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Background: The exact mechanisms underlying the pathogenesis of myocarditis are not always understood, but there is emerging evidence to suggest that genetic factors may play a significant role. Case summary: Herein, we present six cases in which clinical, biochemical, and cardiovascular magnetic resonance data were consistent with myocarditis, and genetic testing subsequently revealed pathogenic filamin C (FLNC) mutations. Three patients presented with ventricular arrhythmias, two with severe biventricular dysfunction, and two suffered sudden cardiac arrest. Three received an implantable cardioverter defibrillator, and one underwent heart transplantation. Cascade testing was useful in identifying other relatives with FLNC mutation. We also present relevant histology results of myocardial specimens showing the presence of lymphocytic infiltration and inflammation, further supporting the potential association between FLNC mutations and a myocarditis phenotype. Discussion: Genetic testing of affected individuals for FLNC mutations and cascade screening in the setting of acute myocarditis may be considered in selected clinical context, such as in acute myocarditis accompanied by severe left ventricular systolic dysfunction, biventricular failure, significant ventricular arrhythmias, or right ventricular involvement.
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The following letter presents an answer of a comment of our work titled "Ross procedure: valve function, clinical outcomes and predictors after 25 years' follow-up," recently published in your journal by Rangwala et al.1 As our colleagues point out, the Ross procedure has excellent survival rates but a significant risk of valve dysfunction and therefore reintervention at follow-up. Although the survival advantage with the Ross procedure appears to be consistent compared with mechanical valve substitutes, this benefit is not as clear compared with biological valve substitutes. However, biological valve substitutes also have significant reintervention rates during follow-up. The different surgical modifications of the Ross procedure have not clearly demonstrated better results in follow-up in terms of autograft reintervention. This procedure can be performed in a medium-volume center with good results as long as adequate patient selection and adequate surgical training are carried out.
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Válvula Aórtica , Humanos , Resultado del Tratamiento , Válvula Aórtica/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Válvula Pulmonar/cirugía , Válvula Pulmonar/trasplante , Prótesis Valvulares Cardíacas , Estudios de Seguimiento , Bioprótesis , Procedimientos Quirúrgicos Cardíacos/métodos , Reoperación/estadística & datos numéricos , Enfermedades de las Válvulas Cardíacas/cirugíaRESUMEN
INTRODUCTION AND OBJECTIVES: Hospitalization for heart failure (HHF) is common in patients with atrial fibrillation (AF) and is associated with increased mortality. The aims of this study were to determine the incidence of HHF, identify the clinical predictors of its occurrence, and develop a new risk scale. METHODS: The incidence of HHF was estimated using data from the prospective single-center REFLEJA registry of outpatients with AF (October 2017-October 2018). A multivariate Cox regression model was calculated to detect HHF predictors, and a nomogram was created for individual risk assessment. RESULTS: Of the 1499 patients included (mean age 73.8±11.1 years, 48.1% women), 127 had HHF (incidence rate of 8.51 per 100 persons/y) and 319 died (rate of death from any cause of 21.1 per 100 persons/y) after a 3-year follow-up. The independent predictors of HHF were age, diabetes, chronic kidney disease, pulmonary hypertension, previous pacemaker implantation, baseline use of diuretics, and moderate-severe aortic regurgitation. The c-statistic for predicting the event was 0.762 (95%CI after boostrapping resampling, 0.753-0.791). The cumulative incidences of the main outcome for the risk scale quartiles were 1.613 (Q1), 3.815 (Q2), 8.378 (Q3), and 20.436 (Q4) cases per 100 persons/y (P <.001). CONCLUSIONS: HHF was common in this AF cohort. The combination of certain clinical characteristics can identify patients with a very high risk of HHF.
