível do Segmento ST/diagnóstico , Adulto Jovem
12.
Am J Cardiol ; 125(1): 146-151, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31711636

RESUMO

Cardiac magnetic resonance (CMR) offers the capability to objectively detect pericarditis by identifying pericardial thickening, edema/inflammation by Short-TI Inversion Recovery-T2 weighted (STIR-T2w) imaging, edema/inflammation or fibrosis by late gadolinium enhancement (LGE), and presence of pericardial effusion. This is especially helpful for the diagnosis of recurrent pericarditis. Aim of the present paper is to assess the diagnostic accuracy of CMR findings as well as their potential prognostic value for the diagnosis of recurrent pericarditis. Multicenter cohort study of consecutive patients with recurrent pericarditis evaluated by CMR. We included 128 consecutive cases (60 males, 47%; mean age 48 ± 14 years). CMR was performed at a mean time of 12 days (95% confidence interval 15 to 21) after the clinical diagnosis. We evaluated the diagnostic accuracy and areas under the receiver operating characteristic (ROC) curve for CMR diagnostic criteria and complications (additional recurrences, cardiac tamponade, and constrictive pericarditis). Areas under the ROC curve were respectively 64% for pericardial thickening, 84% for pericardial edema, 82% for pericardial LGE, and 71% for pericardial effusion. After a mean follow-up of 34 months, recurrences occurred in 52% of patients, tamponade in 6%, and constrictive pericarditis in 11%. Using a multivariable Cox model, elevation of CRP and presence of CMR pericardial thickening were predictors of adverse events, whereas the presence of CMR LGE was associated with a lower risk. The prognostic model for adverse events using gender, age, CRP level, and all CMR variables showed a C-index of 0.84. In conclusion, CMR findings show high diagnostic accuracy and may help identifying patients at higher risk of complications.


Assuntos
Imagem Cinética por Ressonância Magnética/métodos , Pericardite/diagnóstico , Pericárdio/patologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Curva ROC , Recidiva , Estudos Retrospectivos , Índice de Gravidade de Doença
13.
G Ital Cardiol (Rome) ; 20(9): 499-511, 2019 Sep.
Artigo em Italiano | MEDLINE | ID: mdl-31530951

RESUMO

The term MINOCA (myocardial infarction with non-obstructive coronary arteries) defines acute myocardial infarction with angiographic evidence of no significant coronary artery stenosis. Heterogeneous diseases are labelled as MINOCA. Incidence and epidemiological aspects differ on the basis of etiological causes. MINOCA include plaque (causing <50% stenosis) rupture or erosion, coronary embolism and dissection, and coronary artery spasm. Diagnosis may require multiple diagnostic tools, including cardiac imaging or provocative tests, in addition to standard coronary angiography, according to clinical suspicion. Cardiac magnetic resonance plays a key role in confirming the diagnosis and excluding other diseases with similar clinical presentation. Prognosis is not as benign as previously thought, on the opposite it is characterized by morbidity and mortality rates similar to other forms of myocardial infarction. Once the causative mechanism has been identified, appropriate therapy can be delivered.


Assuntos
Infarto do Miocárdio , Vasos Coronários/diagnóstico por imagem , Árvores de Decisões , Humanos , Infarto do Miocárdio/diagnóstico , Infarto do Miocárdio/etiologia , Infarto do Miocárdio/terapia , Prognóstico
16.
Br J Haematol ; 185(3): 523-531, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30828802

RESUMO

Iron overload (IO) is poorly investigated in the congenital haemolytic anaemias (CHAs), a heterogeneous group of rare inherited diseases encompassing abnormalities of the erythrocyte membrane and metabolism, and defects of the erythropoiesis. In this study we systematically evaluated routine iron parameters and cardiac and hepatic magnetic resonance imaging, together with erythropoietin, hepcidin, non-transferrin bound iron (NTBI), and cytokine serum levels in patients with different CHAs. We found that 40% of patients had a liver iron concentration (LIC) >4 mg Fe/g dry weight. Hepatic IO was associated with ferritin levels (P = 0·0025), transferrin saturation (TfSat, P = 0·002) and NTBI (P = 0·003). Moreover, ferritin >500 µg/l plus TfSat >60% was demonstrated as the best combination able to identify increased LIC, and TfSat alteration as more important in cases with discordant values. Possible confounding factors, such as transfusions, hepatic disease, metabolic syndrome and hereditary haemochromatosis-associated mutations, had negligible effects on IO. Erythropoietin and hepcidin levels were increased in CHAs compared with controls, correlating with LIC and ferritin, respectively. Regarding cytokines, γ-interferon (IFN-γ) was increased, and both interleukin 6 and IFN-γ levels positively correlated with ferritin and hepcidin levels. Overall, these findings suggest the existence of a vicious cycle between chronic haemolysis, inflammatory response and IO in CHAs.


