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.

[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.

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.

Examen clínico y maniobra de valsalva en la insuficiencia cardíaca / Clinical examination and the Valsalva maneuver in heart failure

La evaluación de la congestión en pacientes con insuficiencia cardíaca y fracción de eyección reducida (ICFEr) resulta relevante y estrechamente vinculada al curso clínico. El agregado del comportamiento de la presión arterial durante la maniobra de Valsalva en la cabecera del paciente (VAL) podría mejorar la evaluación clínica de congestión cuando la comparamos con los niveles de NT-proBNP y la estimación de la presión media en la aurícula izquierda por Doppler cardíaco, como subrogantes de congestión. Se realizó un examen clínico con el VAL, un examen de laboratorio y un Doppler cardíaco en 69 pacientes ambulatorios con insuficiencia cardíaca y fracción de eyección ≤ 40% en ritmo sinusal. El score de Framingham ≥ 2 (SFr ≥ 2) se empleó para evaluar congestión clínica. VAL fue clasificado como normal o anormal, el NT-proBNP como bajo (< 1000 pg/ml) o alto (≥ 1000 pg/ml) y la relación entre la velocidad del llenado ventricular rápido y la velocidad del Doppler tisular (E/e') como baja < 15 o alta ≥ 15. Se halló que 13/27 pacientes con SFr ≥ 2 tenían NT-proBNP alto (sensibilidad 62%, especificidad 70% y razón de verosimilitud positiva [LR+] de 2.08, p = 0.01). El agregado del VAL al SFr ≥ 2 mejoró la exactitud diagnóstica (sensibilidad 100%, especificidad 64% y LR+ 2.8 p = 0.0004). Comparado con la E/e', SFr ≥ 2 con VAL anormal mostró sensibilidad 86%, especificidad 56% y LR + 1.86 (p = 0.03). En conclusión, el agregado del comportamiento de la presión durante la maniobra de Valsalva podría mejorar la evaluación clínica de la congestión en la insuficiencia cardíaca.

Congestion in heart failure patients with reduced ejection fraction (HFrEF) is relevant and closely linked to the clinical course. Bedside blood pressure measurement during the Valsalva maneuver (Val) added to clinical examination may improve the assessment of congestion when compared to NT-proBNP levels and left atrial pressure (LAP) estimation by Doppler echocardiography, as surrogate markers of congestion in HFrEF. A clinical examination, LAP and blood tests were performed in 69 HFrEF ambulatory patients with left ventricular ejection fraction ≤ 40% and sinus rhythm. Framingham Heart Failure Score (HFS) was used to evaluate clinical congestion; Val was classified as normal or abnormal, NT-proBNP was classified as low (< 1000 pg/ml) or high (≥ 1000 pg/ml) and the ratio between Doppler early mitral inflow and tissue diastolic velocity was used to estimate LAP and was classified as low (E/e'< 15) or high (E/e' ≥ 15). A total of 69 patients with HFrEF were included; 27 had a HFS ≥ 2 and 13 of them had high NT-proBNP. HFS ≥ 2 had a 62% sensitivity, 70% specificity and a positive likelihood ratio of 2.08 (p=0.01) to detect congestion. When Val was added to clinical examination, the presence of a HFS ≥ 2 and abnormal Val showed a 100% sensitivity, 64% specificity and a positive likelihood ratio of 2.8 (p = 0.0004). Compared with LAP, the presence of HFS ≥ 2 and abnormal Val had 86% sensitivity, 54% specificity and a positive likelihood ratio of 1.86 (p = 0.03). In conclusion, an integrated clinical examination with the addition Valsalva maneuver may improve the assessment of congestion in patients with HFrEF.

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.

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.

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.