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.

[Acute myocardial infarction in a young woman with systemic lupus erythematosus]. / Infarto agudo de miocardio en mujer joven con diagnóstico de lupus eritematoso sistémico.

We repor a case of acute ST elevation myocardial infarction in a 22-year-old patient with SLE, hypertension and nephropathy who underwent successful coronary angioplasty to a middle third of the left anterior descending artery. She evolved without signs of heart failure however, due to the delay in diagnosis, she presented severe deterioration of ventricular function. ST segment elevation myocardial infarction is a very rare event in young premenopausal women, but compared to the general population, patients with lupus have at least a 50% higher risk of suffering it regardless their age. In this population, the most frequent causes are vasculitis, early atherosclerosis and secondary thrombosis to antiphospholipid syndrome. In the context of lupus, conditions such as the presence of nephritis have been described as favoring the appearance of myocardial infarction, constituting subgroups of higher risk. The increased risk of AMI in patients with SLE must be taken into account and must be suspected as a differential diagnosis of precordial pain in young women, even those under 25 years of age, a population categorized as having low CV risk according to traditional scores. This would avoid delays in diagnosis and treatment with adverse consequences such as extensive myocardial necrosis and its impact on ventricular systolic function, as occurred in this patient.

Se presenta un caso de infarto agudo de miocardio con elevación del segmento ST en una paciente de 22 años de edad, con LES, HTA y nefropatía a la cual se le realizó angioplastia coronaria exitosa a tercio medio de arteria descendente anterior. Evolucionó sin signos de insuficiencia cardiaca, sin embargo, debido al retraso en el diagnóstico presentó deterioro grave de la función ventricular. El infarto agudo de miocardio con elevación del ST es un evento muy poco frecuente en mujeres pre menopáusicas pero, en comparación con la población general las pacientes con lupus eritematoso sistémico presentan al menos un 50% más de riesgo de padecerlo independientemente de la edad. En esta población, las etiologías más frecuentes son la vasculitis, la aterosclerosis precoz y la trombosis secundaria a síndrome antifosfolipídico. A su vez, en contexto de lupus, se han descripto condiciones como la presencia de nefritis lúpica, que favorecen aún más a la aparición del infarto de miocardio, constituyendo subgrupos de mayor riesgo. El incremento del riesgo de IAM en los pacientes con LES debe tenerse en cuenta y hay que sospechar como diagnóstico diferencial del dolor precordial aún en mujeres jóvenes, incluso menores de 25 años, población categorizada como de bajo riesgo cardiovascular según los scores y criterios tradicionales. Esto evitaría las demoras en el diagnóstico y tratamiento con consecuencias pronósticas adversas como la necrosis miocárdica extensa y su impacto negativo sobre la función sistólica ventricular como ocurrió en esta paciente.

Infarto agudo de miocardio en mujer joven con diagnóstico de lupus eritematoso sistémico / Acute myocardial infarction in a young woman with systemic lupus erythematosus

Resumen Se presenta un caso de infarto agudo de miocardio con elevación del segmento ST en una paciente de 22 años de edad, con LES, HTA y nefropatía a la cual se le realizó angioplastia coronaria exitosa a tercio medio de arteria descendente anterior. Evolucionó sin signos de insuficiencia cardiaca, sin embargo, debido al retraso en el diagnóstico presentó deterioro grave de la función ventricular. El infarto agudo de miocardio con elevación del ST es un evento muy poco frecuente en mujeres pre menopáusicas pero, en comparación con la población general las pacientes con lupus eritematoso sistémico presentan al menos un 50% más de riesgo de padecerlo independientemente de la edad. En esta población, las etiologías más frecuentes son la vasculitis, la aterosclerosis precoz y la trombosis secundaria a síndrome antifosfolipídico. A su vez, en contexto de lupus, se han descripto condiciones como la presencia de nefritis lúpica, que favorecen aún más a la aparición del infarto de miocardio, constituyendo subgrupos de mayor riesgo. El incremento del riesgo de IAM en los pacientes con LES debe tenerse en cuenta y hay que sospechar como diagnóstico diferencial del dolor precordial aún en mu jeres jóvenes, incluso menores de 25 años, población categorizada como de bajo riesgo cardiovascular según los scores y criterios tradicionales. Esto evitaría las demoras en el diagnóstico y tratamiento con consecuencias pronósticas adversas como la necrosis miocárdica extensa y su impacto negativo sobre la función sistólica ventricular como ocurrió en esta paciente.

