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Mitral valve prolapse is a common valve disorder that usually has a benign prognosis unless there is significant regurgitation or LV impairment. However, a subset of patients are at an increased risk of ventricular arrhythmias and sudden cardiac death, which has led to the recognition of "arrhythmic mitral valve prolapse" as a clinical entity. Emerging risk factors include mitral annular disjunction and myocardial fibrosis. While echocardiography remains the primary method of evaluation, cardiac magnetic resonance has become crucial in managing this condition. Cine magnetic resonance sequences provide accurate characterization of prolapse and annular disjunction, assessment of ventricular volumes and function, identification of early dysfunction and remodeling, and quantitative assessment of mitral regurgitation when integrated with flow imaging. However, the unique strength of magnetic resonance lies in its ability to identify tissue changes. T1 mapping sequences identify diffuse fibrosis, in turn related to early ventricular dysfunction and remodeling. Late gadolinium enhancement sequences detect replacement fibrosis, an independent risk factor for ventricular arrhythmias and sudden cardiac death. There are consensus documents and reviews on the use of cardiac magnetic resonance specifically in arrhythmic mitral valve prolapse. However, in this article, we propose an algorithm for the broader use of cardiac magnetic resonance in managing this condition in various scenarios. Future advancements may involve implementing techniques for tissue characterization and flow analysis, such as 4D flow imaging, to identify patients with ventricular dysfunction and remodeling, increased arrhythmic risk, and more accurate grading of mitral regurgitation, ultimately benefiting patient selection for surgical therapy.
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Prolapso da Valva Mitral , Prolapso da Valva Mitral/complicações , Prolapso da Valva Mitral/diagnóstico por imagem , Humanos , Imagem Cinética por Ressonância Magnética/métodos , Imageamento por Ressonância Magnética/métodos , Valva Mitral/diagnóstico por imagemRESUMO
Dystrophin (DMD) gene mutations are associated with skeletal muscle diseases such as Duchenne and Becker Muscular Dystrophy (BMD) and X-linked dilated cardiomyopathy (XL-DCM). To investigate the molecular basis of DCM in a 37-year-old woman. Clinical and genetic investigations were performed. Genetic testing was performed with whole exome sequencing (WES) using the Illumina platform. According to the standard protocol, a variant found by WES was confirmed in all available members of the family by bi-directional capillary Sanger resequencing. The effect of the variant was investigated by using an in silico prediction of pathogenicity. The index case was a 37-year-old woman diagnosed with DCM at the age of 33. A germline heterozygous A>G transversion at nucleotide 10103 in the DMD gene, leading to an aspartic acid-glycine substitution at the amino acid 3368 of the DMD protein (c.10103A>G p.Asp3368Gly), was identified and confirmed by PCR-based Sanger sequencing of the exon 70. In silico prediction suggests that this variant could have a deleterious impact on protein structure and functionality (CADD = 30). The genetic analysis was extended to the first-degree relatives of the proband (mother, father, and sister) and because of the absence of the variant in both parents, the p.Asp3368Gly substitution was considered as occurring de novo. Then, the direct sequencing analysis of her 8-year-old son identified as hemizygous for the same variant. The young patient did not present any signs or symptoms attributable to DCM, but reported asthenia and presented with bilateral calf hypertrophy at clinical examination. Laboratory testing revealed increased levels of creatinine kinase (maximum value of 19,000 IU/L). We report an early presentation of dilated cardiomyopathy in a 33-year-old woman due to a de novo pathogenic variant of the dystrophin (DMD) gene (p.Asp3368Gly). Genetic identification of this variant allowed an early diagnosis of a skeletal muscle disease in her son.
