Como eu faço avaliação ecocardiográfica de pericardite constritiva / My approach to echocardiographic assessment for constrictive pericarditis

A pericardite constritiva (PC) é uma condição na qual a cicatrização e perda de elasticidade do pericárdio resultam em enchimento ventricular prejudicado, disfunção diastólica e insuficiência cardíaca direita. O diagnóstico dessa patologia é desafiador, sendo frequente a necessidade de técnicas de imagem multimodal, dentre as quais a ecocardiografia representa a modalidade de imagem inicial para a avaliação diagnóstica, além de permitir a diferenciação da PC da cardiomiopatia restritiva (CMR) e outras condições que mimetizam constrição. (AU)

Constrictive pericarditis (CP) is a condition in which scarring and loss of elasticity of the pericardium result in impaired ventricular filling, diastolic dysfunction, and right heart failure. The diagnosis of this pathology is challenging, with frequent need for multimodal imaging techniques, among which echocardiography represents the initial imaging modality for the diagnostic evaluation, in addition to allowing the differentiation of CP from restrictive cardiomyopathy (RCM) and other conditions that mimic constriction. (AU)

Fibrosis endomiocárdica: una revisión actualizada a propósito de unos casos en un programa de falla cardiaca / Endomyocardial fibrosis: an updated review, about some cases in a heart failure program

Resumen Introducción La fibrosis endomiocárdica es una enfermedad cardiovascular olvidada, principal causa de cardiomiopatía restrictiva. Se estima que en el mundo hay 10-12 millones de personas con la enfermedad. La mayoría se restringen a zonas de África, Asia y, en Latinoamérica, en Brasil y Colombia. La etiología y la fisiopatología son poco comprendidas. Para su diagnóstico se deben tener en cuenta los datos del paciente, el cuadro clínico y los hallazgos ecocardiográficos. Presentamos los casos confirmados en un centro de referencia cardiovascular. Descripción de los casos: Se han documentado tres casos, de los cuales dos son mujeres. Todos debutaron con un cuadro insidioso de disnea con esfuerzo. Los hallazgos ecocardiográficos fueron disfunción diastólica con patrón restrictivo, insuficiencia moderada de válvulas auriculoventriculares e hipertensión pulmonar; en dos casos se encontraron imágenes anormales endocárdicas en el ventrículo izquierdo y se realizó biopsia endomiocárdica para confirmar el diagnóstico. Ninguno mejoró la clase funcional ni la frecuencia de hospitalizaciones. Conclusiones La fibrosis endomiocárdica debe considerarse en todo paciente con falla cardiaca con un patrón restrictivo asociado y con poca respuesta al manejo farmacológico convencional. Los pacientes presentados tenían hallazgos típicos de la enfermedad, con la particularidad de ser de edades mayores.

Abstract Introduction Endomyocardial fibrosis is one of the neglected cardiovascular disease, the main cause of restrictive cardiomyopathy. There are an estimated 10-12 million people with the disease worldwide. Most are restricted to areas of Africa, Asia and in Latin America in Brazil and Colombia. The etiology and pathophysiology are poorly understood. For its diagnosis, the patient's data, the clinical picture and the echocardiographic findings must be taken into account. We present the confirmed cases in a cardiovascular referral center. Description of the cases Three cases have been documented of which two are women. All debuted with insidious dyspnea on exertion. The echocardiographic findings were diastolic dysfunction with a restrictive pattern, moderate atrioventricular valve insufficiency, and pulmonary hypertension. In two cases, endocardial abnormal images were found in the left ventricle and an endomyocardial biopsy was performed to confirm the diagnosis. None improved functional class or frequency of hospitalizations. Conclusions Endomyocardial fibrosis should be considered in a patient with heart failure with an associated restrictive pattern, and with little response to conventional pharmacological management. The patients presented had typical findings of the disease with the particularity of being older.

Fibrosis endomiocárdica: una cardiomiopatía restrictiva en países en vía de desarrollo / Endomyocardial fibrosis: a restrictive cardiomyopathy in developing countries

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.