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OBJECTIVE: To describe long-term outcomes of the Ross procedure in a single center and retrospective series after 25 years follow-up. METHODS: From 1997-2019 we included all consecutive patients who underwent Ross procedure at our center. Clinical and echocardiographic evaluations were performed at least yearly. Echocardiographic valvular impairment was defined as at least moderate autograft or homograft dysfunction. Reintervention outcomes included surgical and percutaneous approach. RESULTS: 151 Ross procedures were performed (mean age 28±12years, 21 %<16years, 70 %male). After 25 years follow-up (median 18 years, interquartile range 9-21, only 3 patients lost) 12 patients died (8 %); Autograft, homograft or any valve dysfunction were present in 38(26 %), 48(32 %) and 75(51 %), respectively; and reintervention in 22(15%), 17(11%) and 38(26 %) respectively. At 20 years of follow-up, probabilities of survival free from autograft, homograft or any valve dysfunction were 63 %, 60 % and 35 %; and from reintervention, 80 %, 85 % and 67 %, respectively. The learning curve period (first 12 cases) was independently associated to autograft dysfunction (HR 2.78, 95 %CI:1.18-6.53, p = 0.02) and reintervention (HR 3.76, 95 %CI: 1.46-9.70, p = 0.006). Larger native pulmonary diameter was also an independent predictor of autograft reintervention (HR 1.22, 95 %CI:1.03-1.45, p = 0.03). Homograft dysfunction was associated with younger age (HR 5.35, 95 %CI: 2.13-13.47, p<0.001) and homograft reintervention, with higher left ventricle ejection fraction (HR 1,10, 95 %CI:1.02-1.19, p<0.02). CONCLUSIONS: In this 25 years' experience after the Ross procedure, global survival was high, although autograft and homograft dysfunction and reintervention rates were not negligible. Clinical and echocardiographic variables can identify patients with higher risk of events in follow up.
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Muerte , Ecocardiografía , Humanos , Masculino , Adolescente , Adulto Joven , Adulto , Estudios de Seguimiento , Estudios Retrospectivos , Volumen SistólicoRESUMEN
Introduction Our objective was to determine, in "real life" patients, the prevalence of massive and torrential regurgitation among patients diagnosed with severe tricuspid regurgitation (TR), as well as its impact on long-term prognosis. Methods In a single-center retrospective study, all patients with an echocardiographic diagnosis of severe TR attended at a tertiary care hospital of an European country from January 2008 to December 2017 were recruited. Images were analysed off-line to measure the maximum vena contracta (VC) and TR was classified into three groups: severe (VC ≥ 7 mm), massive (VC 14-20 mm), and torrential (VC ≥ 21 mm). The impact of this classification on the combined event of heart failure (HF) admission and all-cause death in follow-up was investigated. Results A total of 614 patients (70 ± 13 years, 72 % women) were included. 81.4 % had severe TR, 15.8 % massive TR, and 2.8 % torrential TR. The 5-year HF-free survival was 42 %, 43 %, and 12 % (p = 0.001), for the different subgroups of severe TR, respectively. After adjusting for baseline characteristics, TR severity was an independent predictor of survival free of the combined end-point: HR 0.91 [95 % CI 0.70-1.18] p = 0.46, for massive TR; and HR 2.5 [95 % CI 1.49-4.21] p = 0.001, for torrential TR considering severe TR as reference. Conclusions The prevalence of massive and torrential TR is not negligible among patients with severe TR in real life. The prognosis is significantly worse for patients with torrential TR measured by the maximum VC.
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Insuficiencia Cardíaca , Insuficiencia de la Válvula Tricúspide , Humanos , Femenino , Masculino , Insuficiencia de la Válvula Tricúspide/diagnóstico por imagen , Insuficiencia de la Válvula Tricúspide/epidemiología , Pronóstico , Estudios Retrospectivos , Prevalencia , Índice de Severidad de la Enfermedad , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/epidemiología , Insuficiencia Cardíaca/complicacionesRESUMEN
INTRODUCTION: Our aim was to investigate the prevalence of atrial fibrillation (AF) and recently diagnosed lung cancer in the outpatient oncology clinic and to describe the clinical profile, management and outcomes of this population. METHODS: Among 6984 patients visited at the outpatient oncology clinics attending lung cancer patients in five university hospitals from 2017 to 2019, all consecutive subjects with recently diagnosed (<1 year) disease and AF were retrospectively selected and events in follow up were registered. RESULTS: A total of 269 patients (3.9 % of all attended, 71 ± 8 years, 91 % male) were included. Charlson, CHA2DS2-VASc and HAS-BLED indexes were 6.7 ± 2.9, 2.9 ± 1.5 y 2.5 ± 1.2, respectively. Tumour stage was I, II, III and IV in 11 %, 11 %, 33 % and 45 % of them, respectively. Anticoagulants were prescribed to 226 patients (84 %): direct anticoagulants (n = 99;44 %), low molecular weight heparins (n = 69;30 %) and vitamin K antagonists (n = 58;26 %). After 46 months of maximum follow-up, 186 patients died (69 %). Cumulative incidences of events at 3 years were 3.3 ± 1.3 % for stroke/systemic embolism (n = 7); 8.9 ± 2.2 % for thrombotic events (n = 18); 9.9 ± 2.6 % for major bleeding (n = 16), and 15.9 ± 3,0 % for cardiovascular events (n = 33). In patients with early stages of cancer (I-II), 2-year mortality was significantly higher in those with cardiovascular events or major bleeding (85 % vs 25 %, p = 0.01). CONCLUSION: Nearly 4 % or all outpatients in the oncology clinic attending lung cancer present recently diagnosed disease and AF. Major bleeding and cardiovascular event rates are high in this population, with an impact on mortality in early stages of cancer.
