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.

Registro OBELISCO-SAC. OBsErvaciones de cLinicas, Instituciones y Servicios de Cardio-Oncología SAC / OBELISCO-SAC Registry. OBsErvations from cLinics, InstitutionS and Cardio-Oncology services SAC

RESUMEN Introducción: La cardio-oncología (CO) es una nueva disciplina, que genera nuevas áreas de trabajo en las instituciones. Desconocemos cuántos equipos de CO existen en nuestro país, su estructura y el manejo de los pacientes. Objetivos: Nuestro objetivo primario es reportar cuántos centros de CO existen en nuestro país, y de ellos cuántos trabajan de acuerdo con las recomendaciones de guías y consensos. Secundariamente, definir la especialidad y formación de los médicos integrantes, si se realiza evaluación de riesgo previo al inicio del tratamiento oncológico, cómo se evalúa la función ventricular y cómo se utilizan los biomarcadores. Material y métodos: Registro nacional, multicéntrico, transversal, descriptivo, y prospectivo que incluyó 51 instituciones generales, de oncología y/o de cardiología que referían poseer grupos de trabajo o servicios de CO. Resultados: De los 51 centros, pertenecen al ámbito público el 47,1% y al privado el 52,9%. El 49% se halla en la Ciudad Autónoma de Buenos Aires, el 17,6% en la Provincia de Buenos Aires y el resto en otros lugares del país. Sobre 47 centros, el 48,9% considera funcionar de acuerdo con las recomendaciones de Guías Internacionales y el Consenso de la Sociedad Argentina de Cardiología. El 27,7% de los centros realiza siempre estratificación de riesgo cardio-oncológico o cardiovascular antes de iniciar el tratamiento. Un 35,3% de los centros deriva siempre a cardiología a los pacientes que iniciarán un tratamiento potencialmente cardiotóxico, un 47,1% ocasionalmente. El 43,1% de los centros realiza ecocardiografía basal en todos los pacientes, el 56,9% solo en algunos. Durante el seguimiento el ecocardiograma se indica de acuerdo con el esquema utilizado en el 64,7% y en el resto según su evolución. Todos los centros evalúan la fracción de eyección ventricular izquierda mediante ecocardiografía, en el 68,1% bidimensional. El 63,8% utiliza el análisis de la deformación longitudinal sistólica global. El 47,1% deriva algunos pacientes a resonancia cardíaca y el 35,3% a tomografía cardíaca. Solo el 7,8% utiliza biomarcadores. El 5,9% indica siempre prevención primaria con antagonistas neurohormonales. El dexrazoxano es utilizado en el 5,9%, la antraciclina liposomal en el 74,5%. Frente a la aparición de cardiotoxicidad, el 76,5% inicia tratamiento cardioprotector. El 41% suspende la quimioterapia, el 47% la modifica. Conclusiones: este es el primer registro nacional de CO. Brinda información y un panorama actual del estado de esta subespecialidad en nuestro país. Casi la mitad de los centros consideró funcionar de acuerdo con Guías y Consensos. Solo un tercio de los pacientes que van a iniciar tratamiento oncológico potencialmente cardiotóxico son derivados a CO. El método más utilizado en nuestro país para evaluar la función ventricular es el ecocardiograma bidimensional, los biomarcadores son poco utilizados.