Compromiso cardíaco por amiloidosis AL: la desemejanza como atributo. Reporte de 3 casos / Cardiac compromise due to AL amyloidosis: dissimilarity as an attribute. Report of 3 cases

RESUMEN La amiloidosis AL (antes denominada amiloidosis primaria) es una entidad sistémica poco frecuente, con incidencia desconocida en el mundo, que puede llegar a presentar compromiso cardíaco en casi la mitad de los pacientes, llevando a una cardiomiopatía restrictiva por depósito de tejido amiloide. A continuación, presentamos 3 casos de pacientes que consultaron por falla cardíaca aguda y síncope, en quienes finalmente se confirmó el diagnóstico de amiloidosis AL. Al final, se realiza una breve revisión de la literatura, enfatizando en los elementos clínicos para un diagnóstico temprano.

SUMMARY AL amyloidosis (formerly called primary amyloidosis) is a rare systemic entity, with an unknown incidence in the world, which can develop heart involvement in almost half of patients, leading to restrictive cardiomyopathy by amyloid tissue deposit. We present 3 cases of patients who consulted for acute heart failure and syncope, in which the diagnosis of AL amyloidosis was finally confirmed. We conclude with a brief review of the literature, emphasizing clinical elements for an early diagnosis.

Identification of variants in TNNI3 gene in two children with restrictive cardiomyopathy / 中华医学遗传学杂志

OBJECTIVE@#To identify the pathogenesis in two patients of restrictive cardiomyopathy (RCM) using high-throughput sequencing.@*METHODS@#Peripheral blood samples from the two patients and their parents were collected and genomic DNAs were extracted to conduct targeted next generation sequencing or whole exome sequencing. Bioinformation analysis was performed to identify the pathogenic variants in genes associated with cardiomyopathy, which were further validated by Sanger sequencing.@*RESULTS@#By high throughput sequencing, we detected a de novo heterozygous variant c.549+1G>T in TNNI3 gene in patient 1. The variant has not been reported previously and was predicted to be pathogenic in line with American College of Medical Genetics and Genomics (ACMG) guidelines (PVS1+PS2+PM2). Another heterozygous variant c.433C>T (p.Arg145Trp) in TNNI3 gene was identified in patient 2 and his father. The variant had been reported as pathogenic variant in Clinvar and HGMD databases; based on ACMG guidelines, the variant was predicted to be likely pathogenic (PS3+PM1+PP3).@*CONCLUSION@#TNNI3 variants may be the causative gene responsible for restrictive cardiomyopathy in the two patients. High throughput sequencing results provide bases for the diagnosis of restrictive cardiomyopathy.

Amiloidose cardíaca: cardiomiopatia infiltrativa com comportamento hemodinâmico restritivo ­ relato de caso / Cardiac amyloidosis: infiltrative cardiomyopathy with restrictive hemodynamic behavior ­ case report

A amiloidose é uma condição rara que descreve um grupo heterogêneo de distúrbios que cursam com a deposição extracelular de agregados proteicos fibrilares em tecidos e órgãos. Relata-se aqui o caso de paciente do sexo masculino, com 76 anos de idade, que, há 2 meses, iniciou quadro progressivo de dispneia aos mínimos esforços. Na investigação, observou-se aumento cardíaco global, e o ecocardiograma evidenciou cardiopatia restritiva infiltrativa e derrame pericárdico. Pela elevada suspeição clínica, foi solicitada ressonância magnética cardíaca, que foi altamente sugestiva de amiloidose cardíaca. Dessa forma, assim como no caso relatado, o acometimento cardíaco possui como principal forma de manifestação o tipo miocardiopatia restritivo, sendo um quadro de insuficiência cardíaca crônica com etiologia de difícil diagnóstico em pacientes acima de 50 anos, com prognóstico bastante reservado. Assim, apesar de permanecer como um desafio diagnóstico para o clínico, sua hipótese deve sempre ser aventada na ausência de outra causa que justifique tais achados (AU)

Histopatología y Strain auricular en pericarditis constrictiva y miocardiopatía restrictiva / Histopathology and Atrial Strain in Constrictive Pericarditis and restrictive cardiomyopathy

Resumen La pericarditis constrictiva y la miocardiopatía restrictiva son enfermedades raras caracterizadas por síntomas de insuficiencia cardíaca congestiva. El objetivo de este estudio es mostrar el diagnóstico diferencial de estas dos patologías, mediante strain auricular y establecer su correlación con la histopatología de corazones correspondientes. Se analizan 2 casos clínicos representativos de cada una de estas patologías. Se analizan sus presentaciones clínicas, los datos ecocardiograficos y en ambos casos se realizaron biopsias endomiocardicas con lo cual se presenta la correlación histológica. El strain auricular permitió evaluar la afección de las aurículas, consideramos que el strain auricular disminuido podría estar relacionado con la presencia de fibrosis.