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Cardiomiopatia Dilatada , Distrofia Muscular de Duchenne , Humanos , Feminino , Adulto , Criança , Distrofina/genética , Cardiomiopatia Dilatada/genética , Distrofia Muscular de Duchenne/genética , MãesRESUMO
Background Scar burden with late gadolinium enhancement (LGE) cardiac MRI (CMR) predicts arrhythmic events in patients with postinfarction in single-center studies. However, LGE analysis requires experienced human observers, is time consuming, and introduces variability. Purpose To test whether postinfarct scar with LGE CMR can be quantified fully automatically by machines and to compare the ability of LGE CMR scar analyzed by humans and machines to predict arrhythmic events. Materials and Methods This study is a retrospective analysis of the multicenter, multivendor CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy (DERIVATE) registry. Patients with chronic heart failure, echocardiographic left ventricular ejection fraction (LVEF) of less than 50%, and LGE CMR were recruited (from January 2015 through December 2020). In the current study, only patients with ischemic cardiomyopathy were included. Quantification of total, dense, and nondense scars was carried out by two experienced readers or a Ternaus network, trained and tested with LGE images of 515 and 246 patients, respectively. Univariable and multivariable Cox analyses were used to assess patient and cardiac characteristics associated with a major adverse cardiac event (MACE). Area under the receiver operating characteristic curve (AUC) was used to compare model performances. Results In 761 patients (mean age, 65 years ± 11, 671 men), 83 MACEs occurred. With use of the testing group, univariable Cox-analysis found New York Heart Association class, left ventricle volume and/or function parameters (by echocardiography or CMR), guideline criterion (LVEF of ≤35% and New York Heart Association class II or III), and LGE scar analyzed by humans or the machine-learning algorithm as predictors of MACE. Machine-based dense or total scar conferred incremental value over the guideline criterion for the association with MACE (AUC: 0.68 vs 0.63, P = .02 and AUC: 0.67 vs 0.63, P = .01, respectively). Modeling with competing risks yielded for dense and total scar (AUC: 0.67 vs 0.61, P = .01 and AUC: 0.66 vs 0.61, P = .005, respectively). Conclusion In this analysis of the multicenter CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy (DERIVATE) registry, fully automatic machine learning-based late gadolinium enhancement analysis reliably quantifies myocardial scar mass and improves the current prediction model that uses guideline-based risk criteria for implantable cardioverter defibrillator implantation. ClinicalTrials.gov registration no.: NCT03352648 Published under a CC BY 4.0 license. Supplemental material is available for this article.
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Cicatriz , Meios de Contraste , Masculino , Humanos , Idoso , Volume Sistólico , Estudos Retrospectivos , Imagem Cinética por Ressonância Magnética/métodos , Gadolínio , Função Ventricular Esquerda , Imageamento por Ressonância Magnética/métodos , Sistema de Registros , Inteligência Artificial , Valor Preditivo dos TestesRESUMO
Although children with Covid-19 generally present with mild symptoms or are often asymptomatic, there is increasing recognition of a delayed multi-organ inflammatory syndrome (MIS-C) following SARS-CoV-2 infection. We report the case of MIS-C associated arrhythmic myocarditis which recovered after anti-inflammatory therapy and immunoglobulin infusion.
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COVID-19 , Miocardite , Adolescente , COVID-19/complicações , Criança , Humanos , Masculino , Miocardite/diagnóstico , Miocardite/etiologia , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/etiologiaRESUMO
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.
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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 EsquerdaRESUMO
INTRODUCTION: The immunosuppressive efficiency obtained in the last decades in kidney transplantation significantly improved graft survival. However, there is still a high risk and incidence of cancer in transplant patients strongly and directly related to the type of immunosuppression. An increasing body of evidence suggests that the PI3K/Akt/mTOR pathway may play a pivotal role in the development and progression of several neoplastic diseases. CASE PRESENTATION: We describe a 47-year-old male patient who received a cadaveric primary renal transplant in November 2008 developing a poorly differentiated infiltrating and ulcerated squamous cell carcinoma (SCC) at the eye level. In this patient, the modification of an immunosuppressive regimen with introduction of rapamycin (mTOR) inhibitors and withdrawal of calcineurin inhibitors (CNIs) led to the resolution of this severe condition. CONCLUSION: The introduction of mTOR inhibitors and withdrawal of CNIs in kidney-transplanted patients with de novo eye SCC should be considered in this clinical setting.