Abstract We repor a case of acute ST elevation myocardial infarction in a 22-year-old patient with SLE, hypertension and nephropathy who underwent successful coronary angioplasty to a middle third of the left an terior descending artery. She evolved without signs of heart failure however, due to the delay in diagnosis, she presented severe deterioration of ventricular function. ST segment elevation myocardial infarction is a very rare event in young premenopausal women, but compared to the general population, patients with lupus have at least a 50% higher risk of suffering it regardless their age. In this population, the most frequent causes are vasculitis, early atherosclerosis and secondary thrombosis to antiphospholipid syndrome. In the context of lupus, conditions such as the presence of nephritis have been described as favoring the appearance of myocardial infarction, constituting subgroups of higher risk. The increased risk of AMI in patients with SLE must be taken into account and must be suspected as a differential diagnosis of precordial pain in young women, even those under 25 years of age, a population categorized as having low CV risk according to traditional scores. This would avoid delays in diagnosis and treatment with adverse consequences such as extensive myocardial necrosis and its impact on ventricular systolic function, as occurred in this patient.

Seguimiento de graduados de la Carrera de Médico Especialista en cardiología de la Universidad de Buenos Aires. Experiencia del Hospital Cosme Argerich / Follow-up of Specialist in Cardiology Graduates at University of Buenos Aires. Cosme Argerich Hospital Experience

RESUMEN El seguimiento de los graduados en una carrera es un indicador de la calidad educativa de la misma. Su labor profesional y académica permite evaluar los resultados de los programas instituidos a largo plazo. Objetivo: Explorar la percepción de los egresados de la Carrera de Médico Especialista (CME) en cardiología de la Universidad de Buenos Aires (UBA) sede Hospital Argerich sobre la calidad de la formación recibida durante la carrera; conocer su inserción laboral en el mundo profesional, y la realización de subespecialidades. Material y métodos: Estudio descriptivo, de corte transversal, realizado del 1° al 31 de enero de 2020. Se diseñó una encuesta de opinión autoadministrada a través de surveymonkey.com. El universo encuestado fueron 28 egresados de 7 promociones consecutivas (año de admisión 2010-2016) de la Carrera de Médico Especialista (CME) en cardiología UBA-sede Argerich. Resultados: Del total de los egresados contestaron la encuesta 25 (89,2%). La edad promedio fue 34 años, sexo masculino 14 (56%). Se realizaron preguntas para la evaluación global, valoración de la actividad científica y académica en la Sede del Hospital, al igual que la valoración del Curso Bianual de Cardiología en la Sociedad Argentina de Cardiología. Las respuestas en su mayoría fueron favorables Los egresados continúan ejerciendo la profesión en un 100%, el 76% se perfeccionó en una subespecialidad. Conclusiones: Los egresados de la CME en cardiología sede Hospital Argerich perciben que su formación ha sido muy buena o excelente y ha contribuido a su desarrollo profesional de manera esencial. Toda la información recabada genera una fuente de información para retroalimentar y optimizar la enseñanza en la institución formadora.

ABSTRACT Background: Follow-up of graduates in a career is an indicator of educational quality, and their professional and academic work allows evaluating the results of long-term instituted programs Objective: The aim of this study was to explore the perception of graduates from the postgraduate specialist course (PSC) in cardiology at University of Buenos Aires (UBA) Hospital Argerich venue on the quality of training received, how they insert in the professional world, and their engagement in subspecialties. Methods: A descriptive, cross-sectional study was carried out from January 1 to 31, 2020. A self-administered opinion survey was designed using surveymonkey.com and submitted to 28 graduates of the PSC in cardiology UBA-Hospital Argerich, corresponding to 7 consecutive promotions (2010-2016 years of admission). Results: The survey was answered by 25 graduates (89.2%). Mean age was 34 years and 14 (56%) were men. The questions explored the global assessment, the evaluation of the scientific and academic activity at the hospital venue as well as that of the Argentine Society of Cardiology Biennial Course of Cardiology. Most of the answers were favorable. All the graduates continue practicing the profession and 76% have received advanced training in a subspecialty. Conclusions: Graduates from the PSC in cardiology at Hospital Argerich perceive that their training has been very good or excellent and has been essential for their professional development. All the data collected are a source of information to provide feedback and optimize teaching in the training institution.

