Fibrosis Endomiocárdica: Presentación de Caso Clínico y Revisión de la literatura / Endomyocardial Fibrosis: Case Report and Literature Review

La fibrosis endomiocárdica (FEM) es una enfermedad rara y potencialmente letal caracterizada por la infiltración de tejido fibrótico en el endocardio y miocardio. Es más comúnmente observada en regiones tropicales y subtropicales, y se cree que es causada por la combinación de factores genéticos y ambientales. Su patogénesis aún no está del todo clara, pero es posible que esté relacionada con una respuesta inmune anormal ante múltiples estímulos, incluyendo infecciones virales, parásitos, trastornos hematológicos y deficiencias alimentarias. Esta condición es una de las principales manifestaciones de los síndromes hipereosinofílicos (SHE). Pese a los avances en el manejo, el pronóstico de los pacientes con FEM persiste siendo pobre, con un alto riesgo de recurrencia y de progresión hacia insuficiencia cardiaca. A continuación, presentaremos el caso de un paciente joven con un cuadro de insuficiencia cardiaca de predominio derecho en el contexto de un síndrome hipereosinofílico de base, en quien se establece el diagnóstico de FEM según lo documentado en las imágenes cardiacas.

Endomyocardial Fibrosis: Case Report and Literature Review. Endomyocardial fibrosis (EMF) is a rare and potentially life-threatening cardiac disorder characterized by the infiltration of fibrotic tissue into the endocardium and myocardium of the heart. It is most seen in tropical and subtropical regions, and it is believed to be caused by a combination of environmental and genetic factors. The exact pathogenesis of EMF is not fully understood, but it is thought to be related to an abnormal immune response to various stimuli, including viral infections, parasites, and dietary deficiencies. This condition is one of the main manifestations of hypereosinophilic syndromes. Despite advances in treatment, the prognosis for patients with EMF remains poor, with a high risk of recurrence and progression to heart failure. We present a case of a young male patient with an episode of right heart failure in the context of a chronic hypereosinophilic syndrome in which the diagnosis of EMF is made with the aid of cardiovascular imaging.

Surgical Management of The Endomyocardial Fibrosis of Right Ventricle Mimicking Tumor with Recurrent Pulmonary Embolism

ABSTRACT Endomyocardial fibrosis is a neglected tropical disease that leads to restrictive cardiomyopathy. Its etiopathogenis is unclear and involves the progression of 3 stages of the disease. Compared with echocardiography, cardiac magnetic resonance imaging shows better apical visualization of obliteration and thrombus and provides an early diagnosis. However, there is no specific drug therapy, although surgery can increase survival. Therefore, surgical resection of the fibrous and thickened endocardium is recommended for symptomatic patients. The risk of mortality increases as the ratio of endocardial fibrous tissue per body surface rises. The aim of this manuscript is to describe the surgical management of the right-sided endomyocardial fibrosis mimicking tumor with recurrent pulmonary embolism.

Myocardial fibrosis-related factors in patients with hypertrophic cardiomyopathy / 中国基层医药

