Endomiocardiofibrose associada a hipereosinofilia: desafio diagnóstico / Endomyocardial fibrosis associated with hypereosinophilia: a diagnostic challenge

INTRODUÇÃO A endomiocardiofibrose (EMF) é uma cardiomiopatia restritiva, caracterizada por envolvimento fibrótico do endocárdio e miocárdio normalmente na região apical, levando a disfunção diastólica, porém, preservando função sistólica. É uma doença de etiologia desconhecida e ainda muito relacionada a países subdesenvolvidos com forte relação às síndromes hipereosinofilicas. Seu diagnóstico pode ser aventado através do ecodopplercardiograma e ressonância magnética cardíaca, podendo muitas vezes ser confundida com trombo em região apical ventricular. DESCRIÇÃO DO CASO Paciente de 38 anos, feminina, proveniente de São Paulo capital. Relata internação anterior há 4 meses em serviço externo por quadro de dor torácica com alteração de enzimas cardíacas, porém, sem evidência de coronariopatia isquêmica na Cinecoronariografia. Evidenciado em ecocardiograma transtorácico (ECOTT) uma hipertrofia excêntrica do ventrículo esquerdo (VE) com imagem hiperecogênica em região apical de 19mm podendo corresponder a trombo. Prosseguindo a investigação, realizada uma ressonância magnética cardíaca descrevendo trombo aderido à parede do ventrículo esquerdo, medindo 2,3 cm, além de hipocinesia dos segmentos apicais e realce miocárdico tardio subendocárdico não transmural nos segmentos apicais. A mesma comparece ao serviço com novo episódio de dispneia e dor precordial, agora com sinais de hipervolemia e hipereosinofilia (>1500). Em novo ECOTT realizado evidenciou-se aumento biatrial, disfunção sistólica do ventrículo direito, hipertensão pulmonar, refluxo de grau importante em valva mitral e tricúspide associado a uma obliteração do ápice em parede endocárdica até base do VE, poupando demais paredes, sugestivo de EMF. Com base nos exames iniciais, configurou-se o diagnóstico de EMF pela ressonância magnética e sua correlação com hipereosinofilia. Dessa forma, após o diagnóstico a paciente evolui com melhora clínica e seguirá tratamento em ambulatório do miocárdio. CONCLUSÕES Relatamos um caso de EMF associada a hipereosinofilia ainda em investigação etiológica. O caso supracitado traz uma paciente jovem proveniente de um país em desenvolvimento com diagnóstico de uma doença ainda pouco compreendida e de difícil diagnóstico, podendo muitas vezes passar-se como um trombo em região apical cardíaca, gerando subdiagnósticos e manejo incorreto.

Endomyocardial fibrosis: past, present, and future.

Endomyocardial fibrosis (EMF) is a neglected idiopathic disorder, predominant in tropical and subtropical regions of the developing world. It is characterized by fibrotic thickening of the endocardium and myocardium of one or both ventricles. EMF was an important cause of heart failure which accounted for up to 20% of the cases in endemic areas of Africa (rural community in Mozambique), but during the last few years, incidents of the disease have decreased considerably. Although its pathogenesis and etiology are not fully understood, its pathology resembles conditions such as eosinophilic cardiomyopathy and hypereosinophilic syndrome. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling, and valvular insufficiency defines the disease. Confined to peculiar and limited geographical areas, the etiology remains blurred and it carries a grim prognosis. Medical care currently remains very challenging as one-third to half of patients with an advanced disease die within 2 years. Surgery in the correct setting can increase survival and especially in patients with advanced heart failure.

[Hypoxic hepatitis in a patient with endomyocardial fibrosis]. / Hepatitis hipóxica en paciente con endomiocardiofibrosis.

Endomyocardial fibrosis is a restrictive cardiomyopathy with high morbidity and mortality rates, prevalent in the sub-Saharan Africa region but infrequent in our population. It has a close relation with blood hypereosinophilia. Hypoxic hepatitis is frequently observed in intensive care units and its diagnosis is clinical. It shows a typical enzyme pattern with high mortality too. There are multiple mechanisms responsible for this condition, such as ischemia, passive congestion and dysoxia. We described the case of a 35 year-old cocaine addict woman diagnosed with endomyocardial fibrosis and hypereosinophilic syndrome who developed cardiogenic shock with hypoxic hepatitis. The patient evolved favorably with the appropriate treatment.