[Experience in infective endocarditis over 30 years in a hospital in Buenos Aires]. / Experiencia en endocarditis infecciosa a lo largo de 30 años en un hospital de Buenos Aires.

Infective endocarditis (IE) is a disease that in recent decades has shown changes in its presentation, diagnosis and treatment. This is a prospective study of 252 patients admitted at a reference hospital in Buenos Aires, Argentina, with a diagnosis of IE and they were grouped according to the decade of admission: Group A: from January 1988 to December 1997 (89 patients -35.3%-), Group B: from January 1998 to December 2007 (88 patients -34.9%-), and Group C: from January 2008 to December 2018 (75 patients -29.8%). The characteristics were analyzed and compared: age, sex, underlying heart disease, blood cultures and germs, presence of vegetations, surgical treatment and in-hospital mortality. Over the three decades, the predisposing heart condition showed that rheumatic valve disease decreased significantly (p < 0.0001) while the IE in cardiac devices also increased significantly (p < 0.0001). The percentage of blood culture-negatives decreased significantly over the years (p < 0.0001). In-hospital mortality showed a downward trend in the last decade (p = 0.069). The development of complications during hospitalization, the indication for surgery, and the presence of heart failure on admission were independent predictors of in-hospital mortality. The presence of vegetations and febrile syndrome on admission were independent predictor for lower mortality. The comparison over the years showed important changes in the epidemiological profile of IE. Probably due to advances in diagnostic techniques, treatment, and the implementation of interdisciplinary IE teams in the last decade, in-hospital mortality shows a strong tendency to decrease.

La endocarditis infecciosa (EI) es una enfermedad que en las últimas décadas ha mostrado cambios en su presentación, diagnóstico y tratamiento. Se realizó un estudio prospectivo de 252 pacientes ingresados en un hospital de referencia en Buenos Aires, Argentina, con diagnóstico de EI, agrupados según década de ingreso: Grupo A: enero 1988 a diciembre 1997 (89 pacientes ­35.3%­), Grupo B: enero 1998 a diciembre 2007 (88 pacientes ­34.9%­), y Grupo C: enero 2008 a diciembre 2018 (75 pacientes ­29.8%). Se analizaron y compararon las características: edad, sexo, cardiopatía de base, hemocultivos y gérmenes, presencia de vegetaciones, tratamiento quirúrgico y mortalidad intrahospitalaria. Durante las tres décadas, la cardiopatía predisponente mostró que la enfermedad valvular reumática disminuyó significativamente (p < 0.0001) mientras que la EI en los dispositivos cardíacos aumentó significativamente (p < 0.0001). El porcentaje de hemocultivos negativos disminuyó significativamente a lo largo de los años (p < 0.0001). La mortalidad hospitalaria mostró una reducción en la última década (p = 0.069). El desarrollo de complicaciones durante la hospitalización, la indicación de cirugía y la presencia de insuficiencia cardíaca al ingreso fueron predictores independientes de mortalidad hospitalaria. La presencia de vegetaciones y síndrome febril al ingreso fueron predictores independientes de menor mortalidad. La comparación a través de los años mostró cambios importantes en el perfil epidemiológico de la EI. Probablemente por el avance en las técnicas diagnósticas, el tratamiento y la implementación de equipos interdisciplinarios de EI de la última década, la mortalidad intrahospitalaria marca una fuerte tendencia a la reducción.

[Infectious endocarditis due to Neisseria gonorrhoeae]. / Endocarditis infecciosa por Neisseria gonorroheae.

We report a case of infectious endocarditis due to Neisseria gonorrhoeae in a 38-year-old male patient with no cardiovascular risk factors or past medical history who presented with prolonged febrile illness, asthenia and weight loss. The blood cultures were positive for gonococcus. He received antibiotic treatment with ceftriaxone for 29 days. The patient developed severe aortic regurgitation and underwent surgical aortic valve replacement with a bileaflet mechanical prosthesis, with favorable outcome.