Objective:To investigate myocardial fibrosis-related factors in patients with hypertrophic cardiomyopathy.Methods:Ninety-six patients with hypertrophic cardiomyopathy who received treatment in Zhoushan Hospital between January 2019 and January 2021 were included in this study. General data of all patients were collected. Cardiovascular magnetic resonance imaging was performed in all patients. Percentage of late gadolinium enhancement (LGE) was calculated. These patients were divided into positive and negative groups according to whether myocardial fibrosis existed. Related parameters were compared between the two groups. Correlations between related parameters and myocardial fibrosis range were analyzed.Results:Patient age in the positive group was significantly lower than that in the negative group [(42.84 ± 14.38) years vs. (50.71 ± 14.74) years, t = 2.04, P < 0.05]. The percentage of patients with New York Heart Association (NYHA) class III/IV heart function, N-terminal pro-brain natriuretic peptide (NT-proBNP) level, cardiac troponin (cTnI) level, creatine kinase-MB level, and myoglobin level in the positive group were 29.41% (20/68), 2 761.73 (1 505.22, 3 784.62) ng/L, 0.971 (0.447, 1.687) μg/L, (3.25 ± 2.65) μg/L and (66.14 ± 31.17) μg/L, respectively, which were significantly higher than those in the negative group [3.57% (1/68), 862.35 (551.48, 1 094.83) ng/L, 0.146 (0.037, 0.256) μg/L, (0.73 ± 0.22) μg/L, (28.82 ± 2.34) μg/L, t = 12.17, 55.28, 3.17, 5.18, 8.18, all P < 0.05]. Left ventricular ejection fraction and cardiac index in the positive group were (62.31 ± 17.89)% and (2.85 ± 0.71) L·min -1·(m 2) -1, respectively, which were significantly lower than those in the negative group ( t = 2.89, 6.18, both P < 0.05). Left ventricular end-diastolic volume (LVEDV), left ventricular maximum wall thickness (LVMWT) and left ventricular mass index (LVMI) in the positive group were (56.32 ± 17.28) mL/m 2, (2.24 ± 0.41) cm, (126.15 ± 12.34) g/m 2, which were significantly higher than those in the negative group ( t = 2.17, 2.75, 13.10, all P < 0.05). In the positive group, 18 patients had moderate and severe hypertrophy, 20 patients had moderate hypertrophy, and 30 patients had mild hypertrophy. There were significant differences in NT-proBNP, cTnI level and (LGE) extent between positive and negative groups ( t = 43.27, 5.28, 11.18, all P < 0.05). NT-proBNP, cTnI level and LGE extent increased with the increase in hypertrophy. Percentage of LGE was negatively correlated with patient age, and it was positively correlated with NT-proBNP, cTnI, LVDSV, LVMWT and LVMI. The differences were statistically significant (all P < 0.05). Conclusion:Patients with hypertrophic cardiomyopathy have a relatively high incidence of myocardial fibrosis. The extent of myocardial fibrosis is negatively correlated with patient age and it is positively correlated with NT-proBNP, cTnI, LVDSV, LVMWT and LVMI.

Fibrose Cardíaca e Mudanças Evolutivas na Função Ventricular Esquerda em Pacientes com Cardiopatia Chagásica Crônica / Cardiac Fibrosis and Changes in Left Ventricle Function in Patients with Chronic Chagas Heart Disease

Resumo Fundamento A cardiopatia chagásica (CC) é uma condição de progressão lenta, cujo principal achado histopatológico é fibrose. Objetivos Avaliar se a fibrose cardíaca aumenta ao longo do tempo e se correlaciona com aumento no tamanho do ventrículo esquerdo (CE) e redução na fração de ejeção (FE) na CC crônica. Métodos Estudo retrospectivo que incluiu 20 indivíduos (50% homens; 60±10 anos) com CC crônica que se submeteram a dois exames de ressonância magnética cardíaca (RMC) com realce tardio com gadolínio em um intervalo mínimo de quatro anos entre os exames. Volume, FE e massa de fibrose do ventrículo esquerdo (VE) foram determinados por RMC. Associações da massa de fibrose na primeira RMC com alterações no volume do VE e FE ventricular esquerda na segunda RMC foram testadas por análise de regressão logística. Valores p<0,05 foram considerados significativos. Resultados Os pacientes foram classificados em: A (n=13; alterações típicas de CC no eletrocardiograma e função sistólica global e segmentar do VE normal) e B1 (n=7; alteração na motilidade da parede do VE e FE ≥45%). O tempo médio entre os dois estudos de RMC foi de 5,4±0,5 anos. Fibrose do VE (em % massa do VE) aumentou de 12,6±7.9% para 18,0±14,1% entre os exames de RMC (p=0,02). A massa de fibrose cardíaca no basal associou-se com uma diminuição > cinco unidades absolutas na FE ventricular esquerda da primeira para a segunda RMC (OR 1,48; IC95% 1,03-2,13; p=0,03). A massa de fibrose do VE foi maior e aumentou entre os dois estudos de RMC no grupo de pacientes que apresentaram diminuição na FE entre os testes. Conclusões Mesmo pacientes em estágios iniciais da CC apresentam um aumento na fibrose do miocárdio ao longo do tempo, e a presença de fibrose do VE no basal está associada a uma diminuição da função sistólica do VE.