La endomiocardiofibrosis es una causa de miocardiopatía restrictiva frecuente en la región de áfrica subsahariana, aunque poco frecuente en nuestra población. Posee estrecha relación con la presencia de hipereosinofilia en sangre y tiene alta morbimortalidad. La hepatitis hipóxica es una afección clínica con un patrón enzimático característico, muy prevalente en unidades de cuidados intensivos y elevada mortalidad. Se reconocen múltiples mecanismos fisiopatológicos, como la isquemia, la congestión venosa y la alteración en la utilización de oxígeno del hepatocito. Describimos el caso de u na paciente de 35 años, consumidora de cocaína, con diagnóstico de endomiocardiofibrosis secundario a síndrome hipereosinofílico idiopático que presentó shock cardiogénico y hepatitis hipóxica asociada. Evolucionó favorablemente con el tratamiento de sostén adecuado.

Hepatitis hipóxica en paciente con endomiocardiofibrosis / Hypoxic hepatitis in a patient with endomyocardial fibrosis

La endomiocardiofibrosis es una causa de miocardiopatía restrictiva frecuente en la región de África subsahariana, aunque poco frecuente en nuestra población. Posee estrecha relación con la presencia de hipereosinofilia en sangre y tiene alta morbimortalidad. La hepatitis hipóxica es una afección clínica con un patrón enzimático característico, muy prevalente en unidades de cuidados intensivos y elevada mortalidad. Se reconocen múltiples mecanismos fisiopatológicos, como la isquemia, la congestión venosa y la alteración en la utilización de oxígeno del hepatocito. Describimos el caso de u na paciente de 35 años, consumidora de cocaína, con diagnóstico de endomiocardiofibrosis secundario a síndrome hipereosinofílico idiopático que presentó shock cardiogénico y hepatitis hipóxica asociada. Evolucionó favorablemente con el tratamiento de sostén adecuado.

Endomyocardial fibrosis is a restrictive cardiomyopathy with high morbidity and mortality rates, prevalent in the sub-Saharan Africa region but infrequent in our population. It has a close relation with blood hypereosinophilia. Hypoxic hepatitis is frequently observed in intensive care units and its diagnosis is clinical. It shows a typical enzyme pattern with high mortality too. There are multiple mechanisms responsible for this condition, such as ischemia, passive congestion and dysoxia. We described the case of a 35 year-old cocaine addict woman diagnosed with endomyocardial fibrosis and hypereosinophilic syndrome who developed cardiogenic shock with hypoxic hepatitis. The patient evolved favorably with the appropriate treatment.

Cardiac manifestations of parasitic diseases.

The heart may be affected directly or indirectly by a variety of protozoa and helminths. This involvement may manifest in different ways, but the syndromes resulting from impairment of the myocardium and pericardium are the most frequent. The myocardium may be invaded by parasites that trigger local inflammatory response with subsequent myocarditis or cardiomyopathy, as occurs in Chagas disease, African trypanosomiasis, toxoplasmosis, trichinellosis and infection with free-living amoebae. In amoebiasis and echinococcosis, the pericardium is the structure most frequently involved with consequent pericardial effusion, acute pericarditis, cardiac tamponade or constrictive pericarditis. Chronic hypereosinophilia due to helminth infections, especially filarial infections, has been associated with the development of tropical endomyocardial fibrosis, a severe form of restrictive cardiomyopathy. Schistosomiasis-associated lung vasculature involvement may cause pulmonary hypertension (PH) and cor pulmonale Tropical pulmonary eosinophilia, which is characterised by progressive interstitial fibrosis and restrictive lung disease, may lead to PH and its consequences may occur in the course of filarial infections. Intracardiac rupture of an Echinococcus cyst can cause membrane or secondary cysts embolisation to the lungs or organs supplied by the systemic circulation. Although unusual causes of cardiac disease outside the endemic areas, heart involvement by parasites should be considered in the differential diagnosis especially of myocardial and/or pericardial diseases of unknown aetiology in both immunocompetent and immunocompromised individuals. In this review, we updated and summarised the current knowledge on the major heart diseases caused by protozoan and metazoan parasites, which either involve the heart directly or otherwise influence the heart adversely.

Evaluation of Galectin-3 as a Novel Biomarker for Chagas Cardiomyopathy.