Se presenta un caso de endocarditis infecciosa por Neisseria gonorrhoeae, en un paciente masculino de 38 años, sin factores de riesgo cardiovascular ni otros antecedentes previos. La sospecha diagnóstica comienza por síndrome febril prolongado, astenia y pérdida de peso, confirmada con rescate de gonococo en los hemocultivos. Cumplió tratamiento antibiótico con ceftriaxona por 29 días. Evoluciona con insuficiencia aórtica grave por lo cual se realiza cirugía de reemplazo valvular por prótesis mecánica bidisco exitosa, con una evolución favorable.

Endocarditis infecciosa por Neisseria gonorroheae / Infectious endocarditis due to Neisseria gonorrhoeae

Resumen Se presenta un caso de endocarditis infecciosa por Neisseria gonorrhoeae, en un paciente masculino de 38 años, sin factores de riesgo cardiovascular ni otros antecedentes previos. La sospecha diagnóstica comienza por síndrome febril prolongado, astenia y pérdida de peso, confirmada con rescate de gonococo en los hemocultivos. Cumplió tratamiento antibiótico con ceftriaxona por 29 días. Evoluciona con insu ficiencia aórtica grave por lo cual se realiza cirugía de reemplazo valvular por prótesis mecánica bidisco exitosa, con una evolución favorable.

Abstract We report a case of infectious endocarditis due to Neisseria gonorrhoeae in a 38-year-old male patient with no cardiovascular risk factors or past medical history who presented with prolonged febrile illness, asthenia and weight loss. The blood cultures were positive for gonococcus. He received antibiotic treatment with ceftriaxone for 29 days. The patient developed severe aortic regurgitation and underwent surgical aortic valve replacement with a bileaflet mechanical prosthesis, with favorable outcome.

RACCOVID-19: primer Registro Argentino de Complicaciones Cardiovasculares en pacientes con COVID-19 / RACCOVID-19: First Argentine Registry of Cardiovascular Complications in COVID-19 Patients

RESUMEN Introducción: La enfermedad por coronavirus (COVID-19) ha causado una de las mayores pandemias conocidas al día de la fecha. La Sociedad Argentina de Cardiología (SAC) y la Federación Argentina de Cardiología (FAC) elaboraron el primer Registro Argentino de Complicaciones Cardiovasculares en pacientes con COVID-19 (RACCOVID-19), con el propósito de relevar, a nivel nacional, su impacto en la evolución hospitalaria de estos pacientes. Objetivos: Documentar la aparición de complicaciones cardiovasculares en pacientes internados por COVID-19 y evaluar predictores de riesgo de dichas complicaciones y su impacto pronóstico. Material y Métodos: Se incluyen datos de 2750 pacientes en 50 centros de 11 provincias del país, desde el 18 de mayo hasta el 31 de octubre de 2020. Resultados: La edad promedio fue de 57±18 años y hubo predominio de sexo masculino (60,2%). La tasa de complicaciones cardiovasculares fue del 15,3%. La insuficiencia cardíaca (43,5%), las arritmias (33,5%) y el daño miocárdico (31,1%) fueron las complicaciones más frecuentes. La mortalidad fue del 19,3%. Un modelo de predicción de sobrevida en la etapa hospitalaria incluyó las siguientes variables: edad, sexo masculino, valores de hematocrito y creatinina al ingreso, existencia de antecedentes patológicos, formas de presentación de COVID-19 graves y presencia de complicaciones cardiovasculares. Conclusiones: El registro RACCOVID-19 mostró una tasa de complicaciones cardiovasculares del 15,3%. La mortalidad total del registro fue del 19,3% y las complicaciones cardiovasculares junto con otras variables de presentación, así como la gravedad del cuadro clínico de COVID-19, forman parte de un perfil de riesgo clínico asociado a mayor mortalidad.

ABSTRACT Background: Coronavirus disease (COVID-19) has caused one of the largest pandemics known to date. The Argentine Society of Cardiology (SAC) and the Argentine Federation of Cardiology (FAC) have developed the First Argentine Registry of Cardiovascular Complications in COVID-19 patients (RACCOVID-19) with the purpose of performing a nationwide review of their impact in the in-hospital evolution of these patients. Objectives: The aim of this study was to record cardiovascular complications in hospitalized patients for COVID-19, and to evaluate risk predictors of these complications and their prognostic impact. Methods: A total of 2750 patients from 50 centers in 11 provinces of the country were included from May 18 to October 31, 2020. Results: Mean age was 57±18 years, with a prevalence of male gender (60.2%). Cardiovascular complications occurred in 15.3% of cases. Heart failure (43.5%), arrhythmias (33.5%) and myocardial injury (31.1%) were the most relevant complications. Mortality was 19.3%, and a predictive model of in-hospital survival included age, male gender, admission hematocrit and creatinine, history of previous diseases, severe forms of COVID-19 presentation and cardiovascular complications. Conclusions: The RACCOVID-19 registry showed 15.3% of cardiovascular complications. Overall mortality was 19.3% and cardiovascular complications together with other presentation variables as well as the clinical severity of COVID-19, are part of a clinical risk profile associated with higher mortality.