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Fibrilación Atrial , Neoplasias Pulmonares , Accidente Cerebrovascular , Humanos , Masculino , Femenino , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/epidemiología , Pacientes Ambulatorios , Estudios Retrospectivos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/epidemiología , Neoplasias Pulmonares/inducido químicamente , Hemorragia/inducido químicamente , Accidente Cerebrovascular/epidemiología , Anticoagulantes/uso terapéutico , Factores de Riesgo , Medición de RiesgoRESUMEN
BACKGROUND: Worsening heart failure (WFH) includes heart failure (HF) hospitalisation, representing a strong predictor of mortality in patients with heart failure with reduced ejection fraction (HFrEF). However, there is little evidence analysing the impact of the number of previous HF admissions. Our main objective was to analyse the clinical profile according to the number of previous admissions for HF and its prognostic impact in the medium and long term. METHODS: A retrospective study of a cohort of patients with HFrEF, classified according to previous admissions: cohort-1 (0-1 previous admission) and cohort-2 (≥2 previous admissions). Clinical, echocardiographic and therapeutic variables were analysed, and the medium- and long-term impacts in terms of hospital readmissions and cardiovascular mortality were assessed. A total of 406 patients were analysed. RESULTS: The mean age was 67.3 ± 12.6 years, with male predominance (73.9%). Some 88.9% (361 patients) were included in cohort-1, and 45 patients (11.1%) were included in cohort-2. Cohort-2 had a higher proportion of atrial fibrillation (49.9% vs. 73.3%; p = 0.003), chronic kidney disease (36.3% vs. 82.2%; p < 0.001), and anaemia (28.8% vs. 53.3%; p = 0.001). Despite having similar baseline ventricular structural parameters, cohort-1 showed better reverse remodelling. With a median follow-up of 60 months, cohort-1 had longer survival free of hospital readmissions for HF (37.5% vs. 92%; p < 0.001) and cardiovascular mortality (26.2% vs. 71.9%; p < 0.001), with differences from the first month. CONCLUSIONS: Patients with HFrEF and ≥2 previous admissions for HF have a higher proportion of comorbidities. These patients are associated with worse reverse remodelling and worse medium- and long-term prognoses from the early stages, wherein early identification is essential for close follow-up and optimal intensive treatment.
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BACKGROUND AND OBJECTIVES: Research on the relationship between body mass index (BMI) and strain values in children and adolescents is limited. Our aim was to analyse the relationship between BMI and strain values of both ventricles and left atrium in children and adolescents. METHODS: Both ventricles and left atrial strain values were compared among different BMI categories in children and adolescents from a town in the South of Spain. RESULTS: Of the 198 subjects, aged 6-17 years, 53% were of normal weight, 26% were overweight and 21% had obesity. Lower absolute values of left ventricular global longitudinal strain (25.9 ± 2.0% vs. 26.9 ± 2.2%, p = 0.002) and right ventricular free wall longitudinal strain (29.5 ± 4.2% vs. 30.8 ± 4.5%, p = 0.04) were found in subjects with obesity and overweight versus subjects with normal weight. A lower right ventricular four-chamber longitudinal strain was also observed in males with obesity and overweight (24.8 ± 3.3% vs. 26.4 ± 3.6%, p = 0.03). Statistically significant negative correlations of BMI were found for all ventricular, but not atrial, strain values in univariate analysis. This association turned non-significant for right ventricular four-chamber longitudinal strain in multivariate analysis. CONCLUSIONS: Utilizing this new strain software, children and adolescents with high BMI were associated with significantly lower values for left and right ventricular free wall longitudinal strain, without impact in left atrial strain.