OBJECTIVES: Chagas cardiomyopathy has worse long-term outcomes than other cardiomyopathies. A biomarker strategy to refer subjects for noninvasive cardiac imaging may help in the early identification of cardiac damage in subjects with Chagas disease. Galectin-3 (Gal-3) is a mediator of cardiac fibrosis shown to be upregulated in animal models of decompensated heart failure. Here we assessed the correlation of Gal-3 with myocardial fibrosis in patients with Chagas disease. METHODS: This study comprised 61 subjects with Chagas disease. All subjects underwent clinical assessments, Doppler echocardiography and magnetic resonance imaging. Plasmatic Gal-3 was determined by ELISA. RESULTS: Delayed enhancement (DE) was identified in 37 of 61 subjects (64%). The total amount of myocardial fibrosis was 9.4% [interquartile interval (IQI): 2.4-18.4]. No differences were observed in Gal-3 concentration according to the presence or absence of myocardial fibrosis, with a median Gal-3 concentration of 11.7 ng/ml (IQI: 9.4-15) in subjects with DE versus 12.9 ng/ml (IQI: 9.2-14) in subjects without DE (p = 0.18). No correlation was found between myocardial fibrosis and Gal-3 concentration (r = 0.098; p = 0.47). CONCLUSIONS: There is no correlation between the degree of myocardial fibrosis and the concentration of Gal-3 in subjects with Chagas disease.

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.

Diagnosis of Rejection by Analyzing Ventricular Late Potentials in Heart Transplant Patients.

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.

Bloqueio atrioventricular avançado: uma manifestação incomum da endomiocardiofibrose / Advanced atrioventricular block: an unusual manifestation od endomyocardial fibrosis

A endomiocardiofibrose é uma causa importante de cardiopatia restritiva na zona tropical, ocasionalmente verificada no Brasil. Caracteriza-se pelo espessamento fibroso do endocárdio e do miocárdio subjacente, com progressão tardia para insuficiência cardíaca grave e prognóstico reservado. Relata-se aqui uma apresentação infrequente da doença: a insuficiência cardíaca de baixo débito, secundária a bloqueio atrioventricular avançado. O paciente foi tratado com sucesso por meio de implante de marcapasso DDD/R e encontra-se em classe funcional I (NYHA) há dois anos.

Endomyocardial fibrosis is an important cause of restrictive cardiomyopathy observed in tropical areas and has been occasionally found in Brazil. It is characterized by fibrous thickening of the endocardium and underlying myocardium with late progression to severe heart failure and poor prognosis. We report an unusual presentation of the disease: low output heart failure secondary to advanced atrioventricular block. The patient was successfully treated by implanting a DDD/R pacemaker and has been in functional class I (NYHA) for 2 years.

Eosinophilic endocarditis and Strongyloides stercoralis.

A 40-year-old woman from El Salvador presented with 3 months of abdominal pain and diarrhea followed by 2 weeks of atypical chest pain and exertional dyspnea and was diagnosed with eosinophilic endocarditis secondary to Strongyloides stercoralis infection. Transthoracic echocardiogram revealed apical masses in the left and right ventricles and a thickened posterior mitral valve leaflet and cardiac magnetic resonance imaging confirmed the presence of a left ventricular apical mass with diffuse subendocardial delayed enhancement consistent with endocardial fibrosis. In conclusion, eosinophilic endocarditis is a rare cause of restrictive cardiomyopathy characterized by endomyocardial fibrosis and apical thrombosis and fibrosis with frequent involvement of the posterior mitral valve leaflet.

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.

An unusual association of endomyocardial fibrosis and hypertrophic cardiomyopathy in a patient with heart failure.

A 69-year-old female patient presented heart failure with preserved ejection fraction and atrial fibrillation. Echocardiography and late gadolinium enhancement magnetic resonance imaging were suggestive of endomyocardial fibrosis (EMF). The patient underwent cardiac surgery, and after surgery, she developed low cardiac output syndrome and died. Postmortem examination revealed residual fibrosis of the left ventricle (LV), mild endocardial fibrous deposition of the right ventricle, and severe concentric, symmetrical LV hypertrophy. Histological examination of the surgically resected material from the LV confirmed EMF. Histopathology of the interventricular septum disclosed myocardial hypertrophy and disarray plus fine interstitial fibrosis, typical of hypertrophic cardiomyopathy. The present case illustrates the association of two different patterns of cardiomyopathies in the same patient.

Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients.

