Ocupação apical por endomiocardiofibrose associada a trombo diagnosticada com ecocardiografia de contraste e resolvida com tratamento clínico / Apical occupation by endomyocardiofibrosis associated with thrombus diagnosed with contrast echocardiography and resolved with clinical treatment

A endomiocardiofibrose é uma doença negligenciada e predominante em países subdesenvolvidos. Apesar de sua frequência, ainda é considerada rara. Assim, a doença é marcada pelos seguintes achados: distorção arquitetural, alterações no enchimento ventricular e alterações da mobilidade segmentar. Ela afetando não só a dinâmica diastólica e sistólica, como também as funções das válvulas cardíacas. Em uma condição mais avançada da doença, pode haver formação de trombos apicais. Neste relato de caso, revisitamos a endomiocardiofibrose, apresentando um caso desafiador de paciente do sexo feminino de 52 anos, com clínica de insuficiência cardíaca classe funcional IV da New York Heart Association. A eletrocardiografia de repouso revelou ritmo sinusal com sobrecarga do ventrículo esquerdo com vetores de alta voltagem, infradesnivelamento retificado do segmento ST e onda T negativa em região anterolateral, podendo ser compatível com padrão de strain. Já na avaliação de imagens ecocardiográficas, revelou dilatação acentuada do átrio esquerdo, com ventrículos sem dilatações e imagem de ocupação apical hiperrefringente no interior do ventrículo esquerdo, sugerindo grande trombo séssil sobreposto à capa fibrosa endocárdica. Embora a conduta cirúrgica seja a mais apoiada em literatura em tais situações, a paciente aqui apresentada recebeu tratamento com anticoagulação oral por 1 mês e 24 dias. Ela evoluiu clinicamente bem, com melhora da classe funcional da New York Heart Association e, em ecocardiografia realizada 2 meses após o início da anticoagulação oral, houve demonstração da regressão da imagem de trombo apical, apoiada com a técnica de contraste endocavitário.(AU)

Endomyocardial fibrosis (EMF) is a neglected but prevalent disease in underdeveloped countries. Despite its frequency, it is still considered a rare disease. It is marked by the following findings: architectural distortion, ventricular filling changes, and segmental mobility changes affecting not only diastolic and systolic dynamics but also heart valve function. Apical thrombi can be formed in more advanced disease. In this case report, we revisit EMF and present the challenging case of a 52-year-old woman with New York Heart Association (NYHA) functional classification (FC) IV heart failure. Resting electrocardiography revealed sinus rhythm with left ventricular (LV) overload, high voltage vectors, rectified ST-segment depression, and a negative T-wave in the anterolateral region compatible with the strain pattern. The evaluation of echocardiographic images showed marked left atrial dilation, no ventricular dilatation, and hyper-refringent apical occupation within the LV suggestive of a large sessile thrombus superimposed on the endocardial fibrous layer. Although surgery is the most supported approach in the literature in such situations, this patient was treated with oral anticoagulants (OAC) for 1 month and 24 days. The patient progressed well with an improved NYHA FC. Endocavitary contrast echocardiography performed 2 months after OAC initiation showed regression of the apical thrombus image. (AU)

Fibrosis endomiocárdica crónica: reporte de caso en área no endémica / Chronic endomyocardial fibrosis: case report in a non-endemic area

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.

Diagnosis of Rejection by Analyzing Ventricular Late Potentials in Heart Transplant Patients / Diagnóstico de Rejeição por Análise de Potenciais Ventriculares Tardios em Doentes Transplantados ao Coração