ABSTRACT Background: Cardio-oncology (CO) is a new discipline that generates new work areas within the institutions. We ignore how many CO teams exist in our country, their structure and how patients are managed. Objectives: Our primary objective is to report how many CO centers exist in our country, and how many of them work according to the recommendations of guidelines and consensus statements. We also want to define the specialty and specific training of the physicians involved, determine if they perform risk assessment before cancer treatment, establish the method used to assess ventricular function and how biomarkers are used. Methods: The OBELISCO registry is a national, multicenter, cross-sectional, descriptive and prospective registry including 51 general hospitals, cancer centers and institutions specialized in cardiology with CO work groups or services. Results: Of the 51 centers, 47.1% were public hospitals and 52.9% were private centers. Most centers were in the Autonomous City of Buenos Aires (49%) and in the Province of Buenos Aires and the rest were distributed throughout the country. Of 47 centers, 48.9% considered that their institution had CO services complying with the recommendations of international guidelines and of the consensus statement of the Argentine Society of Cardiology. Global cardio-oncological or cardiovascular risk assessment is always performed in 27.7% of the centers before starting treatment. Patients who will start potentially cardiotoxic treatment are always referred to cardiology in 35.3% of the centers and are sometimes referred to cardiology in 47.1%. Baseline echocardiography is performed in all the patients before starting treatment in 43.1% of the centers and only in some patients in 56.9%. During follow-up, echocardiography is indicated according to the treatment schedule used in 64.7% and according to the patients' outcome in the rest of the centers. All the centers evaluate left ventricular ejection fraction with echocardiography, and 68.1% use twodimensional echocardiography. Global longitudinal systolic strain is used in 63.8% of the centers. Only 47.1% order cardiac magnetic resonance imaging in some patients, and 35.3% indicate cardiac computed tomography scan. Biomarkers are used in only 7.8% of the centers. Primary prevention with neurohormonal antagonist drugs is always indicated in 5.9% of the centers. Dexrazoxane is used in only 5.9% and liposomal anthracycline in 74.5% If cardiotoxicity develops, 76.5% indicate cardioprotection, 41% discontinue chemotheraphy and 47% modify cancer treatment. Conclusions: This is the first national CO registry. It provides information and a current outlook of the status of this subspecialty in our country. Almost 50% of the centers considered to be functioning in line with guidelines and consensus statements. Only one third of the patients who will initiate cancer treatment with potentially cardiotoxic drugs are referred to CO. Two-dimensional echocardiography is the method most used in our country to evaluate ventricular function; biomarkers are scarcely used.

Mechanical dispersion in Fabry disease assessed with speckle tracking echocardiography.

BACKGROUND: Fabry disease (FD) is a rare X-linked storage disorder caused by deficiency of the lysosomal enzyme α-galactosidase A, and it typically causes multiorgan dysfunction. The main cause of death is heart disease resulting from left ventricular (LV) diastolic dysfunction, LV systolic dysfunction, severe LV hypertrophy (LVH), and sudden death. In several cardiac disorders, LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD). MD has until now not been studied in patients with FD. OBJECTIVE: To investigate the prevalence of MD in patients with FD. METHODS: Complete echocardiographic data and speckle tracking echocardiographic data were collected. MD is an index of inter-segmental discoordination of contraction and is defined as the standard deviation (SD) of the time-to-peak longitudinal negative strain in 17 LV segments with a value >49 milliseconds. Patients with FD were divided into the following 2 groups: group I (patients with FD but no LVH, n = 64) and group II (patients with FD and LVH, n = 25). These groups were compared with a group of healthy subjects (group III, n = 50). Parametric variables were expressed as mean ± SD, and nonparametric variables were expressed as median and inter-quartile range. A P value <.05 was considered significant. RESULTS: A total of 113 patients with FD were included in this study. Of these, 24 (21%) were excluded because of poor imaging quality or presence of comorbidities, and the final study population consisted of 89 patients (mean age of 33.5 ± 14.5 years, 64% female). Group II patients were older than group I patients (46 ± 13 years vs 27 ± 11 years, P < .0001). There was no difference in LV ejection fraction between the 3 groups. There was also no difference in MD between groups I and III (32.4 ms [26-39] vs 32 ms [26-39]). In group II, the MD in 19 patients (76%) was 56 ms (39-80). CONCLUSIONS: To the best of our knowledge, this is the first study to assess the prevalence of MD in patients with FD. MD was observed in 76% of patients with FD and LVH. The use of MD in strain echocardiography may be beneficial in the assessment of patients with FD who develop heart failure.

Comparison of left atrial size and function in hypertrophic cardiomyopathy and in Fabry disease with left ventricular hypertrophy.