BACKGROUND: Endocardial fibrous tissue (FT) deposition is a hallmark of endomyocardial fibrosis (EMF). Echocardiography is a first-line and the standard technique for the diagnosis of this disease. Although late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) allows FT characterization, its role in the diagnosis and prognosis of EMF has not been investigated. METHODS AND RESULTS: Thirty-six patients (29 women; age, 54±12 years) with EMF diagnosis after clinical evaluation and comprehensive 2-dimensional Doppler echocardiography underwent cine-CMR for assessing ventricular volumes, ejection fraction and mass, and LGE-CMR for FT characterization and quantification. Indexed FT volume (FT/body surface area) was calculated after planimetry of the 8 to 12 slices obtained in the short-axis view at end-diastole (mL/m(2)). Surgical resection of FT was performed in 16 patients. In all patients, areas of LGE were confined to the endocardium, frequently as a continuous streak from the inflow tract extending to the apex, where it was usually most prominent. There was a relation between increased FT/body surface area and worse New York Heart Association functional class and with increased probability of surgery (P<0.05). The histopathologic examination of resected FT showed typical features of EMF with extensive endocardial fibrous thickening, proliferation of small vessels, and scarce inflammatory infiltrate. In multivariate analysis, the patients with FT/body surface area >19 mL/m(2) had an increased mortality rate, with a relative risk of 10.8. CONCLUSIONS: Our study provides evidence that LGE-CMR is useful in the diagnosis and prognosis of EMF through quantification of the typical pattern of FT deposition.

Comparação de desempenho entre duas gerações de eletrodos de fixação ativa com esteroides e superfície fractal / Performance comparison between two generation of active fixation leads with steroids and fractal surface / Comparación de desempeño entre dos generaciones de electrodos de fijación activa con esteroides y superficie fractal

Grande parte do sucesso e eficácia de um sistema de estimulação cardíaca artificial é, diretamente, devido a performance dos eletrodos utilizados. Os eletrodos endocárdicos são também os itens que estão mais expostos à ação deletéria do agressivo ambiente em que são instalados. Na evolução técnica desses itens, observa-se principalmente: a facilidade de uso durante o implante; a otimização dos resultados paramétricos e a busca pela máxima confiabilidade. Assim, o presente trabalho compara o desempenho de duas gerações consecutivas de eletrodos bipolares endocárdicos de fixação ativa, da empresa Biotronil GmbH: os modelos Selox SR e Setrox S. Este estudo clínico envolveu 175 Setrox S(43 VVI e 66 DDD - 66 atriais e 109 ventriculares) e 79 Selox SR (SR VVI e 22 DDD - 22 atriais e 57 eletrodos ventriculares), no período de trinta e oito meses. Além dos parâmetros elétricos dos eletrodos...

Much of the success and effectiveness of an artificial cardiac pacing system is directly due to the performance of electrodes in use. The endocardial electrodes are also items that are mostexposed to any deleterious effects from the aggressive environment in which they are installed. It is observed in the technical evolution of these items, namely: the ease of use during the implantation, the optimization of electrical parameters and search for maximum reliability. Thus, this study compares the performance of two consecutive generations of bipolar endocardial electrodes of active fixation, from the company Biotronik GmbH: the models Selox SR and Setrox S. This clinical study involving 175 Setrox S (43 VVI and 66 DDD - 66 atrial and 109 ventricular) and 79 Selox SR (35 VVI and 22 DDD - 22 atrialand 57 ventricular leads) during the thirty-eight months. In addition to the electrical parameters of electrodes...

Gran parte del éxito y la eficacia de un sistema de estimulación cardiaca artificial se debe directamente al desempeño de los electrodos utilizados. Los electrodos endocárdicos son asimismo los componentes que están más expuestos a la acción deletérea del agresivo entorno en el que se los instala.En la evolución técnica de dichos componentes se advierte especialmente: la facilidad de manejo durante el implante; la optimización de los resultados paramétricos y la búsqueda por la máxima confiabilidad. De esa manera, el presente trabajo compara el desempeño de dos generaciones consecutivas de electrodos bipolares endocárdicos de fijación activa, de la empresa Biotronik GmbH: los modelos Selox SR y SetroxS. Este estudio clínico abarcó 175 Setrox S (43 VVI y 66 DDD - 66 auriculares y 109 ventriculares) y 79Selox SR (35 VVI y 22 DDD - 22 auriculares y 57 electrodos ventriculares), en el período de treinta y ocho meses. Además de los parámetros eléctricos de los electrodos...