Background: Heart transplant rejection originates slow and fragmented conduction. Signal-averaged ECG (SAECG) is a stratification method in the risk of rejection. Objective: To develop a risk score for rejection, using SAECG variables. Methods: We studied 28 transplant patients. First, we divided the sample into two groups based on the occurrence of acute rejection (5 with rejection and 23 without). In a second phase, we divided the sample considering the existence or not of rejection in at least one biopsy performed on the follow-up period (rejection pm1: 18 with rejection and 10 without). Results: On conventional ECG, the presence of fibrosis was the only criterion associated with acute rejection (OR = 19; 95% CI = 1.65-218.47; p = 0.02). Considering the rejection pm1, an association was found with the SAECG variables, mainly with RMS40 (OR = 0.97; 95% CI = 0.87-0.99; p = 0.03) and LAS40 (OR = 1.06; 95% IC = 1.01-1.11; p = 0.03). We formulated a risk score including those variables, and evaluated its discriminative performance in our sample. The presence of fibrosis with increasing of LAS40 and decreasing of RMS40 showed a good ability to distinguish between patients with and without rejection (AUC = 0.82; p < 0.01), assuming a cutoff point of sensitivity = 83.3% and specificity = 60%. Conclusion: The SAECG distinguished between patients with and without rejection. The usefulness of the proposed risk score must be demonstrated in larger follow-up studies.

Fundamento: A rejeição do transplante cardíaco origina zonas de condução lenta e fragmentada. O eletrocardiograma de alta resolução (ECGAR) é um método potencial de estratificação de risco da rejeição. Objetivo: Elaborar um escore de risco para rejeição, recorrendo ao ECGAR. Métodos: Estudaram-se 28 pacientes transplantados. Numa primeira fase, baseando-nos no diagnóstico de rejeição aguda, dividimos a amostra em dois grupos (5 pacientes com rejeição, 23 sem rejeição). Numa segunda fase, a divisão da amostra teve em conta o diagnóstico de rejeição em pelo menos uma biopsia realizada durante o seguimento (rejeição pm1) (18 pacientes com rejeição, 10 sem rejeição). Resultados: Para rejeição aguda, a única variável a revelar associação foi fibrose, evidenciando um aumento do risco de rejeição quando presente no ECG (OR = 19; IC 95% = 1,65-218,47; p = 0,02). Para rejeição pm1, constatamos que, para cada diminuição de unidade da RMS40, ocorre aumento de 7% do risco de rejeição (OR = 0,97; IC 95% = 0,87-0,99; p = 0,03) e que o aumento da LAS40 aumenta 1,06 vez o risco de rejeição (OR = 1,06; IC 95% = 1,01-1,11; p = 0,03). Formulamos um escore constituído por essas variáveis e aplicamos aos 28 indivíduos da amostra. A associação de fibrose, valores crescentes da LAS40 e valores decrescentes da RMS40 tem uma boa capacidade para distinguir doentes com e sem rejeição (AUC = 0,82; p < 0,01), assumindo um ponto de corte com sensibilidade = 83,3% e especificidade = 60%. Conclusão: O ECGAR distingue doentes com e sem rejeição. A utilidade do escore proposto deverá ser demonstrada em estudos de seguimento englobando uma amostra de maiores dimensões.

Colecistite acalculosa complicada em pacientews com endomiocardiofibrose / Acalculous cholecystitis in a patient complicated by endomyocardial fibrosis

Relata-se o caso de paciente jovem, do sexo feminino, com quadro clínico de colecistite acalculosa e concomitante endomiocardiofibrose biventricular até então não diagnosticada. A relação entre as duas doenças é discutida, questionando a possibilidade da eosinofilia e da insuficiência cardíaca como fatores etiopatogênicos ou agravantes a ambas as entidades. A paciente evoluiu com morte de causa indeterminada.

Does quantitative left ventricular regional wall motion change after fibrous tissue resection in endomyocardial fibrosis?