Assuntos
Anemia Hemolítica Congênita , Ferritinas/sangue , Hepcidinas/sangue , Interferon gama/sangue , Interleucina-6/sangue , Sobrecarga de Ferro , Transferrina/metabolismo , Adolescente , Adulto , Anemia Hemolítica Congênita/sangue , Anemia Hemolítica Congênita/complicações , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Sobrecarga de Ferro/sangue , Sobrecarga de Ferro/etiologia , Masculino , Valor Preditivo dos Testes
17.
G Ital Cardiol (Rome) ; 20(1): 8-19, 2019 Jan.
Artigo em Italiano | MEDLINE | ID: mdl-30638211

RESUMO

Cardiac magnetic resonance is an accurate and versatile tool for multiparametric morphologic and functional evaluation of the heart and great vessels, with a wide range of clinical applications: from acute and chronic ischemic heart disease to the assessment of the substrate of complex ventricular arrhythmias and the follow-up of patients with valvular and congenital heart disease. The accuracy in cardiac volume and ejection fraction quantification, tissue characterization, valvular regurgitant fraction and cardiac shunt assessment, pharmachologic stress myocardial perfusion and three-dimensional reconstruction of great vessels are the points of strength that have made Cardiac magnetic resonance an invaluable tool for diagnostic, classification and follow-up of patients with various cardiac diseases.


Assuntos
Doenças Cardiovasculares/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Volume Cardíaco/fisiologia , Doenças Cardiovasculares/fisiopatologia , Humanos , Imageamento Tridimensional/métodos , Reprodutibilidade dos Testes , Volume Sistólico/fisiologia
18.
G Ital Cardiol (Rome) ; 19(12): 679-691, 2018 Dec.
Artigo em Italiano | MEDLINE | ID: mdl-30520882

RESUMO

Cardiac magnetic resonance (CMR) has proved to be a powerful tool in the assessment of several cardiac diseases, thanks to its capability to offer multiparametric morphologic and functional evaluation of the heart and great vessels, using neither ionizing radiations nor nephrotoxic contrast medium. The accuracy in quantification of cardiac volumes and ejection fraction (gold standard) together with native and post-contrast myocardial tissue characterization have made CMR an invaluable tool for the diagnosis, prognosis and therapeutic planning in patients with heart failure and cardiomyopathy.


Assuntos
Cardiomiopatias/diagnóstico por imagem , Insuficiência Cardíaca/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Humanos , Prognóstico , Reprodutibilidade dos Testes
20.
Circulation ; 138(11): 1088-1099, 2018 09 11.
Artigo em Inglês | MEDLINE | ID: mdl-29764898

RESUMO

BACKGROUND: There is controversy about the outcome of patients with acute myocarditis (AM), and data are lacking on how patients admitted with suspected AM are managed. We report characteristics, in-hospital management, and long-term outcome of patients with AM based on a retrospective multicenter registry from 19 Italian hospitals. METHODS: A total of 684 patients with suspected AM and recent onset of symptoms (<30 days) were screened between May 2001 and February 2017. Patients >70 years of age and those >50 years of age without coronary angiography were excluded. The final study population comprised 443 patients (median age, 34 years; 19.4% female) with AM diagnosed by either endomyocardial biopsy or increased troponin plus edema and late gadolinium enhancement at cardiac magnetic resonance. RESULTS: At presentation, 118 patients (26.6%) had left ventricular ejection fraction <50%, sustained ventricular arrhythmias, or a low cardiac output syndrome, whereas 325 (73.4%) had no such complications. Endomyocardial biopsy was performed in 56 of 443 (12.6%), and a baseline cardiac magnetic resonance was performed in 415 of 443 (93.7%). Cardiac mortality plus heart transplantation rates at 1 and 5 years were 3.0% and 4.1%. Cardiac mortality plus heart transplantation rates were 11.3% and 14.7% in patients with complicated presentation and 0% in uncomplicated cases (log-rank P<0.0001). Major AM-related cardiac events after the acute phase (postdischarge death and heart transplantation, sustained ventricular arrhythmias treated with electric shock or ablation, symptomatic heart failure needing device implantation) occurred in 2.8% at the 5-year follow-up, with a higher incidence in patients with complicated forms (10.8% versus 0% in uncomplicated AM; log-rank P<0.0001). ß-Adrenoceptor blockers were the most frequently used medications both in complicated (61.9%) and in uncomplicated forms (53.8%; P=0.18). After a median time of 196 days, 200 patients had follow-up cardiac magnetic resonance, and 8 of 55 (14.5%) with complications at presentation had left ventricular ejection fraction <50% compared with 1 of 145 (0.7%) of those with uncomplicated presentation. CONCLUSIONS: In this contemporary study, overall serious adverse events after AM were lower than previously reported. However, patients with left ventricular ejection fraction <50%, ventricular arrhythmias, or low cardiac output syndrome at presentation were at higher risk compared with uncomplicated cases that had a benign prognosis and low risk of subsequent left ventricular systolic dysfunction.


Assuntos
Miocardite , Doença Aguda , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Biópsia , Fármacos Cardiovasculares/uso terapêutico , Feminino , Transplante de Coração , Mortalidade Hospitalar , Hospitalização , Humanos , Itália , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Miocardite/diagnóstico , Miocardite/mortalidade , Miocardite/fisiopatologia , Miocardite/terapia , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Volume Sistólico , Fatores de Tempo , Resultado do Tratamento , Troponina/sangue , Função Ventricular Esquerda , Adulto Jovem
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