Abstract Histopathology and Atrial Strain in Constrictive Pericarditis and restrictive cardiomyopathy Constrictive pericarditis and restrictive cardiomyopathy are rare diseases characterized by congestive heart failure symptoms. The aim of this study is to show the differential diagnosis of these two pathologies using strain and to establish their correlation with histopathology of the corresponding hearts. Two representative clinical cases of each of these pathologies are analyzed. Their clinical presentations, echocardiographic data, and endomyocardial biopsies were performed in both cases, thus presenting the histological correlation. Atrial strain allowed us to evaluate the affection of the atria, we consider that decreased atrial strain could be related to the presence of fibrosis.

Miocardiopatía Restrictiva: Presentación de un caso en pediatría Reporte de Caso / Restrictive Cardiomyopathy: Presentation of a case in pediatrics

Introducción: La miocardiopatía restrictiva (MR) es una entidad poco frecuente y de mal pronóstico en pacientes pediátricos. Se considera la variante menos habitual dentro de las miocardiopatías y en la mayoría de los casos su etiología es desconocida. Tiene un pronóstico grave, salvo que pueda llevarse a cabo un trasplante cardíaco. Caso: Niño de 1 año de edad, con antecedentes de infecciones respiratorias y disnea con la succión del seno materno. Fue ingresado con disnea, cianosis y edema. Al examen físico se describe la presencia de un soplo sistólico grado IV/VI en el borde esternal izquierdo. La frecuencia respiratoria de 60 por minuto, con presencia de tiraje intercostal, subcostal y supraesternal. Con hepatomegalia. Evolución: Laboratorios: presencia de leucocitosis y anemia normocítica normocrómica. En la Rx de tórax cardiomegalia grado IV. En ecocardiograma crecimiento auricular, presencia de foramen oval permeable. La función sistólica conservada con un patrón de relajación restrictivo e hipertensión pulmonar (47 mmHg). La resonancia magnética de corazón reveló el crecimiento de cavidades auriculares; con espesor del miocardio normal. Se manejó como miocardiopatía restrictiva, con furosemida, espironolactona y antiagregantes plaquetarios. Se dio el alta con iguales indicaciones a consulta externa. Conclusión: Los estudios de gabinete como la radiografía y el electrocardiograma pueden arrojar datos importantes para el diagnóstico. El ecocardiograma sigue siendo el mejor estudio para el diagnóstico. El uso de mediciones funcionales como doppler tisular puede ayudar a evidenciar falla diastólica temprana

Introduction: Restrictive cardiomyopathy (RM) is a rare entity with a poor prognosis in pediatric patients. It is considered the least common variant within cardiomyopathies and in most cases its etiology is unknown. He has a dire prognosis, unless a heart transplant can be done. Case: 1-year-old boy, with a history of respiratory infections and dyspnea with suctioning of the mother's breast. He was admitted with dyspnea, cyanosis, and edema. Physical examination describes the presence of a grade IV / VI systolic murmur at the left sternal border. The respiratory rate of 60 per minute, with the presence of intercostal, subcostal and suprasternal pulling. With hepatomegaly. Evolution: Laboratories: presence of leukocytosis and normochromic normocytic anemia. In the chest X-ray cardiomegaly grade IV. In echocardiogram atrial growth, presence of patent foramen ovale. Systolic function preserved with a restrictive relaxation pattern and pulmonary hypertension (47 mmHg). Magnetic resonance imaging of the heart revealed enlargement of the atrial chambers; with normal myocardial thickness. It was managed as restrictive cardiomyopathy, with furosemide, spironolactone, and antiplatelet agents. Outpatient consultation was discharged with the same indications. Conclusion: Cabinet studies such as radiography and electrocardiogram can provide important data for diagnosis. The echocardiogram remains the best study for diagnosis. The use of functional measurements such as tissue Doppler can help to show early diastolic failure