OBJECTIVES: We compared left ventricular regional wall motion, the global left ventricular ejection fraction, and the New York Heart Association functional class pre- and postoperatively. INTRODUCTION: Endomyocardial fibrosis is characterized by fibrous tissue deposition in the endomyocardium of the apex and/or inflow tract of one or both ventricles. Although left ventricular global systolic function is preserved, patients exhibit wall motion abnormalities in the apical and inferoapical regions. Fibrous tissue resection in New York Heart Association FC III and IV endomyocardial fibrosis patients has been shown to decrease morbidity and mortality. METHODS: We prospectively studied 30 patients (20 female, 30±10 years) before and 5±8 months after surgery. The left ventricular ejection fraction was determined using the area-length method. Regional left ventricular motion was measured by the centerline method. Five left ventricular segments were analyzed pre- and postoperatively. Abnormality was expressed in units of standard deviation from the mean motion in a normal reference population. RESULTS: Left ventricular wall motion in the five regions did not differ between pre- and postoperative measurements. Additionally, the left ventricular ejection fraction did not change after surgery (0.45±0.13 percent x 0.43±0.12 percent pre- and postoperatively, respectively). The New York Heart Association functional class improved to class I in 40 percent and class II in 43 percent of patients postoperatively (p<0.05). CONCLUSIONS: Although endomyocardial fibrosis patients have improved clinical symptoms after surgery, the global left ventricular ejection fraction and regional wall motion in these patients do not change. This finding suggests that other explanations, such as improvements in diastolic function, may be operational.

Neglected Cardiomyopathies in Africa

"Cardiomyopathies are forms of disease in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease; hypertension; valve disease and congenital heart disease sufficient to explain he observed myocardial abnormality. They constitute the greatest challenge of all the cardiovascular diseases in Africa; due to the difficulties in diagnosing and managing them; related to the lack of access to specialised investigations or effective interventions in most endemic areas. In this continent; in addition to the ""usual"" forms of cardiomyopathy; there is an increased incidence of specific forms of muscle heart disease like Endomyocardial Fibrosis and Peripartum Cardiomyopathy which; despite affecting thousands of people; have been largely neglected. Their etiology remains unknown; the mechanisms involved are not fully nderstood; and although some improvement in management has been witnessed in recent years much still need to be done to improve the outcome. The current knowledge on epidemiology; determinants; thophysiology; diagnosis and management of Endomyocardial Fibrosis and Peripartum Cardiomyopathy is reviewed in this article; which also presents the ongoing efforts for better understanding of their pathogenesis and the recent improvements in their management."

Endomiocardiofibrose biventricular associada à amiloidose renal / Biventricular endomyocardiofibrosis associated with renal amyloidosis

A endomiocardiofibrose (EMF) é uma cardiomiopatia restritiva, caracterizada por envolvimento fibrótico do endocárdio e miocárdio adjacente, e disfunção diastólica, causada por alteração da distensibilidade, dificultando o enchimento ventricular adequado, estando a função sistólica preservada. Clinicamente, apresenta-se como insuficiência cardíaca, mas, para o diagnóstico etiológico correto, são necessárias perspicácia semiológica, suspeita e investigação clínica. Relatamos o caso de uma paciente portadora de endomiocardiofibrose biventricular associada à amiloidose renal.

Low coronary perfusion pressure is associated with endocardial fibrosis in a rat model of volume overload cardiac hypertrophy

No remodelamento que se segue às sobrecargas de volume não é descrito o aumento de fibrose miocárdica. Após o infarto, entretanto, há hipertrofia do miocárdio remoto com acúmulo de fibrose, particularmente no subendocárdio. Na fístula aorto-cava, tal como no infarto, é possível que a queda da pressão de perfusão coronariana interfira com a fibrose cardíaca. OBJETIVO: Investigar o papel das mudanças hemodinâmicas agudas sobre a fibrose cardíaca na fístula aorto-cava. MÉTODO: Ratos Wistar submetidos a fístula aorto-cava, seguidos por 4 e 8 semanas, constituíram 4 grupos, fístula aorto-cava 4 e fístula aorto-cava 8 (10 ratos cada) e seus respectivos controles (sham-operated controls - Sh), Sh4 e Sh8 (8 ratos cada). A hemodinâmica foi realizada 1 semana após a cirurgia. A hipertrofia e a fibrose foram quantificadas ao final do seguimento pelo diâmetro dos miócitos e pela fração de volume do colágeno. RESULTADOS: Comparados com Sh4 e Sh8, a pressão de pulso, a pressão diastólica final do ventrículo esquerdo e a +dP/dt foram maiores em fístula aorto-cava 4 e fístula aorto-cava 8, enquanto a -dP/dt foi similar. A pressão estimada da perfusão coronariana (mmHg) foi menor em fístula aorto-cava 8 (52,6±4,1) do que em Sh8 (100,8±1,3), mas comparável entre fístula aorto-cava 4 (50,0±8,9) e Sh4 (84,8±2,3). O diâmetro dos miócitos foi maior em fístula aorto-cava 8 e a fibrose intersticial e subendocárdica maior em fístula aorto-cava 4 e fístula aorto-cava 8. Houve correlação inversa e independente da pressão de perfusão coronariana com a fibrose subendocárdica (r2=0,86; p<0,0001) e das pressões sistólica (r2=0,73; p=0,0035) e diastólica final do ventrículo esquerdo (r2=0,55; p=0.0124) com a fibrose intersticial. CONCLUSÃO: A queda precoce da pressão de perfusão coronariana e o aumento das pressões ventriculares após a fístula aorto-cava associam-se com fibrose miocárdica subseqüente.