Experiencia en endocarditis infecciosa a lo largo de 30 años en un hospital de Buenos Aires / Experience in infective endocarditis over 30 years in a hospital in Buenos Aires

Resumen La endocarditis infecciosa (EI) es una enfermedad que en las últimas décadas ha mostrado cambios en su presentación, diagnóstico y tratamiento. Se realizó un estudio prospectivo de 252 pacientes ingresados en un hospital de referencia en Buenos Aires, Argentina, con diagnóstico de EI, agrupados según década de ingreso: Grupo A: enero 1988 a diciembre 1997 (89 pacientes -35.3%-), Grupo B: enero 1998 a diciembre 2007 (88 pacientes -34.9%-), y Grupo C: enero 2008 a diciembre 2018 (75 pacientes -29.8%). Se analizaron y compararon las características: edad, sexo, cardiopatía de base, hemocultivos y gérmenes, presencia de vegetaciones, tratamiento quirúrgico y mortalidad intrahospitalaria. Durante las tres décadas, la cardiopatía predisponente mostró que la enfermedad valvular reumática disminuyó significativamente (p < 0.0001) mientras que la EI en los dispositivos cardíacos aumentó significativamente (p < 0.0001). El porcentaje de hemocultivos negativos disminuyó significativamente a lo largo de los años (p < 0.0001). La mortalidad hospitalaria mostró una reducción en la última década (p = 0.069). El desarrollo de complicaciones durante la hospitalización, la indicación de cirugía y la presencia de insuficiencia cardíaca al ingreso fueron predictores independientes de mortalidad hospitalaria. La presencia de vegetaciones y síndrome febril al ingreso fueron predictores independientes de menor mortalidad. La comparación a través de los años mostró cambios importantes en el perfil epidemiológico de la EI. Probablemente por el avance en las técnicas diagnósticas, el tratamiento y la implementación de equi pos interdisciplinarios de EI de la última década, la mortalidad intrahospitalaria marca una fuerte tendencia a la reducción.

Abstract Infective endocarditis (IE) is a disease that in recent decades has shown changes in its presentation, diagno sis and treatment. This is a prospective study of 252 patients admitted at a reference hospital in Buenos Aires, Argentina, with a diagnosis of IE and they were grouped according to the decade of admission: Group A: from January 1988 to December 1997 (89 patients -35.3%-), Group B: from January 1998 to December 2007 (88 patients -34.9%-), and Group C: from January 2008 to December 2018 (75 patients -29.8%). The characteristics were analyzed and compared: age, sex, underlying heart disease, blood cultures and germs, presence of veg etations, surgical treatment and in-hospital mortality. Over the three decades, the predisposing heart condition showed that rheumatic valve disease decreased significantly (p < 0.0001) while the IE in cardiac devices also increased significantly (p < 0.0001). The percentage of blood culture-negatives decreased significantly over the years (p < 0.0001). In-hospital mortality showed a downward trend in the last decade (p = 0.069). The devel opment of complications during hospitalization, the indication for surgery, and the presence of heart failure on admission were independent predictors of in-hospital mortality. The presence of vegetations and febrile syndrome on admission were independent predictor for lower mortality. The comparison over the years showed important changes in the epidemiological profile of IE. Probably due to advances in diagnostic techniques, treatment, and the implementation of interdisciplinary IE teams in the last decade, in-hospital mortality shows a strong tendency to decrease.

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.

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.

Assessment of coronary flow with transthoracic color Doppler echocardiography in patients with hypertrophic cardiomyopathy.