Abstract Background Chagas heart disease (CHD) is a slow progressing condition with fibrosis as the main histopathological finding. Objectives To study if cardiac fibrosis increases over time and correlates with increase in left ventricular (LV) size and reduction of ejection fraction (EF) in chronic CHD. Methods Retrospective study that included 20 individuals (50% men; 60±10 years) with chronic CHD who underwent two cardiac magnetic resonance imaging (MRI) with late gadolinium enhancement with a minimum interval of four years between tests. LV volume, EF, and fibrosis mass were determined by cardiac MRI. Associations of fibrosis mass at the first cardiac MRI and changes in LV volume and EF at the second cardiac MRI were tested using logistic regression analysis. P values <0.05 were considered significant. Results Patients were classified as follows: A (n=13; changes typical of CHD in the electrocardiogram and normal global and segmental LV systolic function) and B1 (n=7; LV wall motion abnormality and EF≥45%). Mean time between cardiac MRI studies was 5.4±0.5 years. LV fibrosis (in %LV mass) increased from 12.6±7.9% to 18.0±14.1% between MRI studies (p=0.02). Cardiac fibrosis mass at baseline was associated with decrease in >5 absolute units in LV EF from the first to the second MRI (OR 1.48, 95% CI 1.03-2.13, p=0.03). LV fibrosis mass was larger and increased between MRI studies in the group that presented decrease in LV EF between the tests. Conclusions Even patients at an initial stage of CHD show an increase in myocardial fibrosis over time, and the presence of LV fibrosis at baseline is associated with a decrease in LV systolic function.

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.

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

Determinação do Tecido Cicatricial do Miocárdio no Fenômeno de Fluxo Coronário Lento e a Relação entre a Quantidade de Tecido Cicatricial e o Nt-ProBNP / Determination of Myocardial Scar Tissue in Coronary Slow Flow Phenomenon and The Relationship Between Amount of Scar Tissue and Nt-ProBNP

Resumo Fundamento A fisiopatologia e o prognóstico não estão claramente determinados nos pacientes com fenômeno do fluxo coronário lento (FCL). Esses pacientes apresentam várias condições clínicas, que variam desde quadro assintomático até internação hospitalar com morte cardíaca súbita. Objetivos Nosso objetivo foi avaliar os achados da ressonância magnética cardíaca (RMC) com o realce tardio pelo gadolínio (RTG), como um indicador de fibrose miocárdica. Também buscamos determinar a relação entre a presença de fibrose miocárdica e os níveis de NT-proBNP em pacientes com FCL na artéria coronária descendente anterior esquerda (DAE). Métodos Ao todo, 35 pacientes, entre 31 e 75 anos de idade, foram incluídos. Os pacientes estudados (n=19) apresentaram artérias coronárias epicárdicas normais na angiografia, mas tinham FCL na DAE. O grupo controle de pacientes (n=16) apresentou artérias coronárias epicárdicas normais e níveis de escore TIMI normais na angiografia. Em ambos os grupos, os pacientes foram examinados com RMC para a detecção de presença de fibrose miocárdica. Além disso, níveis plasmáticos de NT-proBNP foram medidos. Valores de p < 0,05 foram considerados significativos. Resultados A taxa de fibrose miocárdica foi significativamente maior na RMC para os pacientes com FCL (p=0.018). Uma quantidade variável de tecido cicatricial foi detectada no ápice ventricular esquerdo em 7 pacientes e nas regiões inferior e inferolateral em 3 pacientes. Não foram observadas diferenças nos níveis de NT-proBNP nos pacientes com FCL. Entretanto, os níveis de NT-proBNP foram maiores nos pacientes com FCL, que apresentaram fibrose miocárdica na RMC (p=0.022). Conclusões Em suma, o RTG na RMC mostrou que a cicatriz miocárdica isquêmica pode estar presente nos pacientes com FCL. Esses resultados indicam que o FCL pode nem sempre ser inofensivo. (Arq Bras Cardiol. 2020; 114(3):540-551)

