Video Experimental Relacionado
Updated: Jul 19, 2026
13:42
Measurement of Antibody Effects on Cellular Function of Isolated Cardiomyocytes
Published on: March 8, 2013
La fibroelastosis endocárdica materna asociada a los anticuerpos anti-Ro y anti-La asociados a la fibroelastosis
Lynne E Nield1, Earl D Silverman, Glenn P Taylor
1Department of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Toronto, Ontario, Canada.
Circulation
|February 21, 2002
Resumen
Los anticuerpos maternos vinculados al bloqueo cardíaco congénito pueden causar fibroelastosis endocardiana (EFE) en los bebés. Esta condición tiene una alta tasa de mortalidad, incluso con tratamiento.
Área de la Ciencia:
- Cardiología Cardiología.
- Inmunología Inmunología.
- Neonatología en Neonatología.
Sus antecedentes:
- Los anticuerpos maternos anti-Ro y anti-La están relacionados con el bloqueo cardíaco congénito (CHB).
- La fibroelastosis endocárdica (EFE) rara vez se describe en la CHB mediada por autoanticuerpos, con una historia natural y una patogénesis poco claras.
Objetivo del estudio:
- Investigar la historia natural y la patogénesis de la EFE en fetos y niños con CHB nacidos de madres con anticuerpos anti-Ro o anti-La.
Principales métodos:
- Revisión retrospectiva de la historia clínica, la ecocardiografía y la patología en 5 centros.
- Análisis de 13 fetos y niños con EFE y CHB nacidos de madres positivas para anticuerpos anti-Ro o anti-La.
- Inmunohistoquímica para evaluar la respuesta inmune fetal en los corazones afectados.
Principales resultados:
- La disfunción ventricular severa estuvo presente en todos los casos.
- EFE desarrolló el diagnóstico post-CHB en algunos casos, a pesar del ritmo ventricular.
- Alta mortalidad (85%) debido a la EFE, con 9 muertes y 2 trasplantes cardíacos.
- La patología reveló EFE de moderada a grave, predominantemente en el ventrículo izquierdo.
- La inmunohistoquímica mostró deposición de IgG/IgM y infiltraciones de células T, lo que indica una respuesta inmune fetal.
Conclusiones:
- La fibroelastosis endocárdica (EFE) se produce en el bloqueo cardíaco congénito (CHB) mediado por autoanticuerpos, incluso con pacing.
- La EFE asociada a autoanticuerpos conlleva una tasa de mortalidad muy alta tanto en la vida fetal como en la postnatal.
Más Videos Relacionados
Videos de Conceptos Relacionados
01:19Rh Blood Group
The Rhesus (Rh) antigen is crucial in determining blood groups and ensuring compatibility during blood transfusions.
01:27Myocarditis II: Clinical Features and Diagnostic Tests
Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
01:23Rheumatic Heart Disease I: Introduction
Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
01:29Cardiomyopathy IV: Restrictive Cardiomyopathy
Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...