BACKGROUND: Recent advances in echocardiography have allowed assessment of flow velocity in the epicardial coronary arteries of patients with ischemic heart disease, using transthoracic color Doppler echocardiography (TTDE). However, few data are available regarding coronary blood flow in patients with hypertrophic cardiomyopathy (HCM). OBJECTIVE: To assess the epicardial coronary arteries of patients with HCM. MATERIAL AND METHODS: A group of 25 patients with HCM was assessed prospectively (mean age 57 ± 21 years, 11 male) using TTDE; flow velocities in the epicardial coronary arteries were measured and compared with those obtained in 10 age- and gender-matched controls. Analysis of the diastolic spectral waveform included flow velocity integral (VTI), peak velocity, deceleration time, and deceleration slope measurements, while systolic waveform analysis included peak flow measurement and morphology assessment (positive, absent or negative). An analysis of variance (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. RESULTS: Patients with HCM exhibited an increase in diastolic flow velocity with a rapid deceleration slope and a systolic slope which was decreased, absent or reversed, compared to normal subjects. On linear regression analysis there was no correlation with the type of hypertrophy or magnitude of the intraventricular pressure gradient in patients with obstructive HCM. CONCLUSION: In patients with HCM, noninvasive assessment with TTDE revealed abnormal findings in the distal flow of the epicardial coronary arteries, very similar to those seen in the no-reflow phenomenon. These findings were independent of the type of hypertrophy and magnitude of the intraventricular pressure gradient in patients with obstructive HCM.

[Active infective endocarditis: 152 cases]. / Endocarditis infecciosa activa: 152 casos.

Active infective endocarditis (IE) is a disease of low incidence that has showed changes in presentation, diagnosis and treatment options during the past decades. Despite these advances, mortality remains very high. Our goal was to analyze the characteristics of patients with active IE and their relationship with in-hospital mortality over 16 years. Between 1994 and 2010 we performed a prospective registry of 152 consecutive patients (64.5% male, age 45 ± 16 years) admitted with IE. Clinical characteristics, treatment and inpatient outcomes were analyzed. The most common causes of underlying heart disease were: congenital (21%) and rheumatic fever (13.2%). The reasons for hospitalization were fever (76.3%) and heart failure (40.1%). The infectious agent was identified in 69.7% of cases, and the most frequent was Streptococcus viridans. The echocardiogram showed vegetations in 80.9% of patients and 57.8% of them presented complications (the most frequent was heart failure) during hospitalization. Surgical treatment was indicated in 63.2% of cases, mainly due to heart failure. The overall hospital mortality was 30.2%. The presence of complications, requirement of surgical treatment and refractory heart failure were independent predictors of mortality whereas the single presence of vegetation showed better survival rate. The identification of these predictors could help to improve the outcomes in IE.

Endocarditis infecciosa activa: 152 casos / Active infective endocarditis: 152 cases

La endocarditis infecciosa es una enfermedad de baja incidencia que en las últimas décadas mostró modificaciones respecto de su presentación, posibilidad diagnóstica y tratamiento. A pesar de estos avances, la mortalidad hospitalaria sigue siendo muy elevada. Nuestro objetivo fue analizar las características de los pacientes con endocarditis infecciosa activa y su relación con la mortalidad hospitalaria a lo largo de 16 años. Se realizó un registro prospectivo entre 1994 y 2010 de pacientes ingresados con endocarditis. Se analizaron características clínicas, evolución y tratamiento y se registraron los eventos intrahospitalarios. Ingresaron 152 pacientes, 64.5% varones, edad 45 ± 16 años, las causas más frecuentes de cardiopatía de base fueron: congénita 32 (21%) y reumática 20 (13.2%). Los motivos de internación fueron síndrome febril 116 (76.3%) e insuficiencia cardíaca 61 (40.1%). Se identificó el agente infeccioso en 106 (69.7%) de los casos, el más frecuente fue Streptococcus viridans. El ecocardiograma mostró vegetaciones en 123 (80.9%) de los pacientes y 88 (57.8%) presentaron complicaciones durante su internación, siendo la más frecuente la insuficiencia cardíaca. Se indicó tratamiento quirúrgico en 96 (63.1%) de los casos, fundamentalmente por insuficiencia cardíaca en 66. La mortalidad hospitalaria global fue 46 (30.2%). El desarrollo de complicaciones en la internación, la indicación de cirugía y la presencia de insuficiencia cardíaca refractaria al tratamiento fueron predictores independientes de mortalidad hospitalaria, mientras que la presencia de vegetaciones resultó un predictor independiente de mejor supervivencia. La identificación temprana de estos predictores descriptos podría ayudar a mejorar los resultados.