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Inibidores de Calcineurina/efeitos adversos , Carcinoma de Células Escamosas/etiologia , Neoplasias Oculares/etiologia , Imunossupressores/efeitos adversos , Transplante de Rim/efeitos adversos , Sirolimo/antagonistas & inibidores , Antineoplásicos/uso terapêutico , Carcinoma de Células Escamosas/tratamento farmacológico , Everolimo/uso terapêutico , Neoplasias Oculares/tratamento farmacológico , Humanos , Transplante de Rim/métodos , Masculino , Pessoa de Meia-Idade , Serina-Treonina Quinases TOR/antagonistas & inibidoresRESUMO
Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by neoplastic transformation of pluripotent cells due to a typical cytogenetic and molecular mutation known as Philadelphia (Ph) chromosome. In 2001, the introduction of the tyrosine kinasis inhibitor (TKI) imatinib as a therapeutic strategy for CML with PH chromosome mutation represented an important step towards treatment of these patients, and nowadays, this drug represents the gold therapeutic standard in this clinical setting. A second generation of TKIs (dasatinib, nilotinib, and bosutinib) showed an effective action in all patients with mutations resistant to imatinib. Ponatinib is a third-generation TKI and is the only inhibitor with activity against T3151 mutation. The impact of ponatinib on cardiovascular events was first evaluated in the PACE trial. We therefore report and discuss most relevant evidence currently available on cardiovascular events associated with the use of ponatinib. Though many exams can be used for diagnosis and follow-up of this kind of cardiotoxicity, echocardiography seems to have a pivotal role thanks to its feasibility, availability, and low cost.
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Doenças Cardiovasculares/diagnóstico , Gerenciamento Clínico , Diagnóstico Precoce , Imidazóis/efeitos adversos , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Piridazinas/efeitos adversos , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Seguimentos , Humanos , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Inibidores de Proteínas Quinases/efeitos adversos , Fatores de RiscoRESUMO
BACKGROUND: The presence of viable stunned myocardium recovering after primary angioplasty is not easy to identify in the early phase of acute myocardial infarction (AMI) by noninvasive bed-side methods. We therefore aimed to assess whether a simple electrocardiogram parameter may be of help in identifying the presence of stunned viable myocardium recovering after reperfusion with primary angioplasty. MATERIALS AND METHODS: A total of 14 consecutive patients with ST-elevation AMI (STEMI) were enrolled in the study and underwent QT duration assessment after admission: the difference between QT corrected (QTc) in the ischaemic areas and QTc values in nonischaemic areas was therefore calculated and compared with the presence and the extension of viable stunned myocardium, assessed by comparing akinetic/dyskinetic areas at admission echocardiography with akinetic/dyskinetic areas and extension of scar at 6-month cardiac magnetic resonance imaging (cMRI). RESULTS: In subjects with viable recovering myocardium, 75% had a QTc max > 440 ms (vs. 17%, P = 0·03); higher differential QTc values and smaller scar areas were found (33 ms vs. -17 ms, 14% vs. 27%, P = 0·03, 0·06 respectively). Differential QTc values > 0 were able to identify the presence of viable myocardium with an odds ratio of 35 (P < 0·05, sensitivity 88%, specificity 83%, positive predictive power 88%, negative predictive power of 83%). Differential QTc values were related to the extension of viable recovering myocardium (P < 0·001). CONCLUSION: Viable myocardium recovering after primary angioplasty in STEMI may be predicted by the presence of increased QTc values in ischaemic areas in comparison with nonischaemic areas.
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Síndrome do QT Longo/etiologia , Miocárdio Atordoado/etiologia , Intervenção Coronária Percutânea , Infarto do Miocárdio com Supradesnível do Segmento ST/cirurgia , Cicatriz/etiologia , Cicatriz/fisiopatologia , Eletrocardiografia , Humanos , Síndrome do QT Longo/fisiopatologia , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Miocárdio Atordoado/fisiopatologia , Recuperação de Função Fisiológica/fisiologia , Infarto do Miocárdio com Supradesnível do Segmento ST/fisiopatologia , Volume Sistólico/fisiologia , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
In recent years, the use of cardiac magnetic resonance (CMR) has grown exponentially in clinical practice. The keys for this success are represented by the possibility of tissue characterization, cardiac volumes and myocardial perfusion assessment, biventricular function evaluation, with no use of ionizing radiations and with an extremely interesting profile of reproducibility. The use of late gadolinium enhancement (LGE) nearly compares a non-invasive biopsy for cardiac fibrosis quantification. LGE, however, is partly unable to detect diffuse myocardial disease. These limits are overcome by new acquisition techniques, mainly T1 and T2 mapping, which allow the diagnosis and characterization of various cardiomyopathies, both ischemic and non-ischemic, such as amyloidosis (high T1), Fabry's disease (low T1), hemochromatosis (low T1), dilated and hypertrophic cardiomyopathy and myocarditis. In this review we detail and summarize principal evidence on the use of T1 and T2 mapping for the study and clinical management of cardiomyopathies.