Epidemiological and pathological aspects of cardiomyopathies in cats in Southern Brazil / Aspectos epidemiológicos e patológicos das cardiomiopatias em gatos no Sul do Brasil

Cardiomyopathies are considered one of the most important causes of heart failure in cats and are subdivided into three main morphological types: hypertrophic (HCM), dilated (DCM), and restrictive (RCM). This study aimed to determine the frequency and types of cardiomyopathies in cats diagnosed in southern Brazil, with an emphasis on their epidemiological and pathological aspects. Necropsy reports filed in a veterinary pathology laboratory were reviewed, and cats diagnosed with cardiomyopathy were selected for the study. Animal identification data, history and clinical signs, and gross lesions, were reviewed and compiled. During the study period, 1.594 cat necropsies were performed, of which 72 (4.5%) comprised a diagnosis of cardiomyopathy. HCM was the most frequent followed by CMR and CMD, representing 77.8%, 12.5% and 9.7%, respectively. Age ranged from three months to 18 years, with a median age of seven years. In relation to sex, 62.5% were males and 37.5% females. In 76.4% of the cases, it affected cats without a breed defined. Restrictive mixed dyspnea and hydrothorax were the main signs or findings of the clinical examination. Sudden death and acute paresis of the pelvic limbs due to aortic thromboembolism have also been described. In HCM, myocardial thickening was observed, with a reduction in the ventricular chamber. Hypertrophy, disarray, and fibrosis of the myofibers were the main histological findings. In RCM, whitish and thickened endocardium was seen in most cases. DCM was characterized by dilated cardiac chambers, and microscopic examination revealed no significant findings. The main extra cardiac lesions revealed pulmonary edema and congestion, hydrothorax and chronic passive congestion of the liver. Cardiomyopathies are important causes of death in cats and should be included in the differential diagnosis of patients with cardio respiratory clinical signs and in cases related to sudden death and acute paresis of the pelvic limbs.(AU)

As cardiomiopatias são consideradas umas das mais importantes causas de insuficiência cardíaca em gatos e são subdivididas morfologicamente em três principais tipos: cardiomiopatia hipertrófica (CMH), dilatada (CMD) e restritiva (CMR). Este trabalho teve como objetivo determinar a frequência e os tipos de cardiomiopatias em gatos diagnosticados no Sul do Brasil, abordando seus aspectos epidemiológicos e patológicos. Foram revisados os laudos de necropsias de gatos e selecionados para o estudo de diagnóstico de cardiomiopatia. Os dados referentes à identificação do animal, o histórico/sinais clínicos e lesões macroscópicas foram revisados e compilados. No período estudado, foram realizadas 1.594 necropsias de gatos, destas, 72 (4,5%) compreenderam diagnóstico de cardiomiopatia. A CMH foi a mais frequente seguida pela CMR e CMD, representando 77,8%, 12,5% e 9,7%, respectivamente. A idade variou de três meses a 18 anos, com a idade mediana de sete anos. Em relação ao sexo, 62,5% eram machos e 37,5% fêmeas. Em 76,4% dos casos, afetou gatos sem raça definida. Dispneia mista restritiva e hidrotórax foram os principais sinais ou achados do exame clínico. Morte súbita e paresia aguda de membros pélvicos em razão do tromboembolismo aórtico também foram descritos. Na CMH, observou-se espessamento do miocárdio, com redução da câmara ventricular. Hipertrofia, desarranjo e fibrose das miofibras foram os principais achados histológicos. Na CMR, visualizou-se endocárdio esbranquiçado e espessado na maioria dos casos. A CMD caracterizou-se pela dilatação das câmaras cardíacas, e sem lesão histológica significante. As principais lesões extracardíacas encontradas foram edema e congestão pulmonares, hidrotórax e congestão passiva crônica do fígado. As cardiomiopatias são causas importantes de morte em gatos, devem ser incluídas no diagnóstico diferencial de pacientes com sinais clínicos cardiorrespiratórios e também em casos relacionados a morte súbita e paresia aguda dos membros pélvicos.(AU)

Value of left ventricular myocardial strain derived from cardiac magnetic resonance tissue tracking on differentiating constrictive pericarditis from restrictive cardiomyopathy / 中华心血管病杂志

