Sarcoma sinovial primario de pericardio / Primary pericardial synovial sarcoma

Resumen El sarcoma sinovial primario del pericardio es un tumor muy raro y de mal pronóstico y se sabe poco en cuanto al manejo terapéutico. Presentamos el caso de una paciente de 51 años a quien se le realizó resección quirúrgica incompleta, quimioterapia y radioterapia. Hasta donde sabemos, este es el primer caso de un sarcoma sinovial primario de pericardio que luego de operado se mantuvo asintomático durante 5 años hasta que en una TAC de control se le detectaron metástasis cardiacas que comprometían las cavidades derechas y con quimioterapia, la ecocardiografía demostró la reso lución completa de las mismas.

Abstract Primary pericardial synovial sarcoma is an extraor dinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no pri mary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.

[Primary pericardial synovial sarcoma]. / Sarcoma sinovial primario de pericardio.

Primary pericardial synovial sarcoma is an extraordinarily very rare tumor with a poor prognosis, and little is known about its therapeutic management. We describe the case of a 51-year-old woman patient who underwent incomplete surgical resection, chemotherapy, and radiotherapy. To the best of our knowledge, no primary pericardial synovial sarcoma has been described which, after surgery, remains asymptomatic for 5 years, and until a control CT scan detects cardiac metastases that compromised the lumen of the right cavities and with chemotherapy, echocardiography demonstrated complete resolution of cardiac metastases.

El sarcoma sinovial primario del pericardio es un tumor muy raro y de mal pronóstico y se sabe poco en cuanto al manejo terapéutico. Presentamos el caso de una paciente de 51 años a quien se le realizó resección quirúrgica incompleta, quimioterapia y radioterapia. Hasta donde sabemos, este es el primer caso de un sarcoma sinovial primario de pericardio que luego de operado se mantuvo asintomático durante 5 años hasta que en una TAC de control se le detectaron metástasis cardiacas que comprometían las cavidades derechas y con quimioterapia, la ecocardiografía demostró la resolución completa de las mismas.

[Pulmonary embolism. Clinical suspicion and anatomopathological correlation]. / Tromboembolismo de pulmón. Sospecha clínica y correlación anatomopatológica.

Discrepancies between clinical suspicion and pathological findings in pulmonary embolism (PE) appear to be frequent. The aim of this study was to analyze the prevalence of PE in a necropsy series of patients who have died in an acute care hospital between 1998-2017, its relationship with previous clinical suspicion, and its importance as a cause of death. It is a retrospective observational study of 350 autopsies done at the Department of Pathology. We analyzed the demographic characteristics, main clinical diagnoses stated in the autopsy request form, incidence of PE diagnosed, main autopsy findings related with the cause of death, as well as the concordance between clinical suspicion and autopsy diagnosis. In only 8% of the cases (n = 28) the clinical diagnosis of autopsy request was PE. An autopsy diagnosis of PE was done in 127 cases (36.3%); in 33 cases (25.9%) affected large pulmonary vessels; medium caliber vessels were affected in 75 cases (59.1%), and in 19 cases small vessels. The PE was considered as a contributor or cause of death in 30.9% (n = 108). However, only 15.7% of the confirmed PE cases had previous clinical suspicion. This series of necropsies shows that PE is a high prevalence finding in autopsies at an acute care hospital, and an important cause of death in a 20 years period. The finding of a low concordance with clinical diagnosis should alert the medical community on the importance of clinical suspicion in order to achieve an early diagnosis and treatment of this disease.

Las discrepancias entre la sospecha clínica y los hallazgos patológicos en el tromboembolismo pulmonar (TEP) son frecuentes. El objetivo de este estudio fue analizar la prevalencia de TEP en una serie de necropsias de fallecidos en un hospital general de agudos entre 1998 y 2017, su relación con la sospecha clínica y su importancia como causa de muerte. Es un estudio retrospectivo y observacional de 350 autopsias realizadas en el Servicio de Patología; analiza características demográficas, principales diagnósticos clínicos informados en la solicitud de autopsia (sospecha clínica de muerte), incidencia anatomopatológica de TEP, diagnósticos primarios de autopsia relacionados con la muerte y concordancia entre sospecha clínica y diagnósticos de autopsia. En solo el 8% de las autopsias (n = 28), el TEP fue el diagnóstico clínico informado. En las autopsias, se encontró TEP en 127 casos (36.3%). Afectó arterias pulmonares grandes en 33 casos (25.9%), vasos medianos en 75 (59.1%) y vasos pequeños en 19 casos. El TEP se consideró el principal contribuyente o la causa principal de muerte en un 30.9% (n = 108) de los casos. Sin embargo, solo en el 15.7% de los casos confirmados había una sospecha clínica previa de TEP. Esta serie muestra que el TEP es un hallazgo de alta prevalencia y una causa importante de muerte en autopsias realizadas en un hospital de agudos. La evidencia de la baja concordancia entre los diagnósticos anatomopatológicos y clínicos del TEP alerta sobre la importancia de la sospecha clínica para lograr un tratamiento temprano de la enfermedad.

