ABSTRACT
Resumen Objetivo: La fibrosis endomiocárdica (FE) es una cardiomiopatía restrictiva infrecuente. En América Latina son escasos los reportes. En el presente trabajo se realiza una descripción de una serie de pacientes diagnosticados de FE en Colombia. Método: Realizamos una búsqueda en los registros de imágenes de resonancia magnética (RM) cardiaca realizadas en nuestra institución entre 2016 y 2019 en busca de pacientes con diagnóstico de FE. Se describieron sus características sociodemográficas, clínicas y de imagen. Resultados: Nueve pacientes fueron diagnosticados de FE (el 66.7% mujeres), con una edad promedio de 69 años. Los pacientes presentaron un promedio de 2.6 años de evolución. El principal síntoma referido fue disnea, seguido de síncope, dolor torácico y palpitaciones. En ninguno de ellos se sospechó FE como diagnóstico inicial. En cuanto a los hallazgos ecocardiográficos, se identificó compromiso predominante del ventrículo izquierdo, seguido de compromiso biventricular. Todos los pacientes presentaron patrón de llenado restrictivo con dilatación auricular izquierda severa. En el análisis retrospectivo se cumplieron los criterios de Mocumbi para el diagnóstico de FE en el 100% de los pacientes con gravedad moderada (77.8%). Las imágenes de RM mostraron función sistólica biventricular y volúmenes preservados. Se observó depósito focal de gadolinio subendocárdico a nivel apical y se confirmó la presencia de trombo en el 66% de los casos Conclusión:: La FE es una cardiomiopatía restrictiva infrecuente circunscrita a países tropicales. La mayoría de los pacientes en nuestra serie presentaron compromiso aislado del ventrículo izquierdo, seguido de compromiso biventricular, con función ventricular usualmente preservada.
Abstract Objective: Endomyocardial fibrosis (EF) is an unusual restrictive cardiomyopathy. In Latin America there are few reports. Here, we made a description of patients diagnosed with EF in Colombia. Method: We conducted a search in the records of cardiac magnetic resonance imaging (MRI) performed in our institution between 2016-2019 looking for patients with a diagnosis of EF; sociodemographic, clinical and imaging characteristics were described. Results: Nine patients were diagnosed with EF (66.7% female), with an average age of 69 years. Patients presented an average evolution of 2.6 years. The main reported symptom was dyspnea, followed by syncope, chest pain, and palpitations. None of them was initially suspected for EF. Regarding echocardiographic findings, predominant left ventricular involvement was identified, followed by bi-ventricular involvement. All the patients presented a restrictive filling pattern with severe left atrial dilation. In a retrospective analysis, Mocumbi criteria for diagnosis of EF were met in 100% of the patients, majority with moderate severity (77.8%). Cardiac MRI showed biventricular systolic function and volumes preserved. Focal subendocardial late gadolinium enhancement was observed on the apex and apical thrombus was confirmed in 66% of the patients Conclusion: FE is an uncommon restrictive cardiomyopathy limited to tropical countries. Most of patients in our series presented isolated involvement of left ventricle, followed by bi-ventricular involvement, with ventricular function usually preserved.
Subject(s)
Humans , Male , Female , Middle Aged , Aged , Cardiomyopathy, Restrictive/etiology , Cardiomyopathy, Restrictive/diagnostic imaging , Endomyocardial Fibrosis/diagnostic imaging , Heart Failure , Magnetic Resonance Imaging , Retrospective Studies , Colombia , Contrast Media , GadoliniumABSTRACT
Endomyocardial fibrosis is a known and prevalent cause of restrictive cardiomyopathy in countries of Equatorial Africa, but very rare elsewhere. Although the diagnosis in endemic countries is established by the presence of certain echocardiographic criteria, the gold standard is the endomyocardial biopsy, which shows fibrous tissue in the endocardium. An acute and subacute phase can be distinguished, with associated mortality due to complications of heart failure. In contrast, the chronic phase presents less aggressive behavior, with progression of symptoms. In this phase, surgical treatment may improve survival. We present the case of a 44-year-old patient with good clinical control under pharmacological treatment after 14 months of follow-up.