Objective: To compare left ventricular myocardial mechanics detected by cardiac magnetic resonance tissue tracking(CMR-TT) between patients with constrictive pericarditis(CP) and restrictive cardiomyopathy(RCM),and see if those can be used to differentiate CP from RCM patients. Methods: A total of 23 patients with CP, 20 patients with RCM, who hospitalized in Beijing Anzhen Hospital from January 2014 to April 2019 were included in this study and 25 healthy subjects served as control group, all subjects underwent cardiac magnetic resonance examination. Myocardial mechanics were evaluated by 2-dimensional(2D) and 3-dimensional(3D) CMR-TT in terms of global longitudinal strain(GLS), circumferential strain(GCS), radial strain(GRS) and the lateral wall strain to septal wall strain ratio(lateral/septal ratio) of basal, mid-cavity and apical. The diagnostic area under the receiver operating characteristic curve (ROC) was evaluated for differentiating CP from RCM. Results: Age, sex and heart rate were similar between CP and RCM patients(all P>0.05). 2D-GLS, 3D-GLS, GCS and GRS in CP and RCM groups were significantly lower than those in normal control group(all P<0.05).3D-GLS value was significantly lower in RCM patients than in CP patients(P<0.05), the area under the curve (AUC)=0.787(sensitivity 80%, specificity 78%). 3D-GCS was significantly lower in CP group than in RCM group(P<0.05), the AUC=0.737(sensitivity 80%, specificity 65%). However, there was no significant difference between CP and RCM in 3D-GRS(P>0.05). Compared with RCM, the circumferential and radial lateral/septal ratios of the basal were significantly lower in CP group than in RCM group(both P<0.05), AUC=0.737(sensitivity 70%, specificity 83%) and 0.737 (sensitivity 60%, specificity 87%), respectively. The left ventricular myocardial mechanics strain curve of the CP,RCM and normal control were different. The CP patients presented as " rapidly down-a platform" form, the RCM presented as "slowly down" form, and normal control presented as "rapidly down" form. Conclusion: Evaluating the differences in the diastolic process of left ventricular myocardium and left ventricular myocardial mechanics strain curve is helpful to differentiate CP from RCM patients.

Amiloidosis cardíaca en un paciente con mieloma múltiple / Cardiac amyloidosis in a patient with multiple myeloma

Resumen La amiloidosis sistémica constituye una enfermedad poco frecuente, donde la infiltración cardíaca es la principal causa de morbimortalidad, sin importar la causa subyacente del depósito amiloide. Se reporta el caso de una paciente femenina de 48 años con síndrome nefrótico, insuficiencia cardíaca e inmunocompromiso, estableciéndose el diagnóstico de amiloidosis primaria con infiltración cardíaca secundaria a mieloma múltiple. Se discute brevemente la enfermedad, la importancia del juicio clínico apoyado en medios diagnósticos y los retos terapéuticos actuales.

Abstract Systemic amyloidosis constitutes a non common disease in which cardiac involvement is the leading cause of morbidity and mortality, regardless of the underlying pathogenesis of amyloid production. We present the case of a 48 years old female with nephrotic syndrome, heart failure and immunocompromise in which Primary Amyloidosis with cardiac involvement secondary to Multiple Myeloma is established as diagnosis. The disease is briefly discussed, as well as the value of clinical judgment supported on diagnostic means and the therapeutic challenges now days.

Endomiocardite de Loeffler ­ manifestação cardíaca da síndrome hipereosinofílica: relato de caso / Loeffler's endomyocarditis ­ cardiac manifestation of hypereosinophilic syndrome: case report'

Introdução: A síndrome hipereosinofílica é caracterizada por uma produção aumentada e contínua de eosinófilos e pode levar a lesões teciduais em múltiplos órgãos, como consequência da infiltração eosinofílica. Os pacientes apresentam eosinofilia absoluta no sangue periférico (> 1.500 eosinófilos/mm3) sem uma causa primária de eosinofilia. A manifestação cardíaca desta síndrome geralmente se apresenta como endomiocardite de Loeffler, que constitui uma miocardiopatia restritiva primária resultante da infiltração de eosinófilos no tecido cardíaco. Descrição do caso: Relatamos o caso raro de uma paciente de 64 anos com eosinofilia a esclarecer e comprometimento cardíaco, que teve o diagnóstico estabelecido a partir de exames de imagem. Comentários: Enfatizamos os aspectos clínicos e evolutivos, ressaltando as dificuldades diagnósticas e a importância da investigação de eosinofilias persistentes sem causa aparente, uma vez que o diagnóstico e tratamento precoce podem proporcionar melhores taxas de sobrevida e prognóstico nestes pacientes.

