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.

Imatinib atenua a fibrose miocárdica em associação com a inibição da atividade do PDGFRα / Imatinib attenuates myocardial fibrosis in association with inhibition of the PDGFRα activity

FUNDAMENTO: O Imatinib é um inibidor do receptor tirosina-quinase que foi confirmada como exercendo um efeito inibidor sobre a atividade do receptor do PDGF, fator de crescimento plaquetário (PDGFRα e PDGFRβ). OBJETIVO: Investigar o efeito protetor do Imatinib na fibrose miocárdica em acetato de deoxicorticosterona (DOCA)/ratos com hipertensão induzida por sal. MÉTODOS: Sessenta ratos Sprague-Dawley machos, uninefrectomizados foram distribuídos em três grupos: ratos controles (grupo CON): grupo deoxicorticosterona (grupo DOCA); grupo deoxicorticosterona e Imatinib (grupo DOCA IMA). A Pressão Arterial Sistólica (PAS) foi medida quinzenalmente. Foi estudada a porção apical do ventrículo esquerdo. Foram empregados: coloração vermelho sirius, coloração de hematoxilina-eosina, imuno-histoquímica e ensaio de western blot. RESULTADOS: A PAS nos grupos DOCA e IMA+DOCA foi maior que no grupo CON nos dias 14 e 28. Os animais do grupo DOCA apresentaram fibrose intersticial e perivascular grave no dia 28, e as expressões de PI, PIII, tenascina-C e fibronectina foram significativamente maiores que nos grupos DOCA+IMA e CON. Quando comparados com o grupo CON, os grupos DOCA e DOCA+IMA apresentaram resposta inflamatória de tecido miocárdico e infiltração de monócitos/macrófagos de diferentes graus. As expressões proteicas do PDGF-A, PDGF-C e PDGFRα foram significativamente maiores nos grupos DOCA e DOCA+IMA que no grupo CON, mas a expressão proteica do p-PDGFRα no grupo DOCA+IMA foi menor que no DOCA. CONCLUSÃO: O Imatinib pode exercer efeitos inibitórios sobre a fibrose miocárdica em ratos com hipertensão induzida por DOCA/sal, os quais podem ser atribuídos à inibição da atividade do PDGFR-α.

BACKGROUND: Imatinib is a tyrosine kinase receptor inhibitor that has been confirmed to exert inhibitory effect on the platelet derived growth factor PDGF receptor (PDGFRα and PDGFRβ) activity. OBJECTIVE: To investigate the protective effect of imatinib on the myocardial fibrosis in deoxycorticosterone-acetate (DOCA)/salt induced hypertensive rats. METHODS: Sixty male uninephrectomized Sprague-Dawley rats were assigned to three groups: control rats (CON group); deoxycorticosterone group (DOCA group); deoxycorticosterone and imatinib group (DOCA+IMA group). Systolic blood pressure (SBP) was measured biweekly. The apical portion of the left ventricle was studied. Sirius-Red staining, Hematoxylin-Eosin staining, immunohistochemistry and Western blot assay were employed. RESULTS: SBP in the DOCA group and DOCA+IMA group was higher than that in the CON group on day 14 and 28. Animals in the DOCA group showed severe interstitial and perivascular fibrosis on day 28, and the expressions of PI, PIII, tenascin-C and fibronectin were significantly higher than those in the DOCA+IMA group and CON group. When compared with the CON group, myocardial tissue inflammatory response and monocyte/macrophage infiltration of different degrees were observed in the DOCA group and DOCA+IMA group. Protein expressions of PDGF-A, PDGF-C and PDGFRα were signiflcantly higher in the DOCA and DOCA+IMA groups than those in the CON group, but the p-PDGFRα protein expression in the DOCA+IMA group was lower than that in the DOCA group. CONCLUSION: Imatinib can exert inhibitory effects on myocardial fibrosis in DOCA/salt induced hypertensive rats, which may be attributed to the inhibition of PDGFR-α activity.

Reversibility of cardiac fibrosis in mice chronically infected with Trypanosoma cruzi, under specific chemotherapy

This investigation was performed to verify the effect of specific chemotherapy (Benznidazole or MK-346) on the inflammatory and fibrotic cardiac alterations in mice chronically infected with the strains 21 SF (Type II) and Colombian (Type III) of Trypanosoma cruzi. To obtain chronically infected mice, two groups of 100 Swiss mice each, were infected with either the 21 SF or the Colombian strain (2x 10***4 and 5x 10***4 blood forms respectively). The rate of morality in the acute phase was of 80% for both groups. Twenty surviving mice chronically infected with the 21 SF strain and 20 with the Colombian strain were then divided in treated and untreated groups. Excluding those that died during the course of treatment, 14 mice chronically infected with the 21 SF strain and 15 with the Colombian strain were evaluated in the present study. Chemotherapy was performed with Benznidazole (N-benzil-2-nitro-1-imidazolacetamide) in the dose of 100mg/k.b.w/day, for 60 days, or with the MK-436(3(1-methyl-5 nitroimidazol-2-yl) in two daily doses of 250 mg/k.b.w, for 20 days. Parasitological cure tests were performed (xenodiagnosis, haemoculture, subinovulation of the blood into newborn mice), and serological indirect immunofluorescence test. The treated and untreated mice as well as intact controls were killed at different periods after treatment and the heart were submitted to histopathological study with hematoxilineosin and picrosirius staining; ultrastructural study; collagen immunotyping, fibronectin and laminin identification by immunofluorescence tests. Results: the untreated controls either infected with 21 SF or Colombian strain, showed inflammatory and fibrotic alterations that were mild to moderate with the 21 SF strain and intense with the Colombian strain. Redpicrosirius staining showed bundles of collagen in the interstitial space and around cardiac fibers. Increased deposits of mitritial components and collagen fibers, macrophages and fibroblasts appeared at the ultrastructural examination. Deposits of fibronectin, laminin, pro-III and IV collagens were seen, most intense in those infected with the Colombian strain. Treated mice, parasitologically cured, presented clear-cut regression of the inflamatory lesions and of the interstitial matrix thickening. Mice infected with the Colombian strain and treated with MK-436, was parasitologically cured in 5/6 cases and showed mild inflammatory infiltration and fibrosis. The mice treated with Benznidazole (Colombia