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Sobrepeso , Población Rural , Masculino , Humanos , Niño , Adolescente , Sobrepeso/complicaciones , Obesidad/complicaciones , Índice de Masa Corporal , Atrios CardíacosRESUMEN
Background A percentage of patients with heart failure with reduced ejection fraction (HFrEF) improve left ventricular ejection fraction (LVEF) in the evolution. This entity, defined for the first time in an international consensus as heart failure with improved ejection fraction (HFimpEF), could have a different clinical profile and prognosis than HFrEF. Our main aim was to analyze the differential clinical profile between the two entities, as well as the mid-term prognosis. Material-methods Prospective study of a cohort of patients with HFrEF who had echocardiographic data at baseline and follow-up. A comparative analysis of patients who improved LVEF with those who did not was made. Clinical, echocardiographic and therapeutic variables were analyzed, and the mid-term impact in terms of mortality and hospital readmissions for HF was assessed. Results Ninety patients were analyzed. Mean age was 66.5(10.4) years, with a male predominance (72.2%). Forty five patients (50%) improved LVEF (Group-1,HFimpEF) and forty five patients (50%) sustained reduced LVEF (Group-2,HFsrEF). The mean time to LVEF improvement in Group-1 was 12.6(5.7) months. Group-1 had a more favorable clinical profile: lower prevalence of cardiovascular risk factors, higher prevalence of de novo HF (75.6% vs. 42.2%; p<0.05), lower prevalence of ischemic etiology (22.2% vs. 42.2%; p<0.05), with less basal dilatation of the left ventricle. At the end of follow-up (mean 19(1) months) Group-1 had a lower hospital readmission rate (3.1% vs. 26.7%; p<0.01), as well as lower mortality (0% vs. 24.4%; p<0.01). Conclusion Patients with HFimpEF seem to have a better mid-term prognosis in terms of reduced mortality and hospital admissions. This improvement could be conditioned by the clinical profile of patients HFimpEF (AU)
Introducción Un porcentaje de pacientes con insuficiencia cardiaca y fracción de eyección reducida (IC-FEr) mejoran la fracción de eyección ventricular izquierda (FEVI) en la evolución. Esta entidad se ha definido por primera vez en un consenso internacional como insuficiencia cardiaca y fracción de eyección mejorada (IC-FEm), y podría tener un perfil y pronóstico diferente que IC-FEr. Nuestro objetivo fue analizar el perfil de ambas entidades y su pronóstico a medio plazo. Material y métodos Estudio prospective de una cohorte de pacientes con IC-FEr que tenían datos ecocardiográficos basales y en el seguimiento. Se hizo un análisis comparativo de pacientes con IC-FEm y pacientes con insuficiencia cardiaca y IC-FEpr. Se analizaron variables clínicas, ecocardiográficas y de tratamiento; el impacto clínico a medio plazo se analizó en términos de mortalidad y reingresos hospitalarios por insuficiencia cardiaca. Resultados Se analizaron 90 pacientes, edad media 66,5 (10,4) años (72,2% mujeres). La mitad de los pacientes mejoraron su FEVI, con un tiempo medio hasta la mejoría de 12,6 (5,7) meses. El grupo IC-FEm tenía un perfil clínico más favorable: menor proporción de factores de riesgo cardiovascular, prevalencia más elevada de IC-novo (75,6 vs. 42,2%; p < 0,05), y menor proporción de isquemia (22,2 vs. 42.2%; p < 0,05). Los pacientes con IC-FEm en el seguimiento a medio plazo tenían menor tasa de reingresos (3,1 vs. 26,7%; p < 0,01), y mortalidad (0 vs. 24,4%; p < 0,01). Conclusión Pacientes con IC-FEm parecen tener un mejor pronóstico en términos de mortalidad y reingresos hospitalarios por insuficiencia cardiaca (IC). Esta mejoría clínica podría estar condicionada por el perfil de los pacientes con IC-FEm (AU)
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Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Función Ventricular Izquierda , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Estudios Prospectivos , Estudios de Cohortes , Volumen Sistólico , PronósticoRESUMEN
BACKGROUND: A percentage of patients with heart failure with reduced ejection fraction (HFrEF) improve left ventricular ejection fraction (LVEF) in the evolution. This entity, defined for the first time in an international consensus as heart failure with improved ejection fraction (HFimpEF), could have a different clinical profile and prognosis than HFrEF. Our main aim was to analyze the differential clinical profile between the two entities, as well as the mid-term prognosis. MATERIAL-METHODS: Prospective study of a cohort of patients with HFrEF who had echocardiographic data at baseline and follow-up. A comparative analysis of patients who improved LVEF with those who did not was made. Clinical, echocardiographic and therapeutic variables were analyzed, and the mid-term impact in terms of mortality and hospital readmissions for HF was assessed. RESULTS: Ninety patients were analyzed. Mean age was 66.5(10.4) years, with a male predominance (72.2%). Forty five patients (50%) improved LVEF (Group-1,HFimpEF) and forty five patients (50%) sustained reduced LVEF (Group-2,HFsrEF). The mean time to LVEF improvement in Group-1 was 12.6(5.7) months. Group-1 had a more favorable clinical profile: lower prevalence of cardiovascular risk factors, higher prevalence of de novo HF (75.6% vs. 42.2%; p<0.05), lower prevalence of ischemic etiology (22.2% vs. 42.2%; p<0.05), with less basal dilatation of the left ventricle. At the end of follow-up (mean 19(1) months) Group-1 had a lower hospital readmission rate (3.1% vs. 26.7%; p<0.01), as well as lower mortality (0% vs. 24.4%; p<0.01). CONCLUSION: Patients with HFimpEF seem to have a better mid-term prognosis in terms of reduced mortality and hospital admissions. This improvement could be conditioned by the clinical profile of patients HFimpEF.