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Left ventricular noncompaction (LVNC) is commonly described as a congenital cardiomyopathy characterized by prominent myocardial trabeculae and deep intertrabecular recesses extending in the left ventricular chamber. Clinical presentation can differ considerably from asymptomatic individuals to those presenting with heart failure and other serious complications. Diagnosis is usually made by two-dimensional transthoracic echocardiography or cardiac magnetic resonance. Moreover, even if strain parameters are significantly reduced in patients with LVNC, they are not routinely investigated. Here, we report the case of a previously symptomless patient admitted to the hospital for pulmonary edema. Two-dimensional transthoracic echocardiography showed severe valvular heart disease and left ventricle pronounced trabeculation and remodeling, although speckle tracking echocardiography (STE) demonstrated only mild strain reduction. We, therefore, explore the possibility that STE may be useful to differentiate LVNC cardiomyopathy from LVNC phenotype due to severe remodeling.
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Amyloidosis is a rare infiltrative condition resulting from the extracellular accumulation of amyloid fibrils at the cardiac level. It can be an acquired condition or due to genetic mutations. With the progression of imaging technologies, a non-invasive diagnosis was proposed. In this study, we discuss the role of CMR in cardiac amyloidosis, focusing on the two most common subtypes (AL and ATTR), waiting for evidence-based guidelines to be published.
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The complex pathobiology of lung cancer, and its spread worldwide, has prompted research studies that combine radiomic and genomic approaches. Indeed, the early identification of genetic alterations and driver mutations affecting the tumor is fundamental for correctly formulating the prognosis and therapeutic response. In this work, we propose a radiogenomic workflow to detect the presence of KRAS and EGFR mutations using radiomic features extracted from computed tomography images of patients affected by lung adenocarcinoma. To this aim, we investigated several feature selection algorithms to identify the most significant and uncorrelated sets of radiomic features and different classification models to reveal the mutational status. Then, we employed the SHAP (SHapley Additive exPlanations) technique to increase the understanding of the contribution given by specific radiomic features to the identification of the investigated mutations. Two cohorts of patients with lung adenocarcinoma were used for the study. The first one, obtained from the Cancer Imaging Archive (TCIA), consisted of 60 cases (25% EGFR, 23% KRAS); the second one, provided by the Azienda Ospedaliero-Universitaria 'Ospedali Riuniti' of Foggia, was composed of 55 cases (16% EGFR, 28% KRAS). The best-performing models proposed in our study achieved an AUC of 0.69 and 0.82 on the validation set for predicting the mutational status of EGFR and KRAS, respectively. The Multi-layer Perceptron model emerged as the top-performing model for both oncogenes, in some cases outperforming the state of the art. This study showed that radiomic features can be associated with EGFR and KRAS mutational status in patients with lung adenocarcinoma.
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Hypertrophic cardiomyopathy (HCM) is a cardiac muscle disorder characterized by generally asymmetric abnormal hypertrophy of the left ventricle without abnormal loading conditions (such as hypertension or valvular heart disease) accounting for the left ventricular wall thickness or mass. The incidence of sudden cardiac death (SCD) in HCM patients is about 1% yearly in adults, but it is far higher in adolescence. HCM is the most frequent cause of death in athletes in the Unites States of America. HCM is an autosomal-dominant genetic cardiomyopathy, and mutations in the genes encoding sarcomeric proteins are identified in 30-60% of cases. The presence of this genetic mutation carries more than 2-fold increased risk for all outcomes, including ventricular arrhythmias. Genetic and myocardial substrate, including fibrosis and intraventricular dispersion of conduction, ventricular hypertrophy and microvascular ischemia, increased myofilament calcium sensitivity and abnormal calcium handling, all play a role as arrhythmogenic determinants. Cardiac imaging studies provide important information for risk stratification. Transthoracic echocardiography can be helpful to evaluate left ventricular (LV) wall thickness, LV outflow-tract gradient and left atrial size. Additionally, cardiac magnetic resonance can evaluate the prevalence of late gadolinium enhancement, which when higher than 15% of LV mass is a prognostic maker of SCD. Age, family history of SCD, syncope and non-sustained ventricular tachycardia at Holter ECG have also been validated as independent prognostic markers of SCD. Arrhythmic risk stratification in HCM requires careful evaluation of several clinical aspects. Symptoms combined with electrocardiogram, cardiac imaging tools and genetic counselling are the modern cornerstone for proper risk stratification.