Abstract Background Pathophysiology and prognosis are not clearly determined in patients with the coronary slow flow phenomenon (CSFP). These patients present with various clinical conditions ranging from being asymptomatic to being admitted with sudden cardiac death. Objectives We aimed at assessing the findings of late gadolinium enhancement (LGE) in cardiac magnetic resonance imaging (CMR) as an indicator of myocardial fibrosis. We also aimed at determining the relationship between the presence of myocardial fibrosis and NT-proBNP levels in patients with CSFP in the left anterior descending coronary artery (LAD). Methods A total of 35 patients were enrolled within an age range of 31-75. The study patients (n=19) had normal epicardial coronary arteries at angiography, but they presented with CSFP in the LAD. The control group patients (n=16) had normal epicardial coronary arteries and TIMI scores at normal levels in angiography. In both groups, the patients were examined with CMR for the presence of myocardial fibrosis. In addition, plasma NT-proBNP levels were measured. A p-value < 0.05 was considered significant. Results The rate of myocardial fibrosis was significantly higher in CMR in the patients with CSFP (p=0.018). A variable amount of myocardial scar tissue was detected at the left ventricular apex in 7 patients and at the inferior and inferolateral regions in 3 patients. There was no difference in the level of NT-proBNP in patients with CSFP. However, the NT-proBNP levels were higher in patients with CSFP, who had scar tissue in CMR (p=0.022). Conclusions In conclusion, LGE in CMR showed that ischemic myocardial scarring may exist in patients with CSFP. These results indicate that CSFP may not always be innocent. (Arq Bras Cardiol. 2020; 114(3):540-551)

Cardiac magnetic resonance in tropical endomyocardial fibrosis

Background: Endomyocardial fibrosis has varied presentatation and difficult to diagnose. Aim to elucidate the role of Cardiac Magnetic Resonance (CMR) imaging in the evaluation of Endomyocardial Fibrosis (EMF) and to devise diagnostic criteria for the disease.Methods: Retrospective analysis of cases of restrictive cardiomyopathy referred for Magnetic resonance imaging over a period of 5 years. All patients underwent 1.5 T CMR imaging (Magnetom Avanto, Siemens, Germany) with standard cardiomyopathy protocol. Criteria for diagnosis of RCM included normal sized ventricles, normal/reduced systolic function, uni-/bi-atrial enlargement, normal pericardium and absent septal bounce. Cases diagnosed as EMF on CMR were included in this study. Statistical analysis performed using SPSS software.Results: EMF was diagnosed in 20 patients (31%) [12 males; age 39±18 years]. Ten patients had Right Centricular (RV) EMF, 3 had Left Ventricular (LV) EMF, while 7 had bi-ventricular EMF. Oedema indicating ongoing inflammation was seen in 4 (20%) cases. Apical thrombus was seen in 8(40%) cases and was present in 35% cases of RV and 20% cases of LV involvement. Subendocardial delayed enhancement was always present in the involved ventricle. The RV apex was obliterated in 100% of patients with RV EMF, while LV apex was similarly obliterated in 66% cases with LV disease. Mild-moderate pericardial effusion was observed in 8 patients. On the basis of CMR findings, the disease was classified as early necrotic phase in 1, thrombotic necrotic in 4 and late fibrotic phase in 13 and of different stages in ventricles in 2 cases.Conclusions: EMF was the commonest cause of RCM in this series. Major diagnostic criteria of EMF on CMR include subendocardial delayed enhancement and apical obliteration. Oedema and thrombus are variable findings, depending on disease severity.