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Insuficiencia Cardíaca , Función Ventricular Izquierda , Humanos , Masculino , Anciano , Femenino , Volumen Sistólico , Estudios Prospectivos , PronósticoRESUMEN
BACKGROUND: Heart failure (HF) admission in chronic coronary syndrome (CCS) patients has a prognostic impact. Stratification schemes have been described for predicting this endpoint, but none of them has been externally validated. OBJECTIVES: Our aim was to develop point scores for predicting incident HF admission with data from previous studies, to perform an external validation in an independent prospective cohort and to compare their discriminative ability for this event. METHODS: Independent predictive variables of HF admission in CCS patients without baseline HF were selected from four previous prospective studies (CARE, PEACE, CORONOR and CLARIFY), generating scores based on the relative magnitude of the coefficients of Cox of each variable. Finally, the scores were validated and compared in a monocentric prospective cohort. RESULTS: The validation cohort included 1212 patients followed for up to 17 years, with 171 patients suffering at least one HF admission in the follow-up. Discriminative ability for predicting HF admission was statistically significant for all, and paired comparisons among them were all nonsignificant except for CORONOR score was superior to CLARIFY score (C-statistic 0.73, 95%CI 0.69-0.76 vs. 0.69, 95% CI 0.65-0.73; p = 0.03). CONCLUSION: All tested scores showed significant discriminative ability for predicting incident HF admission in this independent validation study. Their discriminative ability was similar, with significant differences only between the two scores with higher and lower performance.
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Insuficiencia Cardíaca , Humanos , Estudios Prospectivos , Estudios de Cohortes , Síndrome , Factores de Riesgo , Insuficiencia Cardíaca/epidemiología , Pronóstico , Medición de RiesgoRESUMEN
Background: Women and men with chronic coronary syndrome (CCS) have different clinical features and management, and studies on mid-term prognosis have reported conflicting results. Our objective was to investigate the impact of the female sex in the prognosis of the disease in the very long term. Methods and Results: We investigated differential features and very long-term prognosis in 1268 consecutive outpatients with CCS (337 [27%] women and 931 [73%] men). Women were older than men, more likely to have hypertension, diabetes, angina, and atrial fibrillation, and less likely to be exsmoker/active smoker and to have been treated with coronary revascularization (p < 0.05 for all). The prescription of statins, antiplatelets, and betablockers was similar in both groups. After up to 17 years of follow-up (median = 11 years, interquartile range = 4-15 years), cumulative incidences of acute myocardial infarction (10.2% vs. 11.8%) or stroke (11% vs. 10%) at median follow-up were similar, but the risks of major cardiovascular events (acute myocardial infarction, stroke, or cardiovascular death, 41.2% vs. 33.6%), hospital admission for heart failure (20.9% vs. 11.9%), or cardiovascular death (32.3% vs. 22.1%) were significantly higher for women (p < 0.0005), with a nonsignificant trend to higher overall mortality (45.2% vs. 39.1%, p = 0.07). However, after multivariate adjustment, all these differences disappeared. Conclusion: Although women and men with CCS presented a different clinical profile, and crude rates of major cardiovascular events, heart failure and cardiovascular death were higher in women, female sex was not an independent prognostic factor in this study with up to 17 years of follow-up.