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PURPOSE: The Italian Registry of Contrast Material use in Cardiac Computed Tomography (iRCM-CCT) is a multicenter, multivendor, observational study on the use of contrast media (CM) in patients undergoing cardiac computed tomography (CCT). The aim of iRCM-CCT is to assess image quality and safety profile of intravenous CM compounds. MATERIALS AND METHODS: iRCM-CCT enrolled 1842 consecutive patients undergoing CCT (≥50 per site) at 20 cluster sites with the indication of suspected coronary artery disease. Demographic characteristics, CCT, and CM protocols, clinical indications, safety markers, radiation dose reports, qualitative (ie, poor vascular enhancement) and quantitative (ie, HU attenuation values) image parameters were recorded. A centralized coordinating center collected and assessed all image parameters. RESULTS: The cohort included 891 men and 951 women (age: 63±14 y, body mass index: 26±4 kg/m2) studied with ≥64 detector rows computed tomography scanners and different iodinated intravenous CM protocols and compounds (iodixanol, iopamidol, iohexol, iobitridol, iopromide, and iomeprol). The following vascular attenuation was reported: 504±147 HU in the aorta, 451±146 HU in the right coronary artery, 474±146 HU in the left main, 451±146 HU in the left anterior descending artery, and 441±149 HU in the circumflex artery. In 4% of cases the image quality was not satisfactory due to poor enhancement. The following adverse reactions to CM were recorded: 6 (0.3%) extravasations and 17 (0.9%) reactions (11 mild, 4 moderate, 2 severe). CONCLUSIONS: In a multicenter registry on CM use during CCT the prevalence of CM-related adverse reactions was very low. The appropriate use of CM is a major determinant of image quality.
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Meios de Contraste , Doença da Artéria Coronariana , Masculino , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Tomografia Computadorizada por Raios X/métodos , Angiografia Coronária/métodos , Sistema de RegistrosRESUMO
AIMS: Right ventricular systolic dysfunction (RVSD) is an important determinant of outcomes in heart failure (HF) cohorts. While the quantitative assessment of RV function is challenging using 2D-echocardiography, cardiac magnetic resonance (CMR) is the gold standard with its high spatial resolution and precise anatomical definition. We sought to investigate the prognostic value of CMR-derived RV systolic function in a large cohort of HF with reduced ejection fraction (HFrEF). METHODS AND RESULTS: Study cohort comprised of patients enrolled in the CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DefibrillAtor ThErapy registry who had HFrEF and had simultaneous baseline CMR and echocardiography (n = 2449). RVSD was defined as RV ejection fraction (RVEF) <45%. Kaplan-Meier curves and cox regression were used to investigate the association between RVSD and all-cause mortality (ACM). Mean age was 59.8 ± 14.0 years, 42.0% were female, and mean left ventricular ejection fraction (LVEF) was 34.0 ± 10.8. Median follow-up was 959 days (interquartile range: 560-1590). RVSD was present in 936 (38.2%) and was an independent predictor of ACM (adjusted hazard ratio = 1.44; 95% CI [1.09-1.91]; P = 0.01). On subgroup analyses, the prognostic value of RVSD was more pronounced in NYHA I/II than in NYHA III/IV, in LVEF <35% than in LVEF ≥35%, and in patients with renal dysfunction when compared to those with normal renal function. CONCLUSION: RV systolic dysfunction is an independent predictor of ACM in HFrEF, with a more pronounced prognostic value in select subgroups, likely reflecting the importance of RVSD in the early stages of HF progression.