BACKGROUND: Fabry disease (FD) and hypertrophic cardiomyopathy (HCM) are two diseases with a different pathophysiology, both cause left ventricular hypertrophy (LVH) and myocardial fibrosis. Although remodeling and systolic dysfunction of the left atrium (LA) are associated with atrial fibrillation and stroke in HCM, changes in the size and function of the LA have not been well studied in FD with LVH. METHODS: The following groups were studied prospectively, and their respective findings compared: 19 patients with non-obstructive HCM (Group I), 20 patients with a diagnosis of Fabry cardiomyopathy (Group II), and 20 normal subjects matched for sex and age (Group III). Left ventricular mass index was measured using Devereux' formula, left atrial volume with Simpson's biplane method and left atrial mechanical function, including strain and strain rate, was measured using the speckle tracking technique. Strain and strain rate of the reservoir were measured during the three phases: reservoir (SR S), passive conduit (SR E) and atrial contraction (SR A). RESULTS: Patients with HCM had a larger left atrial volume than patients with FD (48.16 ± 14.3 mL/m2 vs 38.9 ± 14.9 mL/m2 respectively, P < .001), but in both disorders there was a severe decrease in left atrial function: reservoir strain in the apical four-chamber view: 17.47% in HCM vs 22.5% in FD, P = .24), strain rate in the apical chamber view: SR A: -0.80/seconds in HCM vs -1.04/seconds in FD (P = .88), SR S: 0.69/seconds in HCM vs 0.93 in FD (P = .12), SR E: -0.80 seconds in HCM vs -0.97/seconds in FD (P = .18). CONCLUSIONS: In this echocardiographic study we used speckle tracking to assess left atrial mechanical function and showed that FD is associated to an atrial cardiomyopathy, affecting the three phasic functions of the LA. Although in patients with HCM left atrial volume is larger than in patients with FD, both disorders exhibit severe decrease in left atrial function. These findings should be considered, given the potentially serious complications that can occur with the two diseases.

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.

Presentación clínica y características ecocardiográficas en pacientes con miocardio no compacto / Clinical Presentation and Echocardiographic Characteristics of Patients with Left Ventricular Noncompaction

El miocardio no compacto es una miocardiopatía genética primaria ocasionada por la detención de la embriogénesis normal del endocardio y el miocardio. Esta anomalía se asocia frecuentemente con arritmias, insuficiencia cardíaca y eventos embólicos. El presente estudio se llevó a cabo con el objetivo de describir la modalidad de presentación clínica y las características electrocardiográficas y ecocardiográficas en portadores de esta miocardiopatía. Se analizaron 22 pacientes con diagnóstico de miocardio no compacto detectados en el laboratorio de ecocardiografía entre julio de 2004 y abril de 2009. Toda la población en estudio fue analizada mediante electrocardiograma y eco-Doppler cardíaco transtorácico y en 12 casos se registró Holter de 24 horas. Se determinaron el peso, la altura y el índice de masa corporal, que se compararon contra 66 pacientes distribuidos en tres grupos de 22 pacientes cada uno: 1) grupo control de personas sin cardiopatías, 2) portadores de miocardiopatía hipertrófica y 3) pacientes con miocardiopatía dilatada idiopática. En la población estudiada con miocardio no compacto predominó el sexo femenino y se observó menor peso e índice de masa corporal en relación con los grupos comparados y el síntoma preponderante fue la disnea. El electrocardiograma mostró ritmo sinusal con trastornos de conducción y arritmia ventricular repetitiva. El eco transtorácico mostró diferentes grados de disfunción ventricular izquierda sistólica y diastólica con áreas de miocardio no compacto predominantes en los territorios medial y apical de los segmentos inferior, posterior y lateral, en algunos casos complicados con trombos intraventriculares y ocasionalmente asociados con fístulas coronarias.

Left ventricular noncompaction is a primary genetic cardiom-yopathy caused by arrest of normal embryogenesis of the endocardium and myocardium. This anomaly is frequently associated with arrhythmias, heart failure and thromboem-bolic events. The goal of the present study was to describe the clinical presentation and the electrocardiographic and echocardiographic characteristics of patients with this cardiomyopathy. Twenty-two patients with left ventricular noncompaction detected by echocardiography between July 2004 and April 2009 were analyzed. Electrocar di ogram and transthoracic Doppler echocardiography was performed to all the patients, and 12 patients underwent 24-hour Holter monitoring. Weight, height and body mass index were calculated and compared with 66 patients distributed in three groups of 22 patients each: 1) control group without heart disease, 2) hypertrophic cardiomyopathy; and 3) idiopathic dilated cardiomyopathy. In the population with left ventricular noncompaction, fe-male gender prevailed, lower weight and body mass index was observed and dyspnea was the most common symptom. The electrocardiogram showed sinus rhythm, conduction distur-bances and repetitive ventricular arrhythmia. Transthoracic echocardiography showed different degrees of systolic and diastolic left ventricular dysfunction with areas of noncom-paction in the mid and apical inferior, posterior and lateral segments; intraventricular thrombi were occasionally seen and sometimes associated with coronary artery fistulas.