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Insuficiencia Cardíaca , Infarto del Miocardio , Accidente Cerebrovascular , Masculino , Humanos , Femenino , Pronóstico , Estudios Prospectivos , Factores de Riesgo , Factores SexualesRESUMEN
Somatic mutations in the ten-eleven translocation methylcytosine dioxygenase 2 gene (TET2) have been associated to hematologic malignancies. More recently, biallelic, and monoallelic germline mutations conferring susceptibility to lymphoid and myeloid cancer have been described. We report two unrelated autoimmune lymphoproliferative syndrome-like patients who presented with T-cell lymphoma associated with novel germline biallelic or monoallelic mutations in the TET2 gene. Both patients presented a history of chronic lymphoproliferation with lymphadenopathies and splenomegaly, cytopenias, and immune dysregulation. We identified the first compound heterozygous patient for TET2 mutations (P1) and the first ALPS-like patient with a monoallelic TET2 mutation (P2). P1 had the most severe form of autosomal recessive disease due to TET2 loss of function resulting in absent TET2 expression and profound increase in DNA methylation. Additionally, the immunophenotype showed some alterations in innate and adaptive immune system as inverted myeloid/plasmacytoid dendritic cells ratio, elevated terminally differentiated effector memory CD8 + T-cells re-expressing CD45RA, regulatory T-cells, and Th2 circulating follicular T-cells. Double-negative T-cells, vitamin B12, and IL-10 were elevated according to the ALPS-like suspicion. Interestingly, the healthy P1's brother carried a TET2 mutation and presented some markers of immune dysregulation. P2 showed elevated vitamin B12, hypergammaglobulinemia, and decreased HDL levels. Therefore, novel molecular defects in TET2 confirm and expand both clinical and immunological phenotype, contributing to a better knowledge of the bridge between cancer and immunity.
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Síndrome Linfoproliferativo Autoinmune , Dioxigenasas , Neoplasias Hematológicas , Masculino , Humanos , Síndrome Linfoproliferativo Autoinmune/diagnóstico , Síndrome Linfoproliferativo Autoinmune/genética , Mutación de Línea Germinal , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/genética , Mutación/genética , Fenotipo , Vitamina B 12 , Proteínas de Unión al ADN/genética , Dioxigenasas/genéticaRESUMEN
Multisystem inflammatory syndrome in children (MIS-C) is a postinfectious condition which usually develops 4 to 6 weeks after SARS-CoV-2 infection in a genetically predisposed individual. Clinical features are heterogeneous and include fever, respiratory compromise, mucocutaneous involvement with conjunctival abnormalities and erythematous exanthem, abdominal pain, and diarrhea. Neurologic and cardiovascular symptoms can also develop, including coronary artery dilatation. Some cases involve 2 or more organs and require critical admission. Echocardiography is the mainstay of cardiac evaluation in the acute setting as well as on outpatient follow-up. We present the case of a 4-month-old female with no past medical or surgical history who presented with a prolonged febrile syndrome associated with severe respiratory illness, gastrointestinal symptoms, and mucocutaneous abnormalities. Diagnosis of MIS-C was established based on clinical findings, persistently elevated markers of systemic inflammation and positive SARS-CoV-2 molecular test and evidence of prior SARS-CoV-2 infection with SARS-CoV-2 IgG positive. Echocardiogram evidenced myopericarditis and coronary aneurysms and patient was deemed candidate for immunomodulatory therapy with intravenous immunoglobulin (IVIg), resulting in favorable clinical and paraclinical outcomes. Few cases of giant coronary aneurysms have been reported in children. There are no existing literature reports about coronary thrombosis or thrombus formation resulting from vascular aneurysmal dilations in this population. As such, the prognosis and natural history of coronary artery aneurysms in the setting of MIS-C remain largely unknown.
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Our aim was to investigate the role of left atrial longitudinal strain (LALS) in the non-invasive diagnosis of acute cellular rejection (ACR) episodes in heart transplant (HTx) recipients. Methods: We performed successive echocardiographic exams in 18 consecutive adult HTx recipients in their first year after HTx within 3 h of the routine surveillance endomyocardial biopsies (EMB) in a single center. LALS parameters were analyzed with two different software. We investigated LALS association with ACR presence, as well as inter-vendor variability in comparable LALS values. Results: A total of 147 pairs of EMB and echo exams were carried out. Lower values of LALS were significantly associated with any grade of ACR presence. Peak atrial longitudinal strain (PALS) offered the best diagnostic value for any grade of ACR, with a C statistic of 0.77 using one software (95% CI 0.68−0.84, p < 0.0005) and 0.64 with the other (95% CI 0.54−0.73, p = 0.013) (p = 0.02 for comparison between both curves). Reproducibility between comparable LALS parameters was poor (intraclass correlation coefficients were 0.60 for PALS, 95% CI 0.42−0.73, p < 0.0005; and 0.42 for PALS rate, 95% CI −0.13−0.68, p < 0.0005). Conclusions: LALS variables might be a sensitive marker of ACR in HTx recipients, principally discriminating between those studies without rejection and those with any grade of ACR. Inter-vendor variability was significant.