Active infective endocarditis (IE) is a disease of low incidence that has showed changes in presentation, diagnosis and treatment options during the past decades. Despite these advances, mortality remains very high. Our goal was to analyze the characteristics of patients with active IE and their relationship with in-hospital mortality over 16 years. Between 1994 and 2010 we performed a prospective registry of 152 consecutive patients (64.5% male, age 45 ± 16 years) admitted with IE. Clinical characteristics, treatment and inpatient outcomes were analyzed. The most common causes of underlying heart disease were: congenital (21%) and rheumatic fever (13.2%). The reasons for hospitalization were fever (76.3%) and heart failure (40.1%). The infectious agent was identified in 69.7% of cases, and the most frequent was Streptococcus viridans. The echocardiogram showed vegetations in 80.9% of patients and 57.8% of them presented complications (the most frequent was heart failure) during hospitalization. Surgical treatment was indicated in 63.2% of cases, mainly due to heart failure. The overall hospital mortality was 30.2%. The presence of complications, requirement of surgical treatment and refractory heart failure were independent predictors of mortality whereas the single presence of vegetation showed better survival rate. The identification of these predictors could help to improve the outcomes in IE.

Endocarditis infecciosa activa: 152 casos / Active infective endocarditis: 152 cases

La endocarditis infecciosa es una enfermedad de baja incidencia que en las últimas décadas mostró modificaciones respecto de su presentación, posibilidad diagnóstica y tratamiento. A pesar de estos avances, la mortalidad hospitalaria sigue siendo muy elevada. Nuestro objetivo fue analizar las características de los pacientes con endocarditis infecciosa activa y su relación con la mortalidad hospitalaria a lo largo de 16 años. Se realizó un registro prospectivo entre 1994 y 2010 de pacientes ingresados con endocarditis. Se analizaron características clínicas, evolución y tratamiento y se registraron los eventos intrahospitalarios. Ingresaron 152 pacientes, 64.5% varones, edad 45 ± 16 años, las causas más frecuentes de cardiopatía de base fueron: congénita 32 (21%) y reumática 20 (13.2%). Los motivos de internación fueron síndrome febril 116 (76.3%) e insuficiencia cardíaca 61 (40.1%). Se identificó el agente infeccioso en 106 (69.7%) de los casos, el más frecuente fue Streptococcus viridans. El ecocardiograma mostró vegetaciones en 123 (80.9%) de los pacientes y 88 (57.8%) presentaron complicaciones durante su internación, siendo la más frecuente la insuficiencia cardíaca. Se indicó tratamiento quirúrgico en 96 (63.1%) de los casos, fundamentalmente por insuficiencia cardíaca en 66. La mortalidad hospitalaria global fue 46 (30.2%). El desarrollo de complicaciones en la internación, la indicación de cirugía y la presencia de insuficiencia cardíaca refractaria al tratamiento fueron predictores independientes de mortalidad hospitalaria, mientras que la presencia de vegetaciones resultó un predictor independiente de mejor supervivencia. La identificación temprana de estos predictores descriptos podría ayudar a mejorar los resultados.(AU)

Active infective endocarditis (IE) is a disease of low incidence that has showed changes in presentation, diagnosis and treatment options during the past decades. Despite these advances, mortality remains very high. Our goal was to analyze the characteristics of patients with active IE and their relationship with in-hospital mortality over 16 years. Between 1994 and 2010 we performed a prospective registry of 152 consecutive patients (64.5% male, age 45 ± 16 years) admitted with IE. Clinical characteristics, treatment and inpatient outcomes were analyzed. The most common causes of underlying heart disease were: congenital (21%) and rheumatic fever (13.2%). The reasons for hospitalization were fever (76.3%) and heart failure (40.1%). The infectious agent was identified in 69.7% of cases, and the most frequent was Streptococcus viridans. The echocardiogram showed vegetations in 80.9% of patients and 57.8% of them presented complications (the most frequent was heart failure) during hospitalization. Surgical treatment was indicated in 63.2% of cases, mainly due to heart failure. The overall hospital mortality was 30.2%. The presence of complications, requirement of surgical treatment and refractory heart failure were independent predictors of mortality whereas the single presence of vegetation showed better survival rate. The identification of these predictors could help to improve the outcomes in IE.(AU)