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Cardiomiopatias , Desfibriladores Implantáveis , Insuficiência Cardíaca , Disfunção Ventricular Direita , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Masculino , Prognóstico , Volume Sistólico , Função Ventricular Esquerda , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/complicações , Desfibriladores Implantáveis/efeitos adversos , Fatores de Risco , Imagem Cinética por Ressonância Magnética/métodos , Cardiomiopatias/complicações , Espectroscopia de Ressonância Magnética/efeitos adversos , Função Ventricular Direita , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/terapia , Disfunção Ventricular Direita/etiologiaRESUMO
BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy is the most effective prophylactic strategy against sudden cardiac death (SCD) in patients with ischemic cardiomyopathy (ICM) and left ventricle ejection fraction (LVEF) ≤35% as detected by transthoracic echocardiograpgy (TTE). This approach has been recently questioned because of the low rate of ICD interventions in patients who received implantation and the not-negligible percentage of patients who experienced SCD despite not fulfilling criteria for implantation. OBJECTIVES: The DERIVATE-ICM registry (CarDiac MagnEtic Resonance for Primary Prevention Implantable CardioVerter DebrillAtor ThErapy; NCT03352648) is an international, multicenter, and multivendor study to assess the net reclassification improvement (NRI) for the indication of ICD implantation by the use of cardiac magnetic resonance (CMR) as compared to TTE in patients with ICM. METHODS: A total of 861 patients with ICM (mean age 65 ± 11 years, 86% male) with chronic heart failure and TTE-LVEF <50% participated. Major adverse arrhythmic cardiac events (MAACE) were the primary endpoints. RESULTS: During a median follow-up of 1,054 days, MAACE occurred in 88 (10.2%). Left ventricular end-diastolic volume index (HR: 1.007 [95% CI: 1.000-1.011]; P = 0.05), CMR-LVEF (HR: 0.972 [95% CI: 0.945-0.999]; P = 0.045) and late gadolinium enhancement (LGE) mass (HR: 1.010 [95% CI: 1.002-1.018]; P = 0.015) were independent predictors of MAACE. A multiparametric CMR weighted predictive derived score identifies subjects at high risk for MAACE compared with TTE-LVEF cutoff of 35% with a NRI of 31.7% (P = 0.007). CONCLUSIONS: The DERIVATE-ICM registry is a large multicenter registry showing the additional value of CMR to stratify the risk for MAACE in a large cohort of patients with ICM compared with standard of care.
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Cardiomiopatias , Desfibriladores Implantáveis , Isquemia Miocárdica , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Feminino , Desfibriladores Implantáveis/efeitos adversos , Meios de Contraste , Imagem Cinética por Ressonância Magnética , Valor Preditivo dos Testes , Gadolínio , Isquemia Miocárdica/complicações , Isquemia Miocárdica/diagnóstico por imagem , Isquemia Miocárdica/terapia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Cardiomiopatias/diagnóstico por imagem , Cardiomiopatias/terapia , Cardiomiopatias/complicações , Espectroscopia de Ressonância Magnética/efeitos adversos , Sistema de Registros , Fatores de RiscoAssuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico , Cardiomiopatia Hipertrófica/diagnóstico , Coração/diagnóstico por imagem , Feocromocitoma/diagnóstico , Cardiomiopatia de Takotsubo/diagnóstico , Neoplasias das Glândulas Suprarrenais/fisiopatologia , Biomarcadores/sangue , Dor no Peito/etiologia , Angiografia Coronária , Diagnóstico Diferencial , Ecocardiografia , Humanos , Síndrome do QT Longo/etiologia , Imageamento por Ressonância Magnética , Masculino , Feocromocitoma/fisiopatologia , Volume Sistólico , Taquicardia Sinusal/etiologia , Troponina I/sangue , Adulto JovemRESUMO
BACKGROUND: The introduction of imatinib and tyrosine kinase inhibitors as therapeutic strategy for Philadelphia chromosome-positive chronic myeloid leukaemia (CML) has represented an important step forward for treatment of this disease. The aim of this study was therefore to evaluate the incidence of cardiovascular adverse events (CVEs) in patients affected by CML treated with TKI in an observational prospective study. METHODS: All consecutive patients affected by CML and treated with TKI in our Institution were enrolled in the study from February 2005 to September 2018 with a clinical, laboratory and instrumental follow-up. RESULTS: Sixty-one consecutive patients were enrolled, 29 with imatinib, 15 with nilotinib, 11 with dasatinib, 3 with bosutinib and 3 with ponatinib. Neither patients in therapy with bosutinib nor with nilotinib had CVE during follow-up. Incidence rates per person/year were 0 for bosutinib and nilotinib, 0.15 for dasatinib, 0.19 for imatinib and 1.69 for ponatinib (Log Rank p < 0.05); differences in terms of incidence of adverse outcomes remained significant also after multivariate correction. CONCLUSIONS: In patients with CML treated with TKIs, therapy with ponatinib was associated with a higher risk of CVE than other TKIs. The lowest incidence of CVE was associated with bosutinib and nilotinib.