Complete regression of myocardial involvement associated with lymphoma following chemotherapy.

Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We describe the case of a 26 year old man who had initially been diagnosed with myocardial infiltration on an echocardiogram, presenting with a testicular mass and unilateral peripheral facial paralysis. On admission, electrocardiograms (ECG) revealed negative T-waves in all leads and ST-segment elevation in the inferior leads. On two-dimensional echocardiography, there was infiltration of the pericardium with mild effusion, infiltrative thickening of the aortic walls, both atria and the interatrial septum and a mildly depressed systolic function of both ventricles. An axillary biopsy was performed and reported as a T-cell lymphoblastic lymphoma (T-LBL). Following the diagnosis and staging, chemotherapy was started. Twenty-two days after finishing the first cycle of chemotherapy, the ECG showed regression of T-wave changes in all leads and normalization of the ST-segment elevation in the inferior leads. A follow-up Two-dimensional echocardiography confirmed regression of the myocardial infiltration. This case report illustrates a lymphoma presenting with testicular mass, unilateral peripheral facial paralysis and myocardial involvement, and demonstrates that regression of infiltration can be achieved by intensive chemotherapy treatment. To our knowledge, there are no reported cases of T-LBL presenting as a testicular mass and unilateral peripheral facial paralysis, with complete regression of myocardial involvement.

Esophageal carcinoma and transthoracic echocardiography.

We report a rare case of a patient with esophageal carcinoma diagnosed using transthoracic echocardiography. This examination proved to be useful in the identification of a paracardiac mediastinal mass. Images of the esophageal carcinoma, of the stent in the esophagus, and the bubbles inside the stent generated with the ingestion of a carbonated beverage, have not been previously published. Therefore, we believe our findings may be useful to other echocardiographers.

Two-dimensional speckle tracking echocardiography for early detection of myocardial damage in young patients with Fabry disease.

Fabry disease (FD) is characterized by left ventricular hypertrophy (LVH). Conventional echocardiography is not sensitive enough to perform the preclinical diagnosis To assess whether longitudinal myocardial strain of the left ventricle (LV), using speckle tracking, is useful to detect early myocardial involvement in FD. Forty-four patients with FD who were diagnosed with genetic testing were prospectively included and were compared to a sex-matched control group. They were divided into three groups: 22 with LVH (Group I), 22 without LVH (Group II), and 22 healthy volunteers (Group III). LV longitudinal strain was measured from the apical views. An ANOVA test was used for multiple comparisons for variables with a normal distribution, and a Kruskal-Wallis test was used for variables with non-Gaussian distribution. Longitudinal LV strain was different in the three groups: it was ≥-15% in at least one segment in all Group I patients, in 50% of patients of Group II and in no patient of Group III. Seventy percent of the segments with abnormal strain in Group II were located in the basal regions (32/46). These findings show that the presence of at least one strain value ≥-15% demonstrates subclinical myocardial dysfunction in patients with preclinical FD. Longitudinal myocardial LV strain measured with speckle tracking is a useful tool to detect early myocardial involvement in young patients with FD. This information allows the detection and treatment of myocardial dysfunction at an early stage, which is of high clinical importance.

Left ventricular thrombus mimicking primary cardiac tumor in a patient with primary antiphospholipid syndrome and recurrent systemic embolism.