Tromboembolismo de pulmón: Sospecha clínica y correlación anatomopatológica / Pulmonary embolism. Clinical suspicion and anatomopathological correlation

Las discrepancias entre la sospecha clínica y los hallazgos patológicos en el tromboembolismo pulmonar (TEP) son frecuentes. El objetivo de este estudio fue analizar la prevalencia de TEP en una serie de necropsias de fallecidos en un hospital general de agudos entre 1998 y 2017, su relación con la sospecha clínica y su importancia como causa de muerte. Es un estudio retrospectivo y observacional de 350 autopsias realizadas en el Servicio de Patología; analiza características demográficas, principales diagnósticos clínicos informados en la solicitud de autopsia (sospecha clínica de muerte), incidencia anatomopatológica de TEP, diagnósticos primarios de autopsia relacionados con la muerte y concordancia entre sospecha clínica y diagnósticos de autopsia. En solo el 8% de las autopsias (n = 28), el TEP fue el diagnóstico clínico informado. En las autopsias, se encontró TEP en 127 casos (36.3%). Afectó arterias pulmonares grandes en 33 casos (25.9%), vasos medianos en 75 (59.1%) y vasos pequeños en 19 casos. El TEP se consideró el principal contribuyente o la causa principal de muerte en un 30.9% (n = 108) de los casos. Sin embargo, solo en el 15.7% de los casos confirmados había una sospecha clínica previa de TEP. Esta serie muestra que el TEP es un hallazgo de alta prevalencia y una causa importante de muerte en autopsias realizadas en un hospital de agudos. La evidencia de la baja concordancia entre los diagnósticos anatomopatológicos y clínicos del TEP alerta sobre la importancia de la sospecha clínica para lograr un tratamiento temprano de la enfermedad.

Discrepancies between clinical suspicion and pathological findings in pulmonary embolism (PE) appear to be frequent. The aim of this study was to analyze the prevalence of PE in a necropsy series of patients who have died in an acute care hospital between 1998-2017, its relationship with previous clinical suspicion, and its importance as a cause of death. It is a retrospective observational study of 350 autopsies done at the Department of Pathology. We analyzed the demographic characteristics, main clinical diagnoses stated in the autopsy request form, incidence of PE diagnosed, main autopsy findings related with the cause of death, as well as the concordance between clinical suspicion and autopsy diagnosis. In only 8% of the cases (n = 28) the clinical diagnosis of autopsy request was PE. An autopsy diagnosis of PE was done in 127 cases (36.3%); in 33 cases (25.9%) affected large pulmonary vessels; medium caliber vessels were affected in 75 cases (59.1%), and in 19 cases small vessels. The PE was considered as a contributor or cause of death in 30.9% (n = 108). However, only 15.7% of the confirmed PE cases had previous clinical suspicion. This series of necropsies shows that PE is a high prevalence finding in autopsies at an acute care hospital, and an important cause of death in a 20 years period. The finding of a low concordance with clinical diagnosis should alert the medical community on the importance of clinical suspicion in order to achieve an early diagnosis and treatment of this disease.

[Simple mechanical reperfusion in acute myocardial infarction: determinants, prognostic implications and relation with thrombus histology]. / Reperfusión mecánica simple en el infarto agudo de miocardio. Determinantes, implicaciones en el pronóstico y relación con la histología del trombo.

Simple mechanical reperfusion (SMR) is defined as reperfusion achieved after wire insertion at the occluded infarct-related artery in primary angioplasty. The determinants and prognostic implications of SMR and its relationship with the histopathology of the rescued thrombus were evaluated in 601 patients with acute myocardial infarction showing ST elevation and pre-procedural total occlusion of the infarct-related artery (TIMI flow 0). Two groups were considered according to the presence of SMR, defined as the visualization of contrast material after crossing the occlusion with the guide wire. SMR was achieved in 303 patients (50.4%) and was found to be associated with less time to treatment (248 vs. 286 minutes; p = 0.001), less deteriorated initial left ventricular function and shock at admission (9.2 vs. 16.4%; p = 0.008), higher successful rate (94.7% vs. 78.5%; p < 0,0001) and of higher rate of thrombus rescue: 70/81 vs. 27/79 patients (p < 0.0001). The right coronary artery was the most frequent infarct-related artery. Histopathology of the retrieved thrombi was available for 160 patients treated with thrombus aspiration. SMR was associated with smaller thrombus, lower contents of leukocytes and erythrocytes, and higher thrombus content of inflammatory cells, cholesterol and collagen crystals from the atheromatous plaque. SMR is an independent predictor of procedure success and its relationship with the anatomy of the thrombus could redefine the indication of thrombus aspiration.