Visceral organ involvement is infrequent in oral submucous fibrosis (OSF).

Associated visceral organ involvement evidence by systemic fibrosis has not been explored in oral submucous fibrosis (OSF). The investigations in this aspect were limited to loco-regional sites of naso/oropharynx and oesophagus. The study of whether the oral fibrosis is part of a systemic spectrum of disease involving multiple organs is an interesting pursuit. With this intention the patients diagnosed on clinical and histological grounds for OSF were concurrently tested by biophysical means for the presence of endomyocardial fibrosis (EMF), pancreatic (PF) and retroperitoneal fibrosis (RPF), which are endemic to the area studied. Twenty-five (n = 25) cases of OSF who visited the Department of Oral pathology & Microbiology. Govt. Dental College, Trivandrum, India for symptomatic relief of their illness comprised the study group. Ten (n = 10) age and sex matched healthy volunteers comprised the control. All the subjects have had undergone cardiologic and gastrointestinal investigations to rule out the possibility of concurrent EMF and PF. The patients were all of Indian ethnic extraction and mostly (> 90%) were from low socio economic classes. The mean age of the patients was 54.16 +/- 14.6 years, including 18 females and 7 males (F:M = 2.57:1). The severity of fibrosis was unrelated to the age of patients (P > 0.05). All the patients were chewers of areca quid (12%)/tobacco (88%). In addition to quid chewing 3/25 (12%) patients smoked 'bidi' and 6/25 (24%) consumed home brewed liquor (arrack/toddy) which contain about 40-50% ethanol. Statistically no relationship was observed between the clinical stages of OSF and severity of epithelial dysplasia in this study (P > 0.05). Out of the 25 patients, 5 (20%) showed sclerotic aortic value which may be an age related finding. Also 7 (28%) patients were found to be hypertensive and interstitial lung disease was present in 2 (8%). The possibility of EMF in one female patient who showed thickened RV apical endocardium was ruled out by cardiac catheterisation. Thus none of the patients showed evidence of endomyocardial fibrosis. The pancreas was found to be hyperchoic in 8(32 1/4) by ultra sonography. Liver was found to be hyperchoic in 6 (24%). Fat stain in stool samples was found to be positive in 13(58%). The hyperchogenecity of pancreas may be due to alcoholism or an underlying endocrine pancreatic insufficiency like diabetes and not due to pancreatic fibrosis. The positivity of fat stain could be due to fatty liver/alcoholism. Thus the study fails to reveal any evidence of pancreatic fibrosis in the group. The lack of any evidence of an associated visceral organ fibrosis in OSF made it prudent to believe that this is a loco-regional disease, initiated by local factors and propagated under their influence without systemic involvement.