Subject(s)
Humans , Female , Adult , Cardiomyopathy, Restrictive/etiology , Endomyocardial Fibrosis/complications , Biopsy/methods , Echocardiography , Chronic Disease , Endomyocardial Fibrosis/diagnosis , Endomyocardial Fibrosis/drug therapyABSTRACT
Restrictive cardiomyopathy is the least common form of cardiomyopathy, and the disease that most often cause it, is the system amyloidosis. We present a 62-year-old with a history of heart failure, which in its assessment highlights the discrepancy between the low voltage ventricular complexes in the electrocardiogram and the severity of left ventricular wall thickness on echocardiography. This discrepancy was the source of suspicion and subsequent confirmation of systemic amyloidosis with cardiac involvement.
Subject(s)
Amyloidosis/diagnosis , Cardiomyopathy, Restrictive/diagnosis , Electrocardiography/methods , Heart Ventricles/diagnostic imaging , Amyloidosis/complications , Cardiomyopathy, Restrictive/etiology , Fatal Outcome , Humans , Heart Failure/complications , Male , Myocardium/pathology , Middle Aged , Heart Ventricles/physiopathologyABSTRACT
As cardiomiopatias restritivas podem possuir diversas etiologias, entre elas destacam-se as doenças de depósito por diferentes materiais, como por desmina. As desminopatias são patologias incomuns, que evoluem com distúrbios de condução, miopatias periféricas e disfunção ventricular. O presente relato descreve paciente com bloqueio átrio ventricular total como evento inicial, sendo acometido posteriormente por alterações musculares esqueléticas e insuficiência cardíaca. Na investigação foi definido diagnóstico de cardiomiopatia restritiva por acúmulo de desmina.
Restrictive cardiomyopathies may have different etiologies, among which we can point out storage diseases by accumulation of different materials such as desmin. Desminopathies are uncommon diseases that progress with conduction abnormalities, peripheral myopathies, and ventricular dysfunction. The present report describes a patient with complete atrioventricular block as the initial event; he later developed skeletal muscle alterations and heart failure. The investigation led to the diagnosis of restrictive cardiomyopathy due to desmin accumulation.
Subject(s)
Adult , Humans , Male , Cardiomyopathy, Restrictive , Desmin/analysis , Biopsy , Cardiomyopathy, Restrictive/diagnosis , Cardiomyopathy, Restrictive/etiology , Heart Block/diagnosis , Muscle, Skeletal/pathologyABSTRACT
Se presenta la experiencia en transplante cardíaco del Hospital Dr. Gustavo Fricke de Viña del Mar desde la creación del Comité de Enfermedades Cardiorespiratorias Terminales en 1998. Actualmente hay 9 pacientes en control. Se analizan los resultados a corto y largo plazo, la terapia utilizada y las complicaciones más frecuentes y su manejo
Subject(s)
Humans , Male , Adolescent , Female , Middle Aged , Immunosuppressive Agents/therapeutic use , Heart Diseases , Immunosuppression Therapy , Heart Failure/pathology , Cardiomyopathy, Dilated/etiology , Cardiomyopathy, Restrictive/etiology , Heart Transplantation , Anti-Infective Agents/therapeutic use , Chile , Respiration, ArtificialABSTRACT
Descrevemos o caso de um paciente masculino de 42 anos, com amiloidose sistêmica, provavelmente primária, comprometendo o coraçäo. Ressaltam-se os aspectos fisiopatológicos, clínicos e a relativa raridade do diagnoóstico in vivo. Discutem-se, ainda que limitados, os aspectos terapêuticos relacionados aos problemas cardíacos.