Reperfusión mecánica simple en el infarto agudo de miocardio: Determinantes, implicaciones en el pronóstico y relación con la histología del trombo / Simple mechanical reperfusion in acute myocardial infarction: determinants, prognostic implications and relation with thrombus histology

La reperfusión mecánica simple (RMS) es la lograda en la angioplastia primaria al atravesar la oclusión en la arteria responsable de infarto con la guía intracoronaria. Para evaluar los determinantes de la RMS, su implicación en el pronóstico y su relación con la histopatología del trombo rescatado, fueron analizados los casos de 601 pacientes con infarto agudo de miocardio con elevación del segmento ST y oclusión total de la arteria responsable de infarto (flujo TIMI 0). Se consideraron dos grupos según la presencia de RMS, definida por la visualización de contraste distal a la lesión luego de atravesarla con la guía intracoronaria. Se realizó el análisis histopatológico de los trombos en un subgrupo de 160 pacientes tratados con tromboaspiración. En 303 (50.4%) pacientes se obtuvo RMS. La RMS se asoció a menor ventana al tratamiento (248 vs. 286 minutos, p = 0.001), menor deterioro de la función sistólica ventricular izquierda inicial y shock al ingreso (9.2 vs.16.4%, p = 0.008), mayor tasa de éxito (94.7 vs. 78.5%, p < 0.0001) y de rescate de trombos (70/81 vs. 27/79 pacientes, p < 0.0001). La arteria responsable de infarto más frecuente fue la coronaria derecha. La RMS se asoció con menor tamaño del trombo, menor concentración de leucocitos y eritrocitos y mayor contenido de células inflamatorias, cristales de colesterol y colágeno provenientes de la placa. La RMS es un predictor independiente de éxito y su relación con la anatomía del trombo podría redefinir la indicación de la tromboaspiración.

Simple mechanical reperfusion (SMR) is defined as reperfusion achieved after wire insertion at the occluded infarct-related artery in primary angioplasty. The determinants and prognostic implications of SMR and its relationship with the histopathology of the rescued thrombus were evaluated in 601 patients with acute myocardial infarction showing ST elevation and pre-procedural total occlusion of the infarct-related artery (TIMI flow 0). Two groups were considered according to the presence of SMR, defined as the visualization of contrast material after crossing the occlusion with the guide wire. SMR was achieved in 303 patients (50.4%) and was found to be associated with less time to treatment (248 vs. 286 minutes; p = 0.001), less deteriorated initial left ventricular function and shock at admission (9.2 vs. 16.4%; p = 0.008), higher successful rate (94.7% vs. 78.5%; p < 0,0001) and of higher rate of thrombus rescue: 70/81 vs. 27/79 patients (p < 0.0001). The right coronary artery was the most frequent infarct-related artery. Histopathology of the retrieved thrombi was available for 160 patients treated with thrombus aspiration. SMR was associated with smaller thrombus, lower contents of leukocytes and erythrocytes, and higher thrombus content of inflammatory cells, cholesterol and collagen crystals from the atheromatous plaque. SMR is an independent predictor of procedure success and its relationship with the anatomy of the thrombus could redefine the indication of thrombus aspiration.

Limitations of multimodality imaging in the diagnosis of pannus formation in prosthetic aortic valve and review of the literature.

Pannus formation is a rare complication and occurs almost exclusively in mechanical prosthetic valves. It consists of fibrous tissue that covers the surface of the prosthesis either concentrically or eccentrically, resulting in valve dysfunction. The pathophysiology seems to be associated to a chronic inflammatory process that explains the late and insidious clinical presentation. This diagnosis should be considered in patients with high transvalvular gradients on transthoracic echo, and workup should be completed with fluoroscopy and transesophageal echocardiography. Treatment is always surgical and recurrence is rare. We present a case of pannus formation in a prosthetic aortic valve and a review of the literature regarding this disorder.

Severe mitral regurgitation after radiotherapy.

We present the case of a 69-year-old patient with a history of gynecological neoplasia and a pulmonary metastasis, who in 1996 underwent chemotherapy and mediastinal radiotherapy followed by cancer remission. Ten years later she presented with heart failure and her Doppler echocardiogram showed severe mitral regurgitation with pulmonary hypertension. In 2011, she underwent a mitral valve replacement with a biological prosthesis and the pathology exam revealed valve damage consistent with radiotherapy-induced changes. This unusual mechanism of mitral regurgitation can be demonstrated clearly by echocardiography and should be disseminated among cardiology physicians and in patients who have survived for long periods after radiotherapy, it is important to remember that cardiac complications may indeed occur, and the treating physician is responsible for detecting them.

Miocarditis de células gigantes que simula un infarto agudo de miocardio / Giant Cell Myocarditis Mimicking an Acute Myocardial Infarction

La miocarditis de células gigantes (MCG) es una entidad rara, de causa desconocida, de probable etiología autoinmune. Puede presentarse como insuficiencia cardíaca refractaria, asociarse con arritmias ventriculares y en otras ocasiones simular un infarto agudo de miocardio. Su pronóstico con frecuencia es ominoso, salvo que se realicen tratamiento inmunosupresor o trasplante cardíaco, este último con elevada recurrencia.

The giant cell myocarditis (GCM) is a rare disease, of unknown origin, and probably of autoimmune etiology. It can occur as refractory heart failure, associate with ventricular arrhythmias, and mimicking an acute myocardial infarction in certain cases. Its prognosis is usually ominous, unless immunosupressive therapy or cardiac transplant is performed, the latter with a high recurrence.