Endomyocardial fibrosis (Davies Disease) coincidental with systemic lupus erythematosus

Los autores presentan el caso de una mujer de 27 años, con signos y síntomas de insuficiencia cardiaca congestiva rebelde al tratamiento, anemia, úlceras en la mucosa gingival, fotosensibilidad y alopecia. Los datos electrocardiográficos, ecocardiográficos, angiográ-ficos y hemodinámicos orientaron el diagnóstico de cardiomiopatía restrictiva, insuficiencia mitral secundaria a prolapso mitral y dilatación biauricular. El examen histológico de la biopsia endomiocárdica, tomada durante el cateterismo cardiaco, mostró signos de fibrosis endomiocárdica y el examen inmunológico resultó compatible con lupus eritematoso sistémico. Hasta donde sabemos, este es el primer caso de fibrosis endomiocárdica (enfermedad de Davies) asociado con lupus eritematoso sistémico publicado en la literatura médica. Permanece incierta la etiología de la fibrosis endomiocárdica y su asociación con lupus eritematoso sistémico sugiere el probable origen autoinmune de este padecimiento.

Endomiocardiofibrose com calcificaçäo maciça do ventrículo esquerdo / Endomyocardial fibrosis associated with massive calcification of the left ventricle

This is the report of a rare case of endomyocardial fibrosis associated with massive calcification of the left ventricle in a male patient with dyspnea on great exertion, which began 5 years earlier and rapidly evolved. Due to lack of information and the absence of clinical signs that could characterize impairment of other organs, the case was initially managed as a disease with a pulmonary origin. With the evolution of the disease and in the presence of radiological images of heterogeneous opacification in the projection of the left ventricle, the diagnostic hypothesis of endomyocardial disease was established. This hypothesis was later confirmed on chest computed tomography. The patient died on the 16th day of the hospital stay, probably because of lack of myocardial reserve, with clinical findings of refractory heart failure, possibly aggravated by pulmonary infection. This shows that a rare disease such as endomyocardial fibrosis associated with massive calcification of the left ventricle may be suspected on a simple chest X-ray and confirmed by computed tomography.

Granulomas cardíacos a ovos de schistosoma e fibrose endomiocárdica / Cardiac Granulomas due to Schistosoma Eggs and Endomyocardial Fibrosis

Evidences of a possible association between endomyocardial fibrosis and schistosomiasis have been recently investigated. We describe the finding of cardiac schistosomal granulomas in a 14 year-old-girl presenting symptoms and signals of right side endomyocardial fibrosis. Refractory ascistis and progressive atrioventricular block were observed during follow-up. Endomyocardial biopsy and post mortem specimens showed inflammatory infiltrates, schistosomal granuloma and fibrosis.

Evidências da possível correlação entre fibrose endomiocárdica e esquistossomose mansônica têm sido recentemente suspeitadas. Relatamos a presença de um granuloma esquistossomótico, em jovem de 14 anos, com quadro clínico de endomiocardiofibrose do coração direito, evoluindo com ascite refratária e bloqueio atrioventricular do 1º grau que progrediu para bloqueio atrioventricular total. As amostras de tecido cardíaco, obtidas durante biopsia transoperatória e análise histopatológica do coração postmortem, revelaram presença de infiltrado inflamatório, granuloma esquistossomótico e fibrose

Endomiocardiofibrose com calcificaçäo endocárdica maciça biventricular / Endomyocardial Fibrosis with Massive Biventricular Endocardial Calcification

Endomiocardiofibrose é uma doença cardíaca endêmica caracterizada pela presença de tecido fibroso no endocárdio, eventualmente se estendendo para o miocáardio.CAlcificaçäo endocárdica maciça de ventrículo esquerdo é um achado raro, com poucos casos descritos na literatura.Relatamos o primeiro caso, de nosso conhecimento, de calcificaçäo endocárdica maciça biventricular associada a endomiocardiofibrose em mulher de 22 anos.

Endomiocardiofibrose / Endomyocardiofibrosis

Relata-se o caso de uma paciente feminina, branca, 67 anos, que apresentou sinais e sintomas de insuficiência cardíaca severa que, após investigaçäo, mostrou tratar-se de miocardiopatia restritiva por endomiocardiofibrose. Os autores fazem uma breve revisäo bibliográfica, destacando os aspectos diagnósticos e terapêuticos desta patologia