Introduction: The hypereosinophilic syndrome is characterized by an increased, continuous production of eosinophils, and it may lead to tissue damage in multiple organs as a consequence of eosinophilic infiltration. Patients with this syndrome present absolute eosinophil count > 1,500 eosinophils/mm3 in the peripheral blood without a primary cause for eosinophilia. The cardiac manifestation of this syndrome usually presents as Loeffler's endomyocarditis, a primary restrictive cardiomyopathy resulting from the infiltration of eosinophils into cardiac tissue. Case description: We report the rare case of a 64-year-old woman with eosinophilia and cardiac involvement, who had the diagnosis established based on imaging tests. Comments: We emphasize the clinical and evolutionary aspects of the condition, highlighting the diagnostic difficulties and the importance of investigating persistent eosinophilia without an apparent cause, as early diagnosis and treatment can provide better survival rates and improved prognosis in these patients.

Etiología de la insuficiencia cardíaca crónica / Chronic heart failure etiology

La insuficiencia cardiaca es el resultado fisiopatológico de múltiples enfermedades. Además de la patologia isquémica, valvular, de la conducción o hipertensiva existen otras etiologías poco sospechadas. En la mayoría de ocasiones se realizan diagnósticos estructurales basados en la imagenologia como cardiomiopatía dilatada o cardiomiopatía restrictiva, lo que lleva al clínico a tratamientos sintomáticos, dejando de lado tratamientos etiológicos específicos que pudiesen preservar la función cardiaca, revertir el daño miocárdico y mejorar la calidad de vida de estos pacientes. Comprender y reconocer que existen factores nutricionales, hormonales, genéticos, infecciosos, medicamentosos, infiltrativos o autoinmunes en la patogénesis de la insuficiencia cardiaca permitirá optar por mejores tratamientos, mejorando las tasas de sobrevida.

Heart failure is a pathophysiologic result of several diseases and it's not only related to ischemic, valvular, arrhythmias or hypertensive. Most of the times structural diagnoses are made based on imaging, like dilated or restrictive cardiomyopathy, which leads to symptomatic treatments instead directed etiological therapies that could preserve heart function, reverse myocardial damage and improve life quality. Understands and recognize that there are nutritional, hormonal, genetic, infectious, drugs, infiltrative or autoimmune factors that could lead to heart failure would help clinicians to recognize them and propose targeted treatments, improving survival rates

Fibrosis endomiocárdica / Endomyocardial fibrosis

Resumen Las miocardiopatías son trastornos intrínsecos del músculo cardíaco. Presentan fenotipos diferenciales que determinan su clasificación; estos son: dilatada, hipertrófica, restrictiva, displasia arritmogénica del ventrículo derecho y no clasificadas. Las miocardiopatías restrictivas se caracterizan por ventrículos de tamaño normal, con grosores de pared normales o ligeramente aumentados, paredes rígidas, disfunción diastólica severa y llenado restrictivo con presiones elevadas. Una de las formas más comunes de miocardiopatía restrictiva es la fibrosis endomiocárdica la cual es endémica en algunas zonas tropicales especialmente en África (países de bajos ingresos), pero en nuestro medio hay pocos reportes de aparición. Su etiología es desconocida, aunque existen diversos mecanismos que han sido involucrados en su fisiopatología. Su diagnóstico se basa en estudios imagenológicos (ecocardiograma transtorácico y resonancia magnética nuclear cardíaca). El pronóstico es muy pobre, y usualmente se diagnostica en etapas muy avanzadas de la enfermedad. Se describe el caso de una paciente femenina, adulta media, que debutó con cardiopatía restrictiva, cuyo diagnóstico final fue fibrosis endomiocárdica.