Primary antiphospholipid syndrome (APS) is a well-defined entity characterized by spontaneous and recurrent abortion, thrombocytopenia and recurrent vascular thromboses (arterial and venous). Left ventricular thrombus mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. In this report we describe a 39 year-old man admitted to hospital presenting with left hemiparesis and a peripheral embolism. He had no history of thrombotic events. Transthoracic echocardiography showed a large, polypoid and mobile mass (4.0 x 1.2 cm) attached to the apex of the left ventricle, highly suggestive of primary cardiac tumor. The patient subsequently underwent open heart surgery. The histological examination showed an older thrombus and a fresh thrombus. Post-operative laboratory tests showed lupus anticoagulant activity, confirming the primary APS diagnosis. The patient initiated treatment with oral anticoagulation (INR levels between 2 and 3) and was discharged 29 days after surgery. At ten month follow-up, he was symptom-free with long-term anticoagulation therapy. No evidence of intracardiac mass recurrence on two-dimensional echocardiography was seen. Intracardiac thrombus has been rarely reported as a complication of primary APS. Left ventricular mass mimicking primary cardiac tumor with recurrent systemic embolism has not been previously reported. Pre-operative investigations could not distinguish such a thrombus from a cardiac tumor and the diagnosis was made post-operatively.

Valve replacement with bileaflet HP-Biplus mechanical prostheses versus standard prostheses: long-term results.

BACKGROUND AND AIM OF THE STUDY: At present, no reports are available regarding the hemodynamic assessment and survival of patients undergoing valve replacement with HP-Biplus prostheses. Hence, the present study was designed to acquire this information. The aim was to assess: (i) any potential hemodynamic differences between the HP-Biplus prosthesis and the 'standard' bileaflet mechanical valves (SJM, ATS, Sorin Bicarbon and CarboMedics); and (ii) the incidence of postoperative complications and long-term mortality. METHODS: The data from 242 patients (139 males, 103 females; mean age 58.4 +/- 14.0 years) who survived mitral or aortic valve surgery with mechanical bileaflet standard prostheses (SJM, ATS, Sorin Bicarbon or CarboMedics), between January 1985 and December 2005, were analyzed retrospectively. Evaluations were conducted consecutively with Doppler echocardiography, and compared with 35 patients (20 males, 15 females; mean age 52.2 +/- 12.8 years) who received an HP-Biplus prosthesis and underwent surgery between January 2000 and December 2005. RESULTS: At seven years after surgery, actuarial survival was 40% for the HP-Biplus prosthesis and 84% for the 'standard' prostheses (p < 0.0001). The HP-Biplus prostheses had a higher rate of reoperations for aortic valves (15.2% versus 1.7%; p < 0.003, OR 10.2), a higher rate of prosthesis dysfunction (62.9% versus 7.8%; p < 0.00001, OR 25), and a higher rate of total events (72% versus 21.8%; p < 0.0001, OR 11). CONCLUSION: The present study was the first to compare long-term results of the HP-Biplus prostheses with those obtained with 'standard' mechanical prostheses. Valve replacement with standard prostheses was shown to carry low morbidity and mortality (21.8%), whereas the HP-Biplus prosthesis showed high morbidity and mortality (92%). The data acquired may be very important when selecting prostheses with better hemodynamic characteristics, and show that the HP-Biplus prosthesis, in the authors' opinion, is not suitable for clinical use.

Retrieval of a leaflet escaped in a Tri-technologies bileaflet mechanical prosthetic valve.

The escape of the prosthetic heart valve disc is one of the causes of prosthetic dysfunction that requires emergency surgery. The removal of the embolized disc should be carried out because of the risk of a progressive extrusion on the aortic wall. Several imaging techniques can be used for the detection of the missing disc localization. In this report we describe a 32-year-old man who underwent mitral valve replacement with a Tri-technologies bileaflet valve three years ago, and was admitted in cardiogenic shock. Transesophageal echocardiography showed acute-onset massive mitral regurgitation. The patient underwent emergency replacement of the prosthetic valve. Only one of the two leaflets remained in the removed prosthetic valve. The missing leaflet could not be found within the cardiac cavity. The abdominal fluoroscopic study and plain radiography were unable to detect the escaped leaflet. The abdominal computed tomography scan and the ultrasound showed the escaped leaflet in the terminal portion of the aortic bifurcation. To retrieve the embolized disc laparotomy and aortotomy were performed three months later. The escaped leaflet shows a fracture of one of the pivot systems caused by structural failure. This kind of failure mode is usually the result of high stress concentration.