Fibrosis miocárdica: hacia una nueva aproximación / Myocardial fibrosis: towards a new approach

Resumen La fibrosis miocárdica, que se evidencia en aquellas enfermedades que se presentan con sobrecarga de presión, como la estenosis aórtica o la cardiopatía hipertensiva, constituye una forma difusa de fibrosis progresiva y de tipo reactivo, con gran impacto en la función cardíaca, manifestada como disfunción diastólica y/o sistólica, desarrollo de arritmias e isquemia miocárdica. Se considera que precede los demás cambios estructurales del ventrículo izquierdo en este tipo de enfermedades y se caracteriza por la presencia de una matriz extracelular rica en fibras de colágeno tipos I y III con alto grado de entrecruzamiento. Constituye un marcador pronóstico y existe una correlación directa entre el grado de fibrosis miocárdica y desenlaces como hospitalización por insuficiencia cardiaca y mortalidad. El abordaje diagnóstico de la fibrosis miocárdica en este grupo de pacientes puede realizarse a través de biomarcadores químicos o de imagen; los primeros aún son materia de estudio para conseguir mayor precisión en sus resultados y la resonancia nuclear cardíaca con técnica de mapeo de T1 es la técnica de imagen más atractiva. El tratamiento de la fibrosis miocárdica en las enfermedades por sobrecarga de presión, está dado por el manejo estándar de la insuficiencia cardíaca, en cuyo caso los antihipertensivos de la familia de los IECA/ARAII se han relacionado en mayor nivel con modificación en el grado de fibrosis miocárdica; sin embargo, se necesitan más estudios para definir el uso de nuevos blancos terapéuticos que complementen el tratamiento y mejoren los desenlaces en este grupo de pacientes.

Abstract Myocardial fibrosis, which is present in those diseases that have a pressure overload, such as aortic stenosis or hypertensive heart disease, are a diffuse form of progressive and reactive-type fibrosis. This has a large impact on cardiac function, manifested as a diastolic and/or systolic dysfunction, development of arrhythmias or myocardial ischaemia. It is believed that it precedes the rest of the left ventricular structural changes in this type of disease, and is characterised by the presence of an extracellular matrix rich in collagen type I and III fibres with a high level of cross-linking. It is a prognostic marker and there is a direct correlation between the level of myocardial fibrosis and outcomes, such as heart failure and mortality. The diagnostic approach of myocardial fibrosis in this group of patients could be made using chemical or imaging biomarkers. The first ones are still study material in order to achieve a better precision in their results, and cardiac nuclear resonance with T1 mapping technique is the most attractive imaging technique. The treatment of myocardial fibrosis in diseases with pressure overload, is given by the standard management of heart failure, in which case the ACEI/ARAII family of hypertensive drugs have been associated at a higher level with a change in the grade of myocardial fibrosis. However, more studies are needed in order to define the use of new therapeutic targets that may complement the treatment and improve the outcomes in this group of patients.

Role of Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Systematic Review and Updated Meta-Analysis of Risk Markers for Sudden Death / Papel da Fibrose Miocárdica na Cardiomiopatia Hipertrófica: Revisão Sistemática e Metanálise Atualizada de Marcadores de Risco para Morte Súbita

Abstract Background: Hypertrophic cardiomyopathy (HCM) is associated with sudden death (SD). Myocardial fibrosis is reportedly correlated with SD. Objective: We performed a systematic review with meta-analysis, updating the risk markers (RMs) in HCM emphasizing myocardial fibrosis. Methods: We reviewed HCM studies that addressed severe arrhythmic outcomes and the certain RMs: SD family history, severe ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia (NSVT) on 24-hour Holter monitoring, abnormal blood pressure response to exercise (ABPRE), myocardial fibrosis and left ventricular outflow tract obstruction (LVOTO) in the MEDLINE, LILACS, and SciELO databases. We used relative risks (RRs) as an effect measure and random models for the analysis. The level of significance was set at p < 0.05. Results: Twenty-one studies were selected (14,901 patients aged 45 ± 16 years; men, 62.8%). Myocardial fibrosis was the major RISK MARKER (RR, 3.43; 95% CI, 1.95-6.03). The other RMs, except for LVOTO, were also predictors: SD family history (RR, 1.75; 95% CI, 1.39-2.20), severe ventricular hypertrophy (RR, 1.86; 95% CI, 1.26-2.74), unexplained syncope (RR, 2.27; 95% CI, 1.69-3.07), NSVT (RR, 2.79; 95% CI, 2.29-3.41), and ABPRE (RR, 1.53; 95% CI, 1.12-2.08). Conclusions: We confirmed the association of myocardial fibrosis and other RMs with severe arrhythmic outcomes in HCM and emphasize the need for new prediction models in managing these patients.