Abstract Cardiomyopathies are intrinsic conditions of the cardiac muscle. They present differential phenotypes that determine their classification. These are: dilated, hypertrophic, restrictive, arrhythmogenic right ventricular and unclassified. Restrictive cardiomyopathies are characterised by larger than normal ventricles with normal or slightly enlarged thickness of the walls, rigid walls, severe diastolic dysfunction and restrictive filling with high pressures. One of the most common restrictive cardiomyopathies is endomyocardial fibrosis, which is endemic to some tropical areas, especially Africa (low income countries), but there are few reports of its occurrence in our environment. Its aetiology is unknown, but there are several mechanisms that have been involved in its pathophysiology. Its diagnosis is based in imaging studies (transthoracic echocardiogram, cardiac nuclear magnetic resonance). Prognosis is very poor, and it is usually diagnoses in the latest stages of the disease. The case of a female, average adult patient that debuted with restrictive cardiomyopathy with a final diagnosis of endomyocardial fibrosis is described.

Fibrosis endomiocárdica / Endomyocardial fibrosis

Resumen La fibrosis endomiocárdica o endocarditis de Löffler es una patología de causa todavía desconocida, esta puede presentarse durante la evolución de diversas enfermedades de causa infecciosa, tumoral, autoinmune, medicamentos, etc. En muchos casos el presentar eosinofilia moderada (más de 1500 eosinófilos/microlitro) por largos períodos de tiempo puede producir toxicidad en diferentes órganos, entre ellos el corazón, produciendo disfunción del mismo por infiltración directa lo cual daña el tejido y también por las proteínas encontradas en los gránulos, principalmente la proteína catiónica eosinofílica y la proteína básica mayor que tienen predilección por el tejido endocárdico, llevando a su destrucción celular, lo que se traducirá en engrosamiento y fibrosis del subendocardio. Estas alteraciones conllevan a la cardiomiopatía restrictiva, siendo la fibrosis endomiocárdica su principal causa. Se presenta el caso de un paciente masculino de 30 a˜nos de edad, que ingresa al hospital por un cuadro de falla cardiaca aguda con evidencia en el ecocardiograma de ingreso de un componente restrictivo biventricular, el cual en diferentes series se presenta hasta en el 51% de los casos. El paciente presentaba una enfermedad hematológica de base, donde la eosinofilia era persistente durante más de 6 meses.

Abstract Endomyocardial fibrosis or Löffler endocarditis is a condition whose cause still remains unknown. It can develop during the progress of multiple infectious or tumour diseases, medication, etc. In many cases, showing moderate eosinophilia (more than 1500 eosinophils/ microliter) for long periods of time can cause organ toxicity, among them the heart. This produces a dysfunction of the heart due to direct infiltration, which damages the tissue, and also due to the proteins found in the granules, mostly eosinophil cationic protein and major basic protein, which have a predilection for endocardial tissue, leading to their cell destruction, which will translate into a subcardial enlargement and fibrosis. These alterations result in restrictive cardiomyopathy, endomyocardial fibrosis being their main cause. We present the case of a 30 year-old male patient who is admitted at the hospital due to acute heart failure with an admission echocardiogram that evidenced a restrictive biventricular component, present in up to 51% of the cases in different series. The patient had a base hematological disorder, where eosinophilia had been persistent for more than 6 months.

Miocardiopatías restrictivas: Valoración por resonancia magnética cardiaca y tomografía multicorte / Restrictive Cardiomyopathies: Evaluation Using Cardiac MRI and Multidetector CT

Las miocardiopatías son condiciones que afectan al miocardio y generan alteración en la función cardiaca. Dentro de las miocardiopatías, el subgrupo de las restrictivas tiene como principal hallazgo la disminución en el llenado ventricular. A continuación se expone una revisión acerca de las miocardiopatías restrictivas, se analizan sus principales causas, y los hallazgos por resonancia magnética cardiaca y por tomografía computarizada. También se incluyen signos por imagen que ayudan a diferenciar las miocardiopatías restrictivas de la pericarditis constrictiva.

Cardiomyopathies are conditions that affect the myocardium and cause alteration in the cardiac function. Within the cardiomyopathies, the restrictive subgroup has as a main finding the decrease in the ventricular filling. In this manuscript we will review the restrictive cardiomyopathies and discuss their main causes, as well as their imaging findings on cardiac magnetic resonance and computed tomography. We will also include imaging signs that helps to differentiate restrictive cardiomyopathies from constrictive pericarditis.