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Jubaea chilensis (Molina) Baill., also named Chilean palm, is an endemic species found in the coastal area of Mediterranean sclerophyllous forest in Chile. It has a highly restricted and fragmented distribution along the coast, being under intense exploitation and anthropogenic impact. Based on 1038 SNP markers, we evaluated the genetic diversity and population structure among six J. chilensis natural groups encompassing 96% of the species distribution. We observed low levels of genetic diversity, a deficit of heterozygotes (mean HE = 0.024; HO = 0.014), and high levels of inbreeding (mean FIS = 0.424). The fixation index (FST) and Nei's genetic distance pairwise comparisons indicated low to moderate structuring among populations. There was no evidence of isolation by distance (r = -0.214, p = 0.799). In the cluster analysis, we observed a closer relationship among Culimo, Cocalán, and Candelaria populations. Migration rates among populations were low, except for some populations with moderate values. The K value that best represented the spatial distribution of genetic diversity was ∆K = 3. Habitat fragmentation, deterioration of the sclerophyllous forest, lack of long-distance dispersers, and a natural regeneration deficit may have driven inbreeding and low levels of genetic diversity in the palm groves of J. chilensis. Although extant populations are not at imminent risk of extinction, the rate of inbreeding could increase and migration could decrease if the effects of climate change and human impact become more acute.
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INTRODUCTION: For a long time, vitamin K antagonists (VKA) were the only oral anticoagulation therapy available to reduce adverse events in atrial fibrillation (AF) patients. Direct-acting oral anticoagulants (DOAC) are at least as effective and safe as VKA with few drug interactions, rapid onset, and short half-life. Four DOACs, dabigatran, apixaban, rivaroxaban, and edoxaban, have demonstrated efficacy and safety for treatment in AF patients. AREAS COVERED: The purpose of this review article is to analyze the current evidence in clinical trials and in real-world populations and performed a new analysis with the estimated effect of those DOACs over the VKA population from the FANTASIIA registry. EXPERT OPINION: In the absence of randomized, controlled head-to-head comparisons between DOACs, high-quality observational data can provide useful information on the comparative effectiveness of DOACs. Current clinical guidelines recommend the management of oral anticoagulation in AF patients with DOACs over VKA for stroke prevention; however, many guidelines generally do not suggest a specific DOAC choice in clinical practice. The revised evidence in this manuscript and our real experience reflects that apixaban and dabigatran show the best efficacy and safety profile.
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Fibrilación Atrial , Accidente Cerebrovascular , Administración Oral , Anticoagulantes , Dabigatrán , Humanos , Piridonas , Sistema de Registros , Rivaroxabán , Vitamina KRESUMEN
ObjetivoEn la amiloidosis cardiaca (AC) el material amiloide puede depositarse en diferentes estructuras cardiacas pudiendo producir diferentes alteraciones electrocardiográficas. El objetivo fue describir qué alteraciones electrocardiográficas son más frecuentes en pacientes con AC, analizando su impacto en la necesidad de marcapasos.MetodosEstudio retrospectivo que incluye pacientes diagnosticados de AC por cadenas ligeras (AC-AL) y AC por transtirretina (AC-TTR), entre enero-2013 y marzo-2021. Se analizó el ritmo basal, el porcentaje con patrón de seudoinfarto, bajo voltaje o alteraciones de la conducción; también se analizó el impacto en la necesidad de marcapasos definitivo.ResultadosSe incluyeron 58 pacientes con AC (20 AC-AL, 38 AC-TTR). Varones (69%), 21 (36%) tenían FA al diagnóstico. El 60% tenía patrón de seudoinfarto, el 35% bajo voltaje y un 22% tenían criterios de hipertrofia ventricular. Dos tercios tenían algún trastorno de conducción: bloqueo auriculoventricular de primer grado, 18 pacientes (31%); 12 bloqueo completo de rama derecha (BCRD), 3 bloqueo completo de rama izquierda (BCRI) y 25 con un hemibloqueo de rama. No hubo diferencias entre AC-AL y AC-TTR. Los pacientes con AC-TTR tuvieron mayor necesidad de marcapasos en el seguimiento (39±40 meses). El bloqueo completo de rama (BCR) fue un predictor de necesidad de marcapasos permanente (HR: 23,43; IC 95%: 4,09-134,09; p=0,01).ConclusionesLas alteraciones electrocardiográficas en pacientes diagnosticados de AC son heterogéneas, siendo la más frecuente la presencia de trastornos de conducción, el patrón de seudoinfarto, seguido del de bajo voltaje. Los pacientes con cualquier BCR en el electrocardiograma basal son más propensos a precisar marcapasos en el seguimiento, sobre todo en AC-TTR. (AU)