Resumo Fundamento: A cardiomiopatia hipertrófica (CMH) está associada à morte súbita (MS). A fibrose miocárdica está supostamente correlacionada à MS. Objetivo: Realizamos uma revisão sistemática com metanálise, atualizando os marcadores de risco (MR) em CMH enfatizando a fibrose miocárdica. Métodos: Revisamos estudos de CMH que abordaram desfechos arrítmicos graves e certos MR: história familiar de MS, hipertrofia ventricular grave, síncope inexplicada, taquicardia ventricular não sustentada (TVNS) na monitorização com Holter de 24 horas, resposta anormal da pressão arterial ao exercício (ABPRE), fibrose miocárdica e obstrução da via de saída do ventrículo esquerdo (VSVE) nas bases de dados MEDLINE, LILACS e SciELO. Utilizamos os riscos relativos (RRs) como uma medida de efeito e modelos aleatórios para a análise. O nível de significância foi estabelecido em p < 0,05. Resultados: Vinte e um estudos foram selecionados (14.901 pacientes com idade de 45 ± 16 anos; homens, 62,8%). A fibrose miocárdica foi o principal MARCADOR DE RISCO (RR, 3,43; IC95%, 1,95-6,03). Os outros MR, exceto obstrução da VSVE, também foram preditores: história familiar de MS (RR, 1,75; IC95%, 1,39-2,20), hipertrofia ventricular grave (RR, 1,86; IC95%, 1,26-2,74), síncope inexplicada (RR, 2,27; IC95%, 1,69-3,07), TVNS (RR, 2,79; IC95%, 2,29-3,41) e ABPRE (RR, 1,53; IC95%, 1,12-2,08). Conclusões: Confirmamos a associação de fibrose miocárdica e outros MR com desfechos arrítmicos graves na CMH e enfatizamos a necessidade de novos modelos de previsão no manejo desses pacientes.

Evaluation of Galectin-3 and myocardial fibrosis in patients with hypertrophic cardiomyopathy / Avaliação de Galectina-3 e fibrose miocárdica em pacientes com cardiomiopatia hipertrófica

Background: Galectin-3 is the designation given to the protein that binds to ß-galactosides, expressed by activated macrophages and described as a cardiac fibrosis mediator. In hypertrophic cardiomyopathy (HCM), myocardial fibrosis is an independent predictor of adverse outcome; however, the association between Galectin-3 and myocardial fibrosis has not been studied in this cardiopathy. Objective: To evaluate the association of Galectin-3 and the presence of myocardial fibrosis in a patient with hypertrophic cardiomyopathy. Methods: Galectin-3 was measured in automated equipment using the Elisa technique in 100 participants divided into two groups: 50 patients with hypertrophic cardiomyopathy and 50 healthy control subjects. All patients with hypertrophic cardiomyopathy underwent magnetic nuclear resonance with the late enhancement technique to investigate myocardial fibrosis. For the statistical analysis, p values < 0.05 were considered statistically significant. Results: Galectin-3 levels were low and did not show significant differences between patients with hypertrophic cardiomyopathy and the control group,10.3 ± 3.1 ng/dL and 11.3 ± 2.6 ng/dL (p = 0.12) respectively. Myocardial fibrosis was a common finding and was identified in 84% (42/50) of patients with HCM, but no differences were observed between Galectin-3 levels when comparing patients with and without fibrosis, 10.3 ± 2.4 ng/dL and 10.1 ± 2.1 ng/dL (p = 0.59). Conclusion: The results did not show an association between Galectin-3 and myocardial fibrosis in patients with hypertrophic cardiomyopathy, suggesting that non-inflammatory mechanisms of myocardial fibrosis formation and cardiac remodeling are involved in this cardiopathy