ABSTRACT
BACKGROUND: The association between Ebstein anomaly and myocardial fibrosis, particularly in the left ventricle, has been controversial. We aimed to assess the prevalence of replacement fibrosis with a focus on the left ventricle (LV) using cardiac magnetic resonance (CMR), make a histopathological association between LV fibrosis and CMR findings, and explore whether LV fibrosis is an independent risk factor for cardiovascular disease mortality using a derived risk score. METHODS: We performed a 12-year (2009-2021) retrospective cohort of adult patients with Ebstein anomaly who underwent CMR. The CMR evaluation included a comprehensive assessment of myocardial fibrosis by late gadolinium enhancement (LGE). Four postmortem samples were obtained from our cohort and stained using Masson trichrome to characterize LV fibrosis. We used Cox-regression analysis to identify and derive a prediction score that associated LV fibrosis with cardiovascular disease mortality. RESULTS: We included 57 adults with Ebstein anomaly (52% men; median age, 29.52 [interquartile range, 21.24-39.17] years), of whom 12 died during follow-up. LGE prevalence by CMR was observed in 52.6% in any chamber; LV-LGE in 29.8%. Histopathological findings revealed a mid-wall pattern with predominantly interstitial fibrosis and minimal replacement fibrosis. LV-LGE was associated with increased risk of cardiovascular disease mortality (hazard ratio, 6.02 [95% CI, 1.22-19.91]) attributable to lateral and mid-wall LV segment involvement. Our mortality score achieved an overall good prediction capacity (R2, 0.435; C statistic, 0.93; Dxy, 0.86). CONCLUSIONS: There is a high prevalence of LV fibrosis replacement in adults with Ebstein anomaly, characterized by specific CMR and histological patterns. Furthermore, LV-LGE fibrosis is an independent predictor of cardiovascular disease mortality, which could be integrated into risk assessment in clinical management.
Subject(s)
Cardiomyopathies , Ebstein Anomaly , Ventricular Dysfunction, Left , Male , Humans , Adult , Female , Retrospective Studies , Heart Ventricles/diagnostic imaging , Contrast Media , Ebstein Anomaly/complications , Ebstein Anomaly/diagnostic imaging , Ebstein Anomaly/epidemiology , Magnetic Resonance Imaging, Cine/adverse effects , Gadolinium , Fibrosis , Magnetic Resonance Spectroscopy/adverse effects , Ventricular Function, LeftSubject(s)
Lupus Erythematosus, Systemic , Myocardial Infarction , Vasculitis , Humans , Coronary Vessels/diagnostic imaging , Myocardial Infarction/complications , Myocardial Infarction/diagnosis , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Vasculitis/complications , Vasculitis/diagnosis , PatientsABSTRACT
BACKGROUND: In Mexico, adult-onset Still's disease (AOSD) is one of the causes of fever of unknown origin (FUO). The aim of this study is to describe a series of AOSD cases from a FUO cohort in order to know the clinical and biochemical characteristics of the cases, as well as to describe the neutrophil-lymphocyte index (NLI), which is a clinical marker of inflammation in autoimmune diseases. CASE REPORT: An observational study of 24 cases with AOSD; 72 % of them were women, the median age was 43 years (IQR 37.7-59.7), and the most frequent manifestations were classic rash (84 %) and arthralgia (100 %). All of them had tested negative for rheumatoid factor, antinuclear antibodies, and hyperferritinemia; 83 % had NLI > 3.08. The most used treatment was the combination of methotrexate with corticosteroids; seven patients required biological therapy, and one of them presented a hypersensitivity reaction. CONCLUSION: When there's FUO, the existence of AOSD should be suspected; also in the presence of rash, arthralgia, hyperferritinemia, and NLI > 3.08.
Antecedentes: En México, la enfermedad de Still del adulto (ESA) es una causa de fiebre de origen desconocido (FOD). El objetivo de este informe fue describir una serie de casos de ESA de una cohorte de FOD para conocer las características clínicas y bioquímicas, así como describir el índice neutrófilo/linfocito (INL), marcador clínico de inflamación en enfermedades autoinmunes. Caso clínico: Estudio observacional de 24 casos con ESA; 72 % fue del sexo femenino, la edad fue de 43 años (37.7-59.7) y las manifestaciones más frecuentes fueron rash clásico (84 %) y artralgias (100 %). Todos tuvieron factor reumatoide, anticuerpos antinucleares negativos e hiperferritinemia; 83 % tuvo INL > 3.08. El tratamiento más empleado fue la combinación de metotrexato y corticosteroides; siete pacientes ameritaron terapia biológica, uno presentó reacción de hipersensibilidad. Conclusión: Ante fiebre de origen desconocido, debe sospecharse ESA si, además, existe rash, artralgias, hiperferritinemia e INL > 3.08.
Subject(s)
Fever of Unknown Origin , Still's Disease, Adult-Onset , Adult , Female , Humans , Lymphocytes , Mexico/epidemiology , Neutrophils , Still's Disease, Adult-Onset/complications , Still's Disease, Adult-Onset/diagnosisABSTRACT
Resumen La endocarditis infecciosa es una patología que requiere un diagnóstico y tratamiento oportuno para prevenir sus graves complicaciones y, ante todo, evitar la muerte del paciente. En el presente artículo, documentamos el abordaje diagnóstico y terapéutico de un caso de endocarditis, en el escenario de una cardiopatía congénita, válvula aórtica bicúspide, atendida en el Instituto Nacional de Cardiología "Ignacio Chávez".
Abstract Infective endocarditis is a condition that requires an early diagnosis and treatment to prevent its severe complications and, above all, avoid death. In this article we document the diagnosis and therapeutics of a case of infective endocarditis in the setting of a congenital heart disease, bicuspid aortic valve, referred to the National Institute of Cardiology "Ignacio Chávez".
ABSTRACT
Resumen La estenosis aórtica supravalvular es una lesión congénita obstructiva del tracto de salida del ventrículo izquierdo. Es la forma menos frecuente dentro de este grupo de lesiones, las cuales, a su vez representan el 6% de las cardiopatías congénitas en pacientes pediátricos. Esta cardiopatía se relaciona más con pacientes pediátricos, sin embargo, presentamos el caso de un paciente masculino de 23 años de edad quien acudió al hospital por haber presentado episodio de disnea súbita en reposo, acompañada de diaforesis, dolor torácico opresivo sin irradiaciones y síncope. Se realizó un ecocardiograma transtorácico con el cual se diagnosticó estenosis aórtica supravalvular.
Abstract Supravalvular aortic stenosis is a left ventricular outflow tract obstructive lesion. It is the least common form of this group of lesions, which only represents 6% of congenital heart disease in children. This condition is commonly diagnosed during childhood; however we present the case of a 23 year old man who was taken to the hospital for having presented a sudden dyspnea episode with diaphoresis, chest pain without radiation and syncope. He was diagnosed with supravalvular aortic stenosis after echocardiography.