AimAmyloidosis is a disease in which amyloid fibrils can be deposited in different cardiac structures, and several electrocardiographic abnormalities can be produced by this phenomenon. The objective of this study was to describe the most common basal electrocardiographic alterations in patients diagnosed with cardiac amyloidosis (CA) and to determine if these abnormalities have an impact on the need of pacemaker.MethodsThis retrospective study included patients who had an established diagnosis of CA [light-chain cardiac amyloidosis (LA-CA) or transthyretin cardiac amyloidosis (TTR-CA)] between January 2013 and March 2021. The baseline heart rate, the percentage of patients with a pseudo-infarct pattern, low-voltage pattern or cardiac conductions disturbances, and the impact of these factors on the need of pacemaker were analysed.ResultsFifty-eight patients with CA (20 with LA-CA and 38 with TTR-CA) were included, and the majority were male (69.0%). Twenty-one patients had atrial fibrillation (AF) at diagnosis. Thirty-five patients had a pseudo-infarct pattern, 35% had a low-voltage pattern, and 22% had criteria for ventricular hypertrophy. Two hirds had a conduction disorder: 18 patients with first degree atrioventricular block, 12 right bundle branch block, 3 left bundle branch block and 25 with a branch hemiblock. There were no differences between LA-CA and TTR-CA. Patients with TTR-CA had a greater need for pacemakers in the folow-up (39±40 meses). Bundle branch block was a predictor of the need for a permanent pacemaker (HR: 23.43; CI 95%: 4.09.134.09; P=.01).ConclusionsElectrocardiographic abnormalities in patients diagnosed wich CA are heterogeneus. Most frecuent is the presence of conduction disorders, the pseudoinfarction pattern, followed by the low voltage pattern. Patients with any bundle branch block at the baseline electrocardiogram need more frecuent to require a pacemaker during follow-up, especially in TTR-CA. (AU)
Asunto(s)
Humanos , Amiloidosis/complicaciones , Amiloidosis/diagnóstico , Fibrilación Atrial , Bloqueo Cardíaco , Infarto , Electrocardiografía , Estudios RetrospectivosRESUMEN
BACKGROUND: Two-dimensional speckle-tracking echocardiography (2DSTE) has been present for years. However, it is underutilized due to the expertise and time requirements for its analysis. Our aims were to provide strain values in a paediatric Spanish population and to assess the feasibility and reproducibility of a new strain software analysis in our environment. METHODS: A cross-sectional study of 156 healthy children aged 6 to 17 years. Longitudinal strain (LS) analysis of the left ventricle, right ventricle, and left atrium was performed. Feasibility and reproducibility were assessed. The associations of clinical and echocardiographic variables with strain values were investigated by multivariate analysis. RESULTS: Mean age was 11 ± 3 years (50% female). Feasibility of LS measurement ranged from 94.2% for left ventricle global LS (LVGLS) to 98.1% for other chamber strain parameters. Strain values were 26.7 ± 2.3% for LVGLS; 30.5 ± 4.4% and 26.9 ± 4% for right ventricle free wall LS (RVFWLS) and four chambers view LS (RV4CLS) respectively; and 57.8 ± 10.5%, 44.9 ± 9.5%, and 12.9 ± 5.5% for left atrium LS reservoir phase (LALSr), conduct phase (LALScd) and contraction phase (LALSct), also respectively. Body surface area (BSA) and age presented a negative correlation with strain values. Higher values were found in females than in males, except for LALScd. Excellent intra- and inter-observer reproducibility were found for right and left ventricular strain measurement, with intraclass correlation coefficients (ICC) ranging from 0.88 to 0.98, respectively. In conclusion, we described strain values in a healthy Spanish paediatric population. LS assessment by this new strain analysis software by semi-automatic manner was